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Konrad Sandhoff - Publications

Affiliations: 
1979- Rheinische Friedrich-Wilhelms-Universität Bonn, Bonn, Nordrhein-Westfalen, Germany 
Area:
Lipid biochemistry
Website:
https://www.limes-institut-bonn.de/en/research/research-departments/unit-3/sandhoff-lab/people/lab-head/#c5876
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Year Citation  Score
2022 Lopergolo D, Berti G, Mari F, Bertini E, Rufa A, Battisti C, Sicurelli F, Renieri A, Federico A, Sandhoff K, Malandrini A. A case of spastic paraplegia type 11 mimicking a GM2-gangliosidosis. Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology. 43: 2849-2852. PMID 35066644 DOI: 10.1007/s10072-021-05841-8  0.268
2021 Breiden B, Sandhoff K. Corrigendum to: Emerging mechanisms of drug-induced phospholipidosis. Biological Chemistry. PMID 34706402 DOI: 10.1515/hsz-2021-0383  0.183
2021 Sandhoff K. Secondary ganglioside GM2 accumulation in mucopolysaccharidoses. Molecular Genetics and Metabolism Reports. 28: 100789. PMID 34485082 DOI: 10.1016/j.ymgmr.2021.100789  0.249
2021 Breiden B, Sandhoff K. Acid Sphingomyelinase, a Lysosomal and Secretory Phospholipase C, Is Key for Cellular Phospholipid Catabolism. International Journal of Molecular Sciences. 22. PMID 34445706 DOI: 10.3390/ijms22169001  0.389
2020 Breiden B, Sandhoff K. Mechanism of Secondary Ganglioside and Lipid Accumulation in Lysosomal Disease. International Journal of Molecular Sciences. 21. PMID 32272755 DOI: 10.3390/Ijms21072566  0.459
2019 Sen NE, Arsovic A, Meierhofer D, Brodesser S, Oberschmidt C, Canet-Pons J, Kaya ZE, Halbach MV, Gispert S, Sandhoff K, Auburger G. In Human and Mouse Spino-Cerebellar Tissue, Ataxin-2 Expansion Affects Ceramide-Sphingomyelin Metabolism. International Journal of Molecular Sciences. 20. PMID 31766565 DOI: 10.3390/Ijms20235854  0.422
2019 Breiden B, Sandhoff K. Emerging mechanisms of drug-induced phospholipidosis. Biological Chemistry. PMID 31408430 DOI: 10.1515/Hsz-2019-0270  0.402
2019 Breiden B, Sandhoff K. Lysosomal Glycosphingolipid Storage Diseases. Annual Review of Biochemistry. 88: 461-485. PMID 31220974 DOI: 10.1146/Annurev-Biochem-013118-111518  0.464
2019 Anheuser S, Breiden B, Sandhoff K. Ganglioside GM2 catabolism is inhibited by storage compounds of mucopolysaccharidoses and by cationic amphiphilic drugs. Molecular Genetics and Metabolism. PMID 31097363 DOI: 10.1016/J.Ymgme.2019.04.007  0.444
2019 Anheuser S, Breiden B, Sandhoff K. Membrane lipids and their degradation compounds control GM2 catabolism at intralysosomal luminal vesicles. Journal of Lipid Research. 60: 1099-1111. PMID 30988135 DOI: 10.1194/Jlr.M092551  0.455
2019 Uhlhorn-Dierks G, Kolter T, Sandhoff K. How Does Nature Cleave Sulfuric Acid Esters? A Novel Posttranslational Modification of Sulfatases. Angewandte Chemie (International Ed. in English). 37: 2453-2455. PMID 29711349 DOI: 10.1002/(Sici)1521-3773(19981002)37:18<2453::Aid-Anie2453>3.0.Co;2-N  0.442
2019 Kolter T, Sandhoff K. Sphingolipids-Their Metabolic Pathways and the Pathobiochemistry of Neurodegenerative Diseases. Angewandte Chemie (International Ed. in English). 38: 1532-1568. PMID 29710982 DOI: 10.1002/(Sici)1521-3773(19990601)38:11<1532::Aid-Anie1532>3.0.Co;2-U  0.386
2019 Sandhoff K. Variation of beta-N-acetylhexosaminidase-pattern in Tay-Sachs disease. Febs Letters. 4: 351-354. PMID 11947222 DOI: 10.1016/0014-5793(69)80274-7  0.343
2019 Sandhoff K. The hydrolysis of Tay-Sachs ganglioside (TSG) by human N-acetyl-beta-D-hexosaminidase A. Febs Letters. 11: 342-344. PMID 11945522 DOI: 10.1016/0014-5793(70)80564-6  0.365
2018 Breiden B, Sandhoff K. Ganglioside Metabolism and Its Inherited Diseases. Methods in Molecular Biology (Clifton, N.J.). 1804: 97-141. PMID 29926406 DOI: 10.1007/978-1-4939-8552-4_5  0.455
2018 Sandhoff R, Sandhoff K. Emerging concepts of ganglioside metabolism. Febs Letters. 592: 3835-3864. PMID 29802621 DOI: 10.1002/1873-3468.13114  0.448
2018 Sandhoff R, Schulze H, Sandhoff K. Ganglioside Metabolism in Health and Disease. Progress in Molecular Biology and Translational Science. 156: 1-62. PMID 29747811 DOI: 10.1016/Bs.Pmbts.2018.01.002  0.478
2018 Bickert A, Kern P, van Uelft M, Herresthal S, Ulas T, Gutbrod K, Breiden B, Degen J, Sandhoff K, Schultze JL, Dörmann P, Hartmann D, Bauer R, Willecke K. Inactivation of ceramide synthase 2 catalytic activity in mice affects transcription of genes involved in lipid metabolism and cell division. Biochimica Et Biophysica Acta. 1863: 734-749. PMID 29653252 DOI: 10.1016/J.Bbalip.2018.04.006  0.415
2018 Sociale M, Wulf AL, Breiden B, Klee K, Thielisch M, Eckardt F, Sellin J, Bülow MH, Löbbert S, Weinstock N, Voelzmann A, Schultze J, Sandhoff K, Bauer R. Ceramide Synthase Schlank Is a Transcriptional Regulator Adapting Gene Expression to Energy Requirements. Cell Reports. 22: 967-978. PMID 29386138 DOI: 10.1016/J.Celrep.2017.12.090  0.384
2017 Bülow MH, Wingen C, Senyilmaz D, Gosejacob D, Sociale M, Bauer R, Schulze H, Sandhoff K, Teleman AA, Hoch M, Sellin J. Unbalanced lipolysis results in lipotoxicity and mitochondrial damage in peroxisome-deficient Pex19 mutants. Molecular Biology of the Cell. PMID 29282281 DOI: 10.1091/Mbc.E17-08-0535  0.392
2017 Sellin J, Schulze H, Paradis M, Gosejacob D, Papan C, Shevchenko A, Psathaki OE, Paululat A, Thielisch M, Sandhoff K, Hoch M. Characterization of Drosophila saposin-related mutants as a model for lysosomal sphingolipid storage diseases. Disease Models & Mechanisms. PMID 28389479 DOI: 10.1242/Dmm.027953  0.445
2017 Graf CG, Schulz C, Schmälzlein M, Heinlein C, Mönnich M, Perkams L, Püttner M, Boos I, Hessefort M, Lombana Sanchez JN, Weyand M, Steegborn C, Breiden B, Ross K, Schwarzmann G, ... Sandhoff K, et al. Synthetic Glycoforms Reveal Carbohydrate-Dependent Bioactivity of Human Saposin D. Angewandte Chemie (International Ed. in English). PMID 28378443 DOI: 10.1002/Anie.201701362  0.403
2017 Schonauer S, Körschen HG, Penno A, Rennhack A, Breiden B, Sandhoff K, Gutbrod K, Dörmann P, Raju DN, Haberkant P, Gerl MJ, Brügger B, Zigdon H, Vardi A, Futerman AH, et al. Identification of a feedback loop involving beta-glucosidase 2 and its product sphingosine sheds light on the molecular mechanisms in Gaucher disease. The Journal of Biological Chemistry. PMID 28258214 DOI: 10.1074/Jbc.M116.762831  0.429
2017 Abdul-Hammed M, Breiden B, Schwarzmann G, Sandhoff K. Lipids regulate the hydrolysis of membrane bound glucosylceramide by lysosomal β-glucocerebrosidase. Journal of Lipid Research. 58: 563-577. PMID 28126847 DOI: 10.1194/Jlr.M073510  0.441
2017 Sandhoff K. Sphingolipidoses membrane lipids regulate and modify sphingolipid catabolism, its enzymes, lipid binding and transfer proteins Molecular Genetics and Metabolism. 120: S118. DOI: 10.1016/J.Ymgme.2016.11.304  0.432
2016 Sandhoff K. Neuronal sphingolipidoses: Membrane lipids and sphingolipid activator proteins regulate lysosomal sphingolipid catabolism. Biochimie. 130: 146-151. PMID 27157270 DOI: 10.1016/J.Biochi.2016.05.004  0.462
2015 Schwarzmann G, Breiden B, Sandhoff K. Membrane-spanning lipids for an uncompromised monitoring of membrane fusion and intermembrane lipid transfer. Journal of Lipid Research. 56: 1861-79. PMID 26269359 DOI: 10.1194/Jlr.M056929  0.416
2015 Anheuser S, Breiden B, Schwarzmann G, Sandhoff K. Membrane lipids regulate ganglioside GM2 catabolism and GM2 activator protein activity. Journal of Lipid Research. 56: 1747-61. PMID 26175473 DOI: 10.1194/Jlr.M061036  0.452
2015 Raju D, Schonauer S, Hamzeh H, Flynn KC, Bradke F, Vom Dorp K, Dörmann P, Yildiz Y, Trötschel C, Poetsch A, Breiden B, Sandhoff K, Körschen HG, Wachten D. Accumulation of glucosylceramide in the absence of the beta-glucosidase GBA2 alters cytoskeletal dynamics. Plos Genetics. 11: e1005063. PMID 25803043 DOI: 10.1371/Journal.Pgen.1005063  0.398
2014 Gantner M, Schwarzmann G, Sandhoff K, Kolter T. Partial synthesis of ganglioside and lysoganglioside lipoforms as internal standards for MS quantification. Journal of Lipid Research. 55: 2692-704. PMID 25341943 DOI: 10.1194/Jlr.D054734  0.379
2014 Oninla VO, Breiden B, Babalola JO, Sandhoff K. Acid sphingomyelinase activity is regulated by membrane lipids and facilitates cholesterol transfer by NPC2. Journal of Lipid Research. 55: 2606-19. PMID 25339683 DOI: 10.1194/Jlr.M054528  0.438
2014 Schäfer M, Willrodt AH, Kurinna S, Link AS, Farwanah H, Geusau A, Gruber F, Sorg O, Huebner AJ, Roop DR, Sandhoff K, Saurat JH, Tschachler E, Schneider MR, Langbein L, et al. Activation of Nrf2 in keratinocytes causes chloracne (MADISH)-like skin disease in mice. Embo Molecular Medicine. 6: 442-57. PMID 24503019 DOI: 10.1002/Emmm.201303281  0.395
2014 Schwarzmann G, Arenz C, Sandhoff K. Labeled chemical biology tools for investigating sphingolipid metabolism, trafficking and interaction with lipids and proteins. Biochimica Et Biophysica Acta. 1841: 1161-73. PMID 24389251 DOI: 10.1016/J.Bbalip.2013.12.011  0.74
2014 Schulze H, Sandhoff K. Sphingolipids and lysosomal pathologies. Biochimica Et Biophysica Acta. 1841: 799-810. PMID 24184515 DOI: 10.1016/J.Bbalip.2013.10.015  0.454
2014 Breiden B, Sandhoff K. The role of sphingolipid metabolism in cutaneous permeability barrier formation. Biochimica Et Biophysica Acta. 1841: 441-52. PMID 23954553 DOI: 10.1016/J.Bbalip.2013.08.010  0.438
2013 Sandhoff K. Metabolic and cellular bases of sphingolipidoses. Biochemical Society Transactions. 41: 1562-8. PMID 24256255 DOI: 10.1042/Bst20130083  0.455
2013 Tecedor L, Stein CS, Schultz ML, Farwanah H, Sandhoff K, Davidson BL. CLN3 loss disturbs membrane microdomain properties and protein transport in brain endothelial cells. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 33: 18065-79. PMID 24227717 DOI: 10.1523/Jneurosci.0498-13.2013  0.409
2013 Saftig P, Sandhoff K. Cancer: Killing from the inside. Nature. 502: 312-3. PMID 24089207 DOI: 10.1038/Nature12692  0.348
2013 Yildiz Y, Hoffmann P, Vom Dahl S, Breiden B, Sandhoff R, Niederau C, Horwitz M, Karlsson S, Filocamo M, Elstein D, Beck M, Sandhoff K, Mengel E, Gonzalez MC, Nöthen MM, et al. Functional and genetic characterization of the non-lysosomal glucosylceramidase 2 as a modifier for Gaucher disease. Orphanet Journal of Rare Diseases. 8: 151. PMID 24070122 DOI: 10.1186/1750-1172-8-151  0.388
2013 Piyanova A, Albayram O, Rossi CA, Farwanah H, Michel K, Nicotera P, Sandhoff K, Bilkei-Gorzo A. Loss of CB1 receptors leads to decreased cathepsin D levels and accelerated lipofuscin accumulation in the hippocampus. Mechanisms of Ageing and Development. 134: 391-9. PMID 23954857 DOI: 10.1016/J.Mad.2013.08.001  0.365
2013 Sandhoff K, Harzer K. Gangliosides and gangliosidoses: principles of molecular and metabolic pathogenesis. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 33: 10195-208. PMID 23785136 DOI: 10.1523/Jneurosci.0822-13.2013  0.468
2012 Sandhoff K. My journey into the world of sphingolipids and sphingolipidoses. Proceedings of the Japan Academy. Series B, Physical and Biological Sciences. 88: 554-82. PMID 23229750 DOI: 10.2183/pjab.88.554  0.365
2012 Ginkel C, Hartmann D, vom Dorp K, Zlomuzica A, Farwanah H, Eckhardt M, Sandhoff R, Degen J, Rabionet M, Dere E, Dörmann P, Sandhoff K, Willecke K. Ablation of neuronal ceramide synthase 1 in mice decreases ganglioside levels and expression of myelin-associated glycoprotein in oligodendrocytes. The Journal of Biological Chemistry. 287: 41888-902. PMID 23074226 DOI: 10.1074/Jbc.M112.413500  0.398
2012 Schäfer M, Farwanah H, Willrodt AH, Huebner AJ, Sandhoff K, Roop D, Hohl D, Bloch W, Werner S. Nrf2 links epidermal barrier function with antioxidant defense. Embo Molecular Medicine. 4: 364-79. PMID 22383093 DOI: 10.1002/Emmm.201200219  0.388
2012 Fehrenschild D, Galli U, Breiden B, Bloch W, Schettina P, Brodesser S, Michels C, Günschmann C, Sandhoff K, Niessen CM, Niemann C. TCF/Lef1-mediated control of lipid metabolism regulates skin barrier function. The Journal of Investigative Dermatology. 132: 337-45. PMID 21938009 DOI: 10.1038/Jid.2011.301  0.395
2011 Farwanah H, Kolter T, Sandhoff K. Mass spectrometric analysis of neutral sphingolipids: methods, applications, and limitations. Biochimica Et Biophysica Acta. 1811: 854-60. PMID 21740983 DOI: 10.1016/J.Bbalip.2011.05.011  0.374
2011 Schulze H, Sandhoff K. Lysosomal lipid storage diseases. Cold Spring Harbor Perspectives in Biology. 3. PMID 21502308 DOI: 10.1101/Cshperspect.A004804  0.45
2011 Tamboli IY, Hampel H, Tien NT, Tolksdorf K, Breiden B, Mathews PM, Saftig P, Sandhoff K, Walter J. Sphingolipid storage affects autophagic metabolism of the amyloid precursor protein and promotes Abeta generation. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 31: 1837-49. PMID 21289194 DOI: 10.1523/Jneurosci.2954-10.2011  0.394
2011 Frateschi S, Camerer E, Crisante G, Rieser S, Membrez M, Charles RP, Beermann F, Stehle JC, Breiden B, Sandhoff K, Rotman S, Haftek M, Wilson A, Ryser S, Steinhoff M, et al. PAR2 absence completely rescues inflammation and ichthyosis caused by altered CAP1/Prss8 expression in mouse skin. Nature Communications. 2: 161. PMID 21245842 DOI: 10.1038/Ncomms1162  0.382
2011 Gallala HD, Breiden B, Sandhoff K. Regulation of the NPC2 protein-mediated cholesterol trafficking by membrane lipids. Journal of Neurochemistry. 116: 702-7. PMID 21214551 DOI: 10.1111/J.1471-4159.2010.07014.X  0.446
2011 Gallala HD, Sandhoff K. Biological function of the cellular lipid BMP-BMP as a key activator for cholesterol sorting and membrane digestion. Neurochemical Research. 36: 1594-600. PMID 21136156 DOI: 10.1007/S11064-010-0337-6  0.421
2011 Schneede A, Schmidt CK, Hölttä-Vuori M, Heeren J, Willenborg M, Blanz J, Domanskyy M, Breiden B, Brodesser S, Landgrebe J, Sandhoff K, Ikonen E, Saftig P, Eskelinen EL. Role for LAMP-2 in endosomal cholesterol transport. Journal of Cellular and Molecular Medicine. 15: 280-95. PMID 19929948 DOI: 10.1111/J.1582-4934.2009.00973.X  0.398
2011 Orsó E, Sigruener A, Scherer M, Liebisch G, Kluenemann H, Sandhoff K, Kolter T, Schmitz G. Lipidomic and transcriptomic profiling of human Niemann–Pick type C macrophages and fibroblasts: implications to disease pathogenesis Chemistry and Physics of Lipids. 164: S27. DOI: 10.1016/J.Chemphyslip.2011.05.090  0.369
2010 Wähe A, Kasmapour B, Schmaderer C, Liebl D, Sandhoff K, Nykjaer A, Griffiths G, Gutierrez MG. Golgi-to-phagosome transport of acid sphingomyelinase and prosaposin is mediated by sortilin Journal of Cell Science. 123: 2502-2511. PMID 20571055 DOI: 10.1242/jcs.067686  0.309
2010 Abdul-Hammed M, Breiden B, Adebayo MA, Babalola JO, Schwarzmann G, Sandhoff K. Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion. Journal of Lipid Research. 51: 1747-60. PMID 20179319 DOI: 10.1194/Jlr.M003822  0.41
2010 Kirkegaard T, Roth AG, Petersen NH, Mahalka AK, Olsen OD, Moilanen I, Zylicz A, Knudsen J, Sandhoff K, Arenz C, Kinnunen PK, Nylandsted J, Jäättelä M. Hsp70 stabilizes lysosomes and reverts Niemann-Pick disease-associated lysosomal pathology. Nature. 463: 549-53. PMID 20111001 DOI: 10.1038/Nature08710  0.739
2010 Kolter T, Sandhoff K. Lysosomal degradation of membrane lipids. Febs Letters. 584: 1700-12. PMID 19836391 DOI: 10.1016/J.Febslet.2009.10.021  0.449
2010 Kolter T, Sandhoff K. ChemInform Abstract: Sphingolipids-Their Metabolic Pathways and the Pathobiochemistry of Neurodegenerative Diseases Cheminform. 30: no-no. DOI: 10.1002/CHIN.199933328  0.265
2010 UHLHORN-DIERKS G, KOLTER T, SANDHOFF K. ChemInform Abstract: How Does Nature Cleave Sulfuric Acid Esters? A Novel Posttranslational Modification of Sulfatases Cheminform. 30: no-no. DOI: 10.1002/CHIN.199903294  0.281
2010 KOLTER T, SANDHOFF K. ChemInform Abstract: Inhibitors of Glycosphingolipid Biosynthesis Cheminform. 28: no-no. DOI: 10.1002/CHIN.199725296  0.244
2010 SCHICK A, KOLTER T, GIANNIS A, SANDHOFF K. ChemInform Abstract: Synthesis of Phosphoramide Analogues of Sphinganine-1-phosphate. Cheminform. 27: no-no. DOI: 10.1002/CHIN.199625242  0.26
2010 KOLTER T, SANDHOFF K. ChemInform Abstract: New Brain Lipids That Induce Sleep Cheminform. 27: no-no. DOI: 10.1002/CHIN.199608328  0.225
2010 SCHICK A, KOLTER T, GIANNIS A, SANDHOFF K. ChemInform Abstract: Synthesis of Phosphonate Analogues of Sphinganine-1-phosphate and Sphingosine-1-phosphate. Cheminform. 27: no-no. DOI: 10.1002/CHIN.199605200  0.269
2010 BAER T, KRATZER B, WILD R, SANDHOFF K, SCHMIDT RR. ChemInform Abstract: Synthesis of Sphingosines. Part 8. Synthesis of Methyl-Branched Sphingosines. Cheminform. 24: no-no. DOI: 10.1002/CHIN.199332276  0.233
2010 BIRK R, SANDHOFF K, SCHMIDT RR. ChemInform Abstract: Epoxides of Phytosphingosine and Derivatives, Potential Inhibitors of Sphingosine Biosynthesis. Cheminform. 24: no-no. DOI: 10.1002/CHIN.199322273  0.256
2010 PLEWE M, SANDHOFF K, SCHMIDT RR. ChemInform Abstract: Synthesis of 4-Epoxy-4-C-methyleneglycosylceramides, Potential Glycosyltransferase Inhibitors. Cheminform. 23: no-no. DOI: 10.1002/CHIN.199246237  0.248
2010 BRENNER-WEISS G, GIANNIS A, SANDHOFF K. ChemInform Abstract: Synthesis of Potential Inhibitors of the Glycosphingolipid Biosynthesis. Cheminform. 23: no-no. DOI: 10.1002/CHIN.199245287  0.255
2010 KUHN C, LEHMANN J, SANDHOFF K. ChemInform Abstract: Irreversible Deactivation of Human β-Hexosaminidase A with the Alkylating Pseudodisaccharide (3,4,6/5)-6-(2-Acetamido-2-deoxy-β- D-glucopyranosylthio)-3,4-epoxy-5-hydroxycyclohexene Cheminform. 23: no-no. DOI: 10.1002/CHIN.199233275  0.231
2010 HENK T, GIANNIS A, SANDHOFF K. ChemInform Abstract: Wittig Olefination of Unprotected Carbohydrates with a Semistabilized Ylide. Cheminform. 23: no-no. DOI: 10.1002/chin.199220276  0.619
2010 ATS S, LAUSBERG E, LEHMANN J, SANDHOFF K. ChemInform Abstract: Synthesis of 4-Acetamido-3,7-anhydro-2-azi-1,2,4-trideoxy-D-glycero-D-gulo-octitol (V), a Good Competitive Inhibitor and Potential Photoaffinity Reagent for Human β-Hexosaminidase. Cheminform. 22: no-no. DOI: 10.1002/CHIN.199110303  0.257
2009 Bauer R, Voelzmann A, Breiden B, Schepers U, Farwanah H, Hahn I, Eckardt F, Sandhoff K, Hoch M. Schlank, a member of the ceramide synthase family controls growth and body fat in Drosophila. The Embo Journal. 28: 3706-16. PMID 19834458 DOI: 10.1038/Emboj.2009.305  0.408
2009 Imgrund S, Hartmann D, Farwanah H, Eckhardt M, Sandhoff R, Degen J, Gieselmann V, Sandhoff K, Willecke K. Adult ceramide synthase 2 (CERS2)-deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas. The Journal of Biological Chemistry. 284: 33549-60. PMID 19801672 DOI: 10.1074/Jbc.M109.031971  0.394
2009 Bach C, Gilch S, Rost R, Greenwood AD, Horsch M, Hajj GN, Brodesser S, Facius A, Schädler S, Sandhoff K, Beckers J, Leib-Mösch C, Schätzl HM, Vorberg I. Prion-induced activation of cholesterogenic gene expression by Srebp2 in neuronal cells. The Journal of Biological Chemistry. 284: 31260-9. PMID 19748890 DOI: 10.1074/Jbc.M109.004382  0.401
2009 Farwanah H, Wirtz J, Kolter T, Raith K, Neubert RH, Sandhoff K. Normal phase liquid chromatography coupled to quadrupole time of flight atmospheric pressure chemical ionization mass spectrometry for separation, detection and mass spectrometric profiling of neutral sphingolipids and cholesterol. Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences. 877: 2976-82. PMID 19646933 DOI: 10.1016/J.Jchromb.2009.07.008  0.369
2009 Matzner U, Breiden B, Schwarzmann G, Yaghootfam A, Fluharty AL, Hasilik A, Sandhoff K, Gieselmann V. Saposin B-dependent reconstitution of arylsulfatase A activity in vitro and in cell culture models of metachromatic leukodystrophy. The Journal of Biological Chemistry. 284: 9372-81. PMID 19224915 DOI: 10.1074/Jbc.M809457200  0.4
2009 Martino S, di Girolamo I, Cavazzin C, Tiribuzi R, Galli R, Rivaroli A, Valsecchi M, Sandhoff K, Sonnino S, Vescovi A, Gritti A, Orlacchio A. Neural precursor cell cultures from GM2 gangliosidosis animal models recapitulate the biochemical and molecular hallmarks of the brain pathology. Journal of Neurochemistry. 109: 135-47. PMID 19166507 DOI: 10.1111/J.1471-4159.2009.05919.X  0.41
2009 Eggeling C, Ringemann C, Medda R, Schwarzmann G, Sandhoff K, Polyakova S, Belov VN, Hein B, Von Middendorff C, Schönle A, Hell SW. Direct observation of the nanoscale dynamics of membrane lipids in a living cell Nature. 457: 1159-1162. PMID 19098897 DOI: 10.1038/Nature07596  0.406
2009 Schulze H, Kolter T, Sandhoff K. Principles of lysosomal membrane degradation: Cellular topology and biochemistry of lysosomal lipid degradation. Biochimica Et Biophysica Acta. 1793: 674-83. PMID 19014978 DOI: 10.1016/J.Bbamcr.2008.09.020  0.451
2009 Wendeler M, Sandhoff K. Hexosaminidase assays. Glycoconjugate Journal. 26: 945-52. PMID 18473163 DOI: 10.1007/s10719-008-9137-5  0.314
2009 Sandhoff K. Membrane lipids and degenerative lysosomal storage disorders Clinical Therapeutics. 31: S179-S180. DOI: 10.1016/S0149-2918(09)00425-1  0.285
2009 Gritti A, Martino S, Girolamo Id, Cavazzin C, Tiribuzi R, Sandhoff K, Rivaroli A, Sonnino S, Vescovi A, Orlacchio A. 60. Neural precursor cell cultures from GM2-gangliosidosis murine models recapitulate the biochemical hallmarks of the brain pathology Molecular Genetics and Metabolism. 96: S25. DOI: 10.1016/J.Ymgme.2008.11.061  0.352
2008 Rossmann M, Schultz-Heienbrok R, Behlke J, Remmel N, Alings C, Sandhoff K, Saenger W, Maier T. Crystal structures of human saposins C andD: implications for lipid recognition and membrane interactions. Structure (London, England : 1993). 16: 809-17. PMID 18462685 DOI: 10.1016/J.Str.2008.02.016  0.4
2008 Lastres-Becker I, Brodesser S, Lütjohann D, Azizov M, Buchmann J, Hintermann E, Sandhoff K, Schürmann A, Nowock J, Auburger G. Insulin receptor and lipid metabolism pathology in ataxin-2 knock-out mice. Human Molecular Genetics. 17: 1465-81. PMID 18250099 DOI: 10.1093/Hmg/Ddn035  0.382
2008 Charles RP, Guitard M, Leyvraz C, Breiden B, Haftek M, Haftek-Terreau Z, Stehle JC, Sandhoff K, Hummler E. Postnatal requirement of the epithelial sodium channel for maintenance of epidermal barrier function. The Journal of Biological Chemistry. 283: 2622-30. PMID 18039670 DOI: 10.1074/Jbc.M708829200  0.395
2008 Kirkegaard-Sørensen T, Roth A, Moilanen I, Zylicz A, Schmitt E, Grüenberg J, Arenz C, Garrido C, Sandhoff K, Kinnunen PKJ, Nylandsted J, Jäättel M. 45 Interaction between Hsp70 and bis(monoacylglycero)phosphate stabilizes lysosomes and promotes cell survival Apmis. 116: 436-437. DOI: 10.1111/J.1600-0463.2008.00Abs1165_53.X  0.707
2008 Luetjohann D, Tamboli I, Prager K, Thal D, Thelen K, Wahle T, Breiden B, Sandhoff K, Leuven Fv, Walter J. ROLE OF PRESENILINS IN LIPID HOMEOSTASIS Atherosclerosis Supplements. 9: 30. DOI: 10.1016/S1567-5688(08)70113-2  0.27
2008 Tamboli IY, Walter J, Sandhoff K. P1-454: Role of sphingolipids in regulation of Alzhimer's disease-associated proteins metabolism Alzheimer's & Dementia. 4: T353-T353. DOI: 10.1016/J.Jalz.2008.05.1037  0.379
2007 Schulze H, Schepers U, Sandhoff K. Overexpression and mass spectrometry analysis of mature human acid ceramidase. Biological Chemistry. 388: 1333-43. PMID 18020949 DOI: 10.1515/Bc.2007.152  0.436
2007 Breiden B, Gallala H, Doering T, Sandhoff K. Optimization of submerged keratinocyte cultures for the synthesis of barrier ceramides. European Journal of Cell Biology. 86: 657-73. PMID 17714827 DOI: 10.1016/J.Ejcb.2007.02.006  0.412
2007 Remmel N, Locatelli-Hoops S, Breiden B, Schwarzmann G, Sandhoff K. Saposin B mobilizes lipids from cholesterol-poor and bis(monoacylglycero)phosphate-rich membranes at acidic pH. Unglycosylated patient variant saposin B lacks lipid-extraction capacity. The Febs Journal. 274: 3405-20. PMID 17561962 DOI: 10.1111/J.1742-4658.2007.05873.X  0.44
2007 Babalola JO, Wendeler M, Breiden B, Arenz C, Schwarzmann G, Locatelli-Hoops S, Sandhoff K. Development of an assay for the intermembrane transfer of cholesterol by Niemann-Pick C2 protein. Biological Chemistry. 388: 617-26. PMID 17552909 DOI: 10.1515/Bc.2007.063  0.746
2007 Farwanah H, Pierstorff B, Schmelzer CE, Raith K, Neubert RH, Kolter T, Sandhoff K. Separation and mass spectrometric characterization of covalently bound skin ceramides using LC/APCI-MS and Nano-ESI-MS/MS. Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences. 852: 562-70. PMID 17368999 DOI: 10.1016/J.Jchromb.2007.02.030  0.369
2007 Gareus R, Huth M, Breiden B, Nenci A, Rösch N, Haase I, Bloch W, Sandhoff K, Pasparakis M. Normal epidermal differentiation but impaired skin-barrier formation upon keratinocyte-restricted IKK1 ablation. Nature Cell Biology. 9: 461-9. PMID 17351639 DOI: 10.1038/Ncb1560  0.406
2007 Rummel A, Eichner T, Weil T, Karnath T, Gutcaits A, Mahrhold S, Sandhoff K, Proia RL, Acharya KR, Bigalke H, Binz T. Identification of the protein receptor binding site of botulinum neurotoxins B and G proves the double-receptor concept. Proceedings of the National Academy of Sciences of the United States of America. 104: 359-64. PMID 17185412 DOI: 10.1073/Pnas.0609713104  0.38
2007 Jennemann R, Sandhoff R, Langbein L, Kaden S, Rothermel U, Gallala H, Sandhoff K, Wiegandt H, Gröne HJ. Integrity and barrier function of the epidermis critically depend on glucosylceramide synthesis. The Journal of Biological Chemistry. 282: 3083-94. PMID 17145749 DOI: 10.1074/Jbc.M610304200  0.442
2007 Sandhoff K. Cellular Sphingolipid Metabolism and Membrane Digestion Gbm Annual Spring Meeting Mosbach 2007. 2007. DOI: 10.1240/sav_gbm_2007_m_001812  0.292
2007 Schulze H, Bartelsen O, Schepers U, Sandhoff K. Expression of human acid ceramidase in insect cells. Analysis of processing, glycosylation and disulfide bond pattern. Gbm Annual Spring Meeting Mosbach 2007. 2007. DOI: 10.1240/sav_gbm_2007_m_001721  0.262
2007 Sandhoff K. Cellular sphingolipid metabolism and membrane digestion Chemistry and Physics of Lipids. 149: S7-S8. DOI: 10.1016/J.Chemphyslip.2007.06.017  0.392
2006 Locatelli-Hoops S, Remmel N, Klingenstein R, Breiden B, Rossocha M, Schoeniger M, Koenigs C, Saenger W, Sandhoff K. Saposin A mobilizes lipids from low cholesterol and high bis(monoacylglycerol)phosphate-containing membranes: patient variant Saposin A lacks lipid extraction capacity. The Journal of Biological Chemistry. 281: 32451-60. PMID 16905746 DOI: 10.1074/Jbc.M607281200  0.446
2006 Kolter T, Sandhoff K. Sphingolipid metabolism diseases. Biochimica Et Biophysica Acta. 1758: 2057-79. PMID 16854371 DOI: 10.1016/J.Bbamem.2006.05.027  0.411
2006 John M, Wendeler M, Heller M, Sandhoff K, Kessler H. Characterization of human saposins by NMR spectroscopy. Biochemistry. 45: 5206-16. PMID 16618109 DOI: 10.1021/bi051944  0.429
2006 Schultz-Heienbrok R, Remmel N, Klingenstein R, Rossocha M, Sandhoff K, Saenger W, Maier T. Crystallization and preliminary characterization of three different crystal forms of human saposin C heterologously expressed in Pichia pastoris. Acta Crystallographica. Section F, Structural Biology and Crystallization Communications. 62: 117-20. PMID 16511279 DOI: 10.1107/S1744309105043186  0.373
2006 Wendeler M, Werth N, Maier T, Schwarzmann G, Kolter T, Schoeniger M, Hoffmann D, Lemm T, Saenger W, Sandhoff K. The enzyme-binding region of human GM2-activator protein. The Febs Journal. 273: 982-91. PMID 16478472 DOI: 10.1111/J.1742-4658.2006.05126.X  0.47
2006 Winau F, Weber S, Sad S, de Diego J, Hoops SL, Breiden B, Sandhoff K, Brinkmann V, Kaufmann SH, Schaible UE. Apoptotic vesicles crossprime CD8 T cells and protect against tuberculosis. Immunity. 24: 105-17. PMID 16413927 DOI: 10.1016/J.Immuni.2005.12.001  0.352
2006 Sandhoff K. Sphingolipid Metabolism and Membrane Dynamics Gbm Annual Spring Meeting Mosbach 2006. 2006. DOI: 10.1240/sav_gbm_2006_m_001603  0.268
2006 Tamboli IY, Hampel H, Sandhoff K, Walter J. P4-052: Accumulation of sphingolipids increases secretion of the amyloid β-peptide by stabilization of the β-amyloid precursor protein Alzheimer's & Dementia. 2: S528-S529. DOI: 10.1016/J.Jalz.2006.05.1790  0.341
2006 Tamboli IY, Prager K, Barth E, Heneka M, Sandhoff K, Walter J. Modulation of proteolytic processing by glycosphingolipids generates amyloid β-peptide Sphingolipid Biology. 319-328. DOI: 10.1007/4-431-34200-1_25  0.203
2005 Yamashita T, Allende ML, Kalkofen DN, Werth N, Sandhoff K, Proia RL. Conditional LoxP-flanked glucosylceramide synthase allele controlling glycosphingolipid synthesis. Genesis (New York, N.Y. : 2000). 43: 175-80. PMID 16283624 DOI: 10.1002/Gene.20167  0.393
2005 Kolter T, Winau F, Schaible UE, Leippe M, Sandhoff K. Lipid-binding proteins in membrane digestion, antigen presentation, and antimicrobial defense. The Journal of Biological Chemistry. 280: 41125-8. PMID 16230343 DOI: 10.1074/Jbc.R500015200  0.424
2005 Kolter T, Sandhoff K. Principles of lysosomal membrane digestion: stimulation of sphingolipid degradation by sphingolipid activator proteins and anionic lysosomal lipids. Annual Review of Cell and Developmental Biology. 21: 81-103. PMID 16212488 DOI: 10.1146/Annurev.Cellbio.21.122303.120013  0.47
2005 Koochekpour S, Zhuang YJ, Beroukhim R, Hsieh CL, Hofer MD, Zhau HE, Hiraiwa M, Pattan DY, Ware JL, Luftig RB, Sandhoff K, Sawyers CL, Pienta KJ, Rubin MA, Vessella RL, et al. Amplification and overexpression of prosaposin in prostate cancer. Genes, Chromosomes & Cancer. 44: 351-64. PMID 16080200 DOI: 10.1002/Gcc.20249  0.339
2005 Schwarzmann G, Wendeler M, Sandhoff K. Synthesis of novel NBD-GM1 and NBD-GM2 for the transfer activity of GM2-activator protein by a FRET-based assay system. Glycobiology. 15: 1302-11. PMID 16079415 DOI: 10.1093/Glycob/Cwj018  0.46
2005 Norris AJ, Whitelegge JP, Yaghoubian A, Alattia JR, Privé GG, Toyokuni T, Sun H, Brooks MN, Panza L, Matto P, Compostella F, Remmel N, Klingenstein R, Sandhoff K, Fluharty C, et al. A novel mass spectrometric assay for the cerebroside sulfate activator protein (saposin B) and arylsulfatase A. Journal of Lipid Research. 46: 2254-64. PMID 16061947 DOI: 10.1194/Jlr.M500188-Jlr200  0.377
2005 Leyvraz C, Charles RP, Rubera I, Guitard M, Rotman S, Breiden B, Sandhoff K, Hummler E. The epidermal barrier function is dependent on the serine protease CAP1/Prss8. The Journal of Cell Biology. 170: 487-96. PMID 16061697 DOI: 10.1083/Jcb.200501038  0.388
2005 Martino S, Marconi P, Tancini B, Dolcetta D, De Angelis MG, Montanucci P, Bregola G, Sandhoff K, Bordignon C, Emiliani C, Manservigi R, Orlacchio A. A direct gene transfer strategy via brain internal capsule reverses the biochemical defect in Tay-Sachs disease. Human Molecular Genetics. 14: 2113-23. PMID 15961412 DOI: 10.1093/Hmg/Ddi216  0.396
2005 Tamboli IY, Prager K, Barth E, Heneka M, Sandhoff K, Walter J. Inhibition of glycosphingolipid biosynthesis reduces secretion of the beta-amyloid precursor protein and amyloid beta-peptide. The Journal of Biological Chemistry. 280: 28110-7. PMID 15923191 DOI: 10.1074/Jbc.M414525200  0.394
2005 Koochekpour S, Sartor O, Hiraiwa M, Lee TJ, Rayford W, Remmel N, Sandhoff K, Minokadeh A, Patten DY. Saposin C stimulates growth and invasion, activates p42/44 and SAPK/JNK signaling pathways of MAPK and upregulates uPA/uPAR expression in prostate cancer and stromal cells. Asian Journal of Andrology. 7: 147-58. PMID 15897971 DOI: 10.1111/J.1745-7262.2005.00037.X  0.367
2005 Pavlů-Pereira H, Asfaw B, Poupctová H, Ledvinová J, Sikora J, Vanier MT, Sandhoff K, Zeman J, Novotná Z, Chudoba D, Elleder M. Acid sphingomyelinase deficiency. Phenotype variability with prevalence of intermediate phenotype in a series of twenty-five Czech and Slovak patients. A multi-approach study. Journal of Inherited Metabolic Disease. 28: 203-27. PMID 15877209 DOI: 10.1007/s10545-005-5671-5  0.283
2005 Chavez JA, Holland WL, Bär J, Sandhoff K, Summers SA. Acid ceramidase overexpression prevents the inhibitory effects of saturated fatty acids on insulin signaling Journal of Biological Chemistry. 280: 20148-20153. PMID 15774472 DOI: 10.1074/Jbc.M412769200  0.372
2005 Yamashita T, Wu YP, Sandhoff R, Werth N, Mizukami H, Ellis JM, Dupree JL, Geyer R, Sandhoff K, Proia RL. Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions. Proceedings of the National Academy of Sciences of the United States of America. 102: 2725-30. PMID 15710896 DOI: 10.1073/Pnas.0407785102  0.36
2005 Sawatzki P, Damm I, Pierstorff B, Hupfer H, Sandhoff K, Kolter T. Site-specific cleavage--a model system for the identification of lipid-modified glutamate residues in proteins. Chembiochem : a European Journal of Chemical Biology. 6: 178-85. PMID 15593111 DOI: 10.1002/Cbic.200400189  0.415
2005 Sandhoff K, Kolter T. Topology of glycosphingolipid degradation. Trends in Cell Biology. 6: 98-103. PMID 15157485 DOI: 10.1016/0962-8924(96)80999-8  0.47
2004 Reichelt J, Breiden B, Sandhoff K, Magin TM. Loss of keratin 10 is accompanied by increased sebocyte proliferation and differentiation. European Journal of Cell Biology. 83: 747-59. PMID 15679119 DOI: 10.1078/0171-9335-00429  0.364
2004 Gallala H, Macheleidt O, Doering T, Schreiner V, Sandhoff K. Nitric oxide regulates synthesis of gene products involved in keratinocyte differentiation and ceramide metabolism. European Journal of Cell Biology. 83: 667-79. PMID 15679111 DOI: 10.1078/0171-9335-00425  0.409
2004 Schueler UH, Kolter T, Kaneski CR, Zirzow GC, Sandhoff K, Brady RO. Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease. Journal of Inherited Metabolic Disease. 27: 649-58. PMID 15669681 DOI: 10.1023/B:Boli.0000042959.44318.7C  0.442
2004 Kölzer M, Ferlinz K, Bartelsen O, Hoops SL, Lang F, Sandhoff K. Functional characterization of the postulated intramolecular sphingolipid activator protein domain of human acid sphingomyelinase. Biological Chemistry. 385: 1193-5. PMID 15653433 DOI: 10.1515/Bc.2004.154  0.455
2004 Koochekpour S, Sartor O, Lee TJ, Zieske A, Patten DY, Hiraiwa M, Sandhoff K, Remmel N, Minokadeh A. Prosaptide TX14A stimulates growth, migration, and invasion and activates the Raf-MEK-ERK-RSK-Elk-1 signaling pathway in prostate cancer cells. The Prostate. 61: 114-23. PMID 15305334 DOI: 10.1002/Pros.20082  0.344
2004 Gopalakrishnan MM, Grosch HW, Locatelli-Hoops S, Werth N, Smolenová E, Nettersheim M, Sandhoff K, Hasilik A. Purified recombinant human prosaposin forms oligomers that bind procathepsin D and affect its autoactivation. The Biochemical Journal. 383: 507-15. PMID 15255780 DOI: 10.1042/Bj20040175  0.406
2004 Buccoliero R, Bodennec J, Van Echten-Deckert G, Sandhoff K, Futerman AH. Phospholipid synthesis is decreased in neuronal tissue in a mouse model of Sandhoff disease Journal of Neurochemistry. 90: 80-88. PMID 15198669 DOI: 10.1111/J.1471-4159.2004.02457.X  0.421
2004 Wicklow BA, Ivanovich JL, Plews MM, Salo TJ, Noetzel MJ, Lueder GT, Cartegni L, Kaback MM, Sandhoff K, Steiner RD, Triggs-Raine BL. Severe subacute GM2 gangliosidosis caused by an apparently silent HEXA mutation (V324V) that results in aberrant splicing and reduced HEXA mRNA American Journal of Medical Genetics. 127: 158-166. PMID 15108204 DOI: 10.1002/Ajmg.A.20633  0.362
2004 Kölzer M, Werth N, Sandhoff K. Interactions of acid sphingomyelinase and lipid bilayers in the presence of the tricyclic antidepressant desipramine. Febs Letters. 559: 96-8. PMID 14960314 DOI: 10.1016/S0014-5793(04)00033-X  0.448
2004 Wendeler M, Hoernschemeyer J, John M, Werth N, Schoeniger M, Lemm T, Hartmann R, Kessler H, Sandhoff K. Expression of the GM2-activator protein in the methylotrophic yeast Pichia pastoris, purification, isotopic labeling, and biophysical characterization. Protein Expression and Purification. 34: 147-57. PMID 14766311 DOI: 10.1016/J.Pep.2003.11.010  0.451
2004 Wendeler M, Hoernschemeyer J, Hoffmann D, Kolter T, Schwarzmann G, Sandhoff K. Photoaffinity labelling of the human GM2-activator protein. Mechanistic insight into ganglioside GM2 degradation. European Journal of Biochemistry. 271: 614-27. PMID 14728689 DOI: 10.1111/J.1432-1033.2003.03964.X  0.453
2004 Winau F, Schwierzeck V, Hurwitz R, Remmel N, Sieling PA, Modlin RL, Porcelli SA, Brinkmann V, Sugita M, Sandhoff K, Kaufmann SH, Schaible UE. Saposin C is required for lipid presentation by human CD1b. Nature Immunology. 5: 169-74. PMID 14716313 DOI: 10.1038/Ni1035  0.409
2004 Winau F, Schwierzeck V, Hurwitz R, Remmel N, Sieling PA, Modlin RL, Porcelli SA, Brinkmann V, Sugita M, Sandhoff K, Kaufmann SHE, Schaible UE. Erratum: Corrigendum: Saposin C is required for lipid presentation by human CD1b Nature Immunology. 5: 344-344. DOI: 10.1038/Ni0304-344C  0.362
2004 Walter J, Prager K, Diallo M, Wahle T, Lütjohann D, Haass C, Sandhoff K, Schepers U, Tamboli I. O2-03-08 Glycosphingolipids regulate subcellular transport of the β-amyloid procursor protein and the generation of amyloid β-peptide Neurobiology of Aging. 25. DOI: 10.1016/S0197-4580(04)80126-X  0.339
2003 Diallo M, Arenz C, Schmitz K, Sandhoff K, Schepers U. Long Endogenous dsRNAs Can Induce Complete Gene Silencing in Mammalian Cells and Primary Cultures Oligonucleotides. 13: 381-392. PMID 15000829 DOI: 10.1089/154545703322617069  0.72
2003 Wendeler M, Reilaender H, Hoernschemeyer J, Schwarzmann G, Kolter T, Sandhoff K. Recombinant ganglioside GM2 synthase--expression in insect cells and enzyme assay. Methods in Enzymology. 363: 476-89. PMID 14579597 DOI: 10.1016/S0076-6879(03)01073-5  0.392
2003 Hepbildikler ST, Wendeler M, Sandhoff R, Sandhoff K. Interaction of the GM2 activator protein with sulfated and sialylated glycosphingolipids. Methods in Enzymology. 363: 207-22. PMID 14579577 DOI: 10.1016/S0076-6879(03)01053-X  0.36
2003 Diallo M, Arenz C, Schmitz K, Sandhoff K, Schepers U. RNA interference: analyzing the function of glycoproteins and glycosylating proteins in mammalian cells. Methods in Enzymology. 363: 173-90. PMID 14579575 DOI: 10.1016/S0076-6879(03)01051-6  0.735
2003 Kölzer M, Arenz C, Ferlinz K, Werth N, Schulze H, Klingenstein R, Sandhoff K. Phosphatidylinositol-3,5-Bisphosphate is a potent and selective inhibitor of acid sphingomyelinase. Biological Chemistry. 384: 1293-8. PMID 14515991 DOI: 10.1515/Bc.2003.144  0.753
2003 Pelled D, Riebeling C, Van Echten-Deckert G, Sandhoff K, Futerman AH. Reduced rates of axonal and dendritic growth in embryonic hippocampal neurones cultured from a mouse model of Sandhoff disease Neuropathology and Applied Neurobiology. 29: 341-349. PMID 12887594 DOI: 10.1046/J.1365-2990.2003.00455.X  0.344
2003 Mantipragada SB, Horváth LI, Arias HR, Schwarzmann G, Sandhoff K, Barrantes FJ, Marsh D. Lipid-protein interactions and effect of local anesthetics in acetylcholine receptor-rich membranes from Torpedo marmorata electric organ. Biochemistry. 42: 9167-75. PMID 12885251 DOI: 10.1021/Bi034485Q  0.4
2003 He X, Okino N, Dhami R, Dagan A, Gatt S, Schulze H, Sandhoff K, Schuchman EH. Purification and characterization of recombinant, human acid ceramidase. Catalytic reactions and interactions with acid sphingomyelinase. The Journal of Biological Chemistry. 278: 32978-86. PMID 12815059 DOI: 10.1074/Jbc.M301936200  0.447
2003 Sandhoff K, Kolter T. Biosynthesis and degradation of mammalian glycosphingolipids. Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 358: 847-61. PMID 12803917 DOI: 10.1098/Rstb.2003.1265  0.483
2003 Sadeghlar F, Remmel N, Breiden B, Klingenstein R, Schwarzmann G, Sandhoff K. Physiological relevance of sphingolipid activator proteins in cultured human fibroblasts. Biochimie. 85: 439-48. PMID 12770782 DOI: 10.1016/S0300-9084(03)00020-8  0.474
2003 Okino N, He X, Gatt S, Sandhoff K, Ito M, Schuchman EH. The reverse activity of human acid ceramidase. The Journal of Biological Chemistry. 278: 29948-53. PMID 12764132 DOI: 10.1074/Jbc.M303310200  0.451
2003 Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W. The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease. Journal of Molecular Biology. 328: 669-81. PMID 12706724 DOI: 10.1016/S0022-2836(03)00311-5  0.395
2003 Ozkara HA, Sandhoff K. A new point mutation (G412 to A) at the last nucleotide of exon 3 of hexosaminidase alpha-subunit gene affects splicing. Brain & Development. 25: 203-6. PMID 12689701 DOI: 10.1016/S0387-7604(02)00219-X  0.363
2003 Ozkara HA, Sandhoff K. Characterization of two Turkish beta-hexosaminidase mutations causing Tay-Sachs disease. Brain & Development. 25: 191-4. PMID 12689698 DOI: 10.1016/S0387-7604(02)00213-9  0.391
2003 Lansmann S, Schuette CG, Bartelsen O, Hoernschemeyer J, Linke T, Weisgerber J, Sandhoff K. Human acid sphingomyelinase. European Journal of Biochemistry. 270: 1076-88. PMID 12631268 DOI: 10.1046/J.1432-1033.2003.03435.X  0.428
2003 Yamashita T, Hashiramoto A, Haluzik M, Mizukami H, Beck S, Norton A, Kono M, Tsuji S, Daniotti JL, Werth N, Sandhoff R, Sandhoff K, Proia RL. Enhanced insulin sensitivity in mice lacking ganglioside GM3. Proceedings of the National Academy of Sciences of the United States of America. 100: 3445-9. PMID 12629211 DOI: 10.1073/Pnas.0635898100  0.351
2003 Betz R, Sandhoff K, Fischer KD, van Echten-Deckert G. Detection and identification of Vav1 protein in primary cultured murine cerebellar neurons and in neuroblastoma cells (SH-SY5Y and Neuro-2a). Neuroscience Letters. 339: 37-40. PMID 12618295 DOI: 10.1016/S0304-3940(02)01457-X  0.399
2003 Wendeler M, Lemm T, Weisgerber J, Hoernschemeyer J, Bartelsen O, Schepers U, Sandhoff K. Expression of recombinant human GM2-activator protein in insect cells: purification and characterization by mass spectrometry. Protein Expression and Purification. 27: 259-66. PMID 12597885 DOI: 10.1016/S1046-5928(02)00599-5  0.413
2003 Elsen L, Betz R, Schwarzmann G, Sandhoff K, van Echten-Deckert G. Identification of ceramide binding proteins in neuronal cells: a critical point of view. Neurochemical Research. 27: 717-27. PMID 12374206 DOI: 10.1023/A:1020288403626  0.425
2003 Doering T, Brade H, Sandhoff K. Sphingolipid metabolism during epidermal barrier development in mice. Journal of Lipid Research. 43: 1727-33. PMID 12364557 DOI: 10.1194/Jlr.M200208-Jlr200  0.397
2003 Sawatzki P, Mikeska T, Sandhoff K, Nieger M, Kolter T. 6(S)-Methyl-3(S)-(1-methylethyl)piperazin-2-one Acta Crystallographica Section E Structure Reports Online. 59: o171-o173. DOI: 10.1107/S1600536803000862  0.345
2002 Nätzker S, Heinemann T, Figueroa-Perez S, Schnieders B, Schmidt RR, Sandhoff K, van Echten-Deckert G. Cis-4-methylsphingosine phosphate induces apoptosis in neuroblastoma cells by opposite effects on p38 and ERK mitogen-activated protein kinases. Biological Chemistry. 383: 1885-94. PMID 12553725 DOI: 10.1515/Bc.2002.212  0.502
2002 Martino S, Cavalieri C, Emiliani C, Dolcetta D, Cusella De Angelis MG, Chigorno V, Severini GM, Sandhoff K, Bordignon C, Sonnino S, Orlacchio A. Restoration of the GM2 ganglioside metabolism in bone marrow-derived stromal cells from Tay-Sachs disease animal model. Neurochemical Research. 27: 793-800. PMID 12374215 DOI: 10.1023/A:1020256924099  0.36
2002 Jeyakumar M, Smith D, Eliott-Smith E, Cortina-Borja M, Reinkensmeier G, Butters TD, Lemm T, Sandhoff K, Perry VH, Dwek RA, Platt FM. An inducible mouse model of late onset Tay-Sachs disease. Neurobiology of Disease. 10: 201-10. PMID 12270683 DOI: 10.1006/Nbdi.2002.0511  0.265
2002 Macheleidt O, Kaiser HW, Sandhoff K. Deficiency of epidermal protein-bound omega-hydroxyceramides in atopic dermatitis. The Journal of Investigative Dermatology. 119: 166-73. PMID 12164940 DOI: 10.1046/J.1523-1747.2002.01833.X  0.405
2002 Laurent-Matha V, Lucas A, Huttler S, Sandhoff K, Garcia M, Rochefort H. Procathepsin D interacts with prosaposin in cancer cells but its internalization is not mediated by LDL receptor-related protein. Experimental Cell Research. 277: 210-9. PMID 12083803 DOI: 10.1006/Excr.2002.5556  0.37
2002 Kolter T, Proia RL, Sandhoff K. Combinatorial ganglioside biosynthesis. The Journal of Biological Chemistry. 277: 25859-62. PMID 12011101 DOI: 10.1074/Jbc.R200001200  0.393
2002 Mizukami H, Mi Y, Wada R, Kono M, Yamashita T, Liu Y, Werth N, Sandhoff R, Sandhoff K, Proia RL. Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage. The Journal of Clinical Investigation. 109: 1215-21. PMID 11994410 DOI: 10.1172/Jci14530  0.394
2002 Schueler U, Kaneski C, Murray G, Sandhoff K, Brady RO. Uptake of mannose-terminal glucocerebrosidase in cultured human cholinergic and dopaminergic neuron cell lines. Neurochemical Research. 27: 325-30. PMID 11958535 DOI: 10.1023/A:1014915430398  0.405
2002 Li CM, Park JH, Simonaro CM, He X, Gordon RE, Friedman AH, Ehleiter D, Paris F, Manova K, Hepbildikler S, Fuks Z, Sandhoff K, Kolesnick R, Schuchman EH, Hepbiloikler S. Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotes. Genomics. 79: 218-24. PMID 11829492 DOI: 10.1006/Geno.2002.6686  0.442
2002 Sandhoff K. The GM2-gangliosidoses and the elucidation of the beta-hexosaminidase system. Advances in Genetics. 44: 67-91. PMID 11597000 DOI: 10.1016/S0065-2660(01)44072-7  0.426
2001 Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K. Physiological substrates for human lysosomal beta -hexosaminidase S. The Journal of Biological Chemistry. 277: 2562-72. PMID 11707436 DOI: 10.1074/Jbc.M105457200  0.418
2001 Ferlinz K, Kopal G, Bernardo K, Linke T, Bär J, Breiden B, Neumann U, Lang F, Schuchman EH, Sandhoff K. Human acid ceramidase: Processing, glycosylation, and lysosomal targeting Journal of Biological Chemistry. 276: 35352-35360. PMID 11451951 DOI: 10.1074/Jbc.M103066200  0.44
2001 Schuette CG, Weisgerber J, Sandhoff K. Complete analysis of the glycosylation and disulfide bond pattern of human beta-hexosaminidase B by MALDI-MS. Glycobiology. 11: 549-56. PMID 11447134 DOI: 10.1093/Glycob/11.7.549  0.413
2001 Schuette CG, Pierstorff B, Huettler S, Sandhoff K. Sphingolipid activator proteins: proteins with complex functions in lipid degradation and skin biogenesis. Glycobiology. 11: 81R-90R. PMID 11445546 DOI: 10.1093/Glycob/11.6.81R  0.449
2001 Panigone S, Bergomas R, Fontanella E, Prinetti A, Sandhoff K, Grabowski GA, Delia D. Up-regulation of prosaposin by the retinoid HPR and its effect on ceramide production and integrin receptors. Faseb Journal : Official Publication of the Federation of American Societies For Experimental Biology. 15: 1475-7. PMID 11387262 DOI: 10.1096/fj.00-0531fje  0.199
2001 Linke T, Wilkening G, Lansmann S, Moczall H, Bartelsen O, Weisgerber J, Sandhoff K. Stimulation of acid sphingomyelinase activity by lysosomal lipids and sphingolipid activator proteins. Biological Chemistry. 382: 283-90. PMID 11308026 DOI: 10.1515/Bc.2001.035  0.473
2001 Grassme H, Jekle A, Riehle A, Schwarz H, Berger J, Sandhoff K, Kolesnick R, Gulbins E. CD95 signaling via ceramide-rich membrane rafts. The Journal of Biological Chemistry. 276: 20589-96. PMID 11279185 DOI: 10.1074/Jbc.M101207200  0.339
2001 Werth N, Schuette CG, Wilkening G, Lemm T, Sandhoff K. Degradation of membrane-bound ganglioside GM2 by beta -hexosaminidase A. Stimulation by GM2 activator protein and lysosomal lipids. The Journal of Biological Chemistry. 276: 12685-90. PMID 11278374 DOI: 10.1074/Jbc.M007970200  0.467
2001 Bär J, Linke T, Ferlinz K, Neumann U, Schuchman EH, Sandhoff K. Molecular analysis of acid ceramidase deficiency in patients with Farber disease Human Mutation. 17: 199-209. PMID 11241842 DOI: 10.1002/Humu.5  0.406
2001 Kolter T, Magin TM, Sandhoff K. Biomolecule function: no reliable prediction from cell culture. Traffic (Copenhagen, Denmark). 1: 803-4. PMID 11208070 DOI: 10.1034/J.1600-0854.2000.011007.X  0.323
2001 Kawai H, Allende ML, Wada R, Kono M, Sango K, Deng C, Miyakawa T, Crawley JN, Werth N, Bierfreund U, Sandhoff K, Proia RL. Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures. The Journal of Biological Chemistry. 276: 6885-8. PMID 11133999 DOI: 10.1074/Jbc.C000847200  0.385
2001 Linke T, Wilkening G, Sadeghlar F, Mozcall H, Bernardo K, Schuchman E, Sandhoff K. Interfacial Regulation of Acid Ceramidase Activity: Stimulation of ceramide degradation by lysosomal lipids and sphingolipid activator proteins Journal of Biological Chemistry. 276: 5760-5768. PMID 11104761 DOI: 10.1074/Jbc.M006846200  0.472
2001 Potratz A, Hüttler S, Bierfreund U, Proia RL, Suzuki K, Sandhoff K. Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency. Biochimica Et Biophysica Acta. 1502: 391-7. PMID 11068181 DOI: 10.1016/S0925-4439(00)00063-6  0.415
2001 Peter-Katalinic J, Sandhoff K. Highlight: Glycobiology Biological Chemistry. 382. DOI: 10.1515/BC.2001.021  0.181
2000 Hoffmann P, Sandhoff K, Marsh D. Comparative dynamics and location of chain spin-labelled sphingomyelin and phosphatidylcholine in dimyristoyl phosphatidylcholine membranes studied by EPR spectroscopy. Biochimica Et Biophysica Acta. 1468: 359-66. PMID 11018679 DOI: 10.1016/S0005-2736(00)00276-5  0.359
2000 Schepers U, Lemm T, Herzog V, Sandhoff K. Characterization of regulatory elements in the 5'-flanking region of the GM2 activator gene Biological Chemistry. 381: 531-544. PMID 10987359 DOI: 10.1515/Bc.2000.069  0.381
2000 Strelow A, Bernardo K, Adam-Klages S, Linke T, Sandhoff K, Krönke M, Adam D. Overexpression of acid ceramidase protects from tumor necrosis factor-induced cell death. The Journal of Experimental Medicine. 192: 601-12. PMID 10974027 DOI: 10.1084/Jem.192.5.601  0.392
2000 Wilkening G, Linke T, Uhlhorn-Dierks G, Sandhoff K. Degradation of membrane-bound ganglioside GM1. Stimulation by bis(monoacylglycero)phosphate and the activator proteins SAP-B and GM2-AP. The Journal of Biological Chemistry. 275: 35814-9. PMID 10942779 DOI: 10.1074/Jbc.M006568200  0.477
2000 Sadeghlar F, Sandhoff K, van Echten-Deckert G. Cell type specific localization of sphingomyelin biosynthesis. Febs Letters. 478: 9-12. PMID 10922460 DOI: 10.1016/S0014-5793(00)01818-4  0.395
2000 Kolter T, Doering T, Wilkening G, Werth N, Sandhoff K. Recent advances in the biochemistry of glycosphingolipid metabolism. Biochemical Society Transactions. 27: 409-15. PMID 10917612 DOI: 10.1042/Bst0270409  0.339
2000 Kase R, Bierfreund U, Klein A, Kolter T, Utsumi K, Itoha K, Sandhoff K, Sakuraba H. Characterization of two alpha-galactosidase mutants (Q279E and R301Q) found in an atypical variant of Fabry disease. Biochimica Et Biophysica Acta. 1501: 227-35. PMID 10838196 DOI: 10.1016/S0925-4439(00)00024-7  0.402
2000 Huwiler A, Kolter T, Pfeilschifter J, Sandhoff K. Physiology and pathophysiology of sphingolipid metabolism and signaling. Biochimica Et Biophysica Acta. 1485: 63-99. PMID 10832090 DOI: 10.1016/S1388-1981(00)00042-1  0.336
2000 Romiti E, Vasta V, Meacci E, Farnararo M, Linke T, Ferlinz K, Sandhoff K, Bruni P. Characterization of sphingomyelinase activity released by thrombin-stimulated platelets. Molecular and Cellular Biochemistry. 205: 75-81. PMID 10821424 DOI: 10.1023/A:1007041329052  0.395
2000 Bernardo K, Krut O, Wiegmann K, Kreder D, Micheli M, Schäfer R, Sickman A, Schmidt WE, Schröder JM, Meyer HE, Sandhoff K, Krönke M. Purification and characterization of a magnesium-dependent neutral sphingomyelinase from bovine brain. The Journal of Biological Chemistry. 275: 7641-7. PMID 10713073 DOI: 10.1074/JBC.275.11.7641  0.343
2000 Wrobe D, Henseler M, Huettler S, Pascual Pascual SI, Chabas A, Sandhoff K. A non-glycosylated and functionally deficient mutant (N215H) of the sphingolipid activator protein B (SAP-B) in a novel case of metachromatic leukodystrophy (MLD). Journal of Inherited Metabolic Disease. 23: 63-76. PMID 10682309 DOI: 10.1023/A:1005603014401  0.432
2000 Wendeler M, Kolter T, Sandhoff K. Pathology of Glycosphingolipid Metabolism: The Molecular and Cellular Basis of Neurodegenerative Disease The Neuroscientist. 6: 285-296. DOI: 10.1177/107385840000600411  0.408
2000 Ozkara HA, Sandhoff K. Characterisation of the effect of a 12 bp deletion mutation in exon 10 and a point mutation in exon 12 on beta-hexosaminidase alpha-subunit gene by site-directed mutagenesis Biochemical Society Transactions. 28: A441-A441. DOI: 10.1042/Bst028A440C  0.333
1999 Ferlinz K, Linke T, Bartelsen O, Weiler M, Sandhoff K. Stimulation of lysosomal sphingomyelin degradation by sphingolipid activator proteins. Chemistry and Physics of Lipids. 102: 35-43. PMID 11001559 DOI: 10.1016/S0009-3084(99)00073-0  0.471
1999 Li CM, Park JH, He X, Levy B, Chen F, Arai K, Adler DA, Disteche CM, Koch J, Sandhoff K, Schuchman EH. The human acid ceramidase gene (ASAH): Structure, chromosomal location, mutation analysis, and expression Genomics. 62: 223-231. PMID 10610716 DOI: 10.1006/Geno.1999.5940  0.397
1999 Möbius W, Herzog V, Sandhoff K, Schwarzmann G. Gangliosides are transported from the plasma membrane to intralysosomal membranes as revealed by immuno-electron microscopy Bioscience Reports. 19: 307-316. PMID 10589996 DOI: 10.1023/A:1020502525572  0.381
1999 Knoll F, Kolter T, Sandhoff K. Sphingolipid photoaffinity labels. Methods in Enzymology. 311: 568-600. PMID 10563350 DOI: 10.1016/S0076-6879(00)11106-1  0.44
1999 Bierfreund U, Kolter T, Sandhoff K. Sphingolipid hydrolases and activator proteins. Methods in Enzymology. 311: 255-76. PMID 10563332 DOI: 10.1016/S0076-6879(00)11088-2  0.493
1999 Linke T, Lansmann S, Sandhoff K. Purification of acid ceramidase from human placenta. Methods in Enzymology. 311: 201-7. PMID 10563326 DOI: 10.1016/S0076-6879(00)11082-1  0.473
1999 Lansmann S, Bartelsen O, Sandhoff K. Purification and characterization of recombinant human acid sphingomyelinase expressed in insect Sf21 cells. Methods in Enzymology. 311: 149-56. PMID 10563319 DOI: 10.1016/S0076-6879(00)11075-4  0.473
1999 Pohlentz G, Kaes C, Sandhoff K. In vitro assays for enzymes of ganglioside synthesis. Methods in Enzymology. 311: 82-94. PMID 10563313 DOI: 10.1016/S0076-6879(00)11069-9  0.461
1999 Schuette CG, Doering T, Kolter T, Sandhoff K. The glycosphingolipidoses-from disease to basic principles of metabolism. Biological Chemistry. 380: 759-66. PMID 10494825 DOI: 10.1515/Bc.1999.096  0.408
1999 Reichelt J, Doering T, Schnetz E, Fartasch M, Sandhoff K, Magin AM. Normal ultrastructure, but altered stratum corneum lipid and protein composition in a mouse model for epidermolytic hyperkeratosis. The Journal of Investigative Dermatology. 113: 329-34. PMID 10469329 DOI: 10.1046/J.1523-1747.1999.00702.X  0.397
1999 Yamashita T, Wada R, Sasaki T, Deng C, Bierfreund U, Sandhoff K, Proia RL. A vital role for glycosphingolipid synthesis during development and differentiation Proceedings of the National Academy of Sciences of the United States of America. 96: 9142-9147. PMID 10430909 DOI: 10.1073/Pnas.96.16.9142  0.384
1999 Möbius W, Herzog V, Sandhoff K, Schwarzmann G. Intracellular distribution of a biotin-labeled ganglioside, GM1, by immunoelectron microscopy after endocytosis in fibroblasts Journal of Histochemistry and Cytochemistry. 47: 1005-1014. PMID 10424884 DOI: 10.1177/002215549904700804  0.405
1999 Heinemann T, Bulwin GC, Randall J, Schnieders B, Sandhoff K, Volk HD, Milford E, Gullans SR, Utku N. Genomic organization of the gene coding for TIRC7, a novel membrane protein essential for T cell activation. Genomics. 57: 398-406. PMID 10329006 DOI: 10.1006/Geno.1999.5751  0.388
1999 Giehl A, Lemm T, Bartelsen O, Sandhoff K, Blume A. Interaction of the GM2-activator protein with phospholipid-ganglioside bilayer membranes and with monolayers at the air-water interface. European Journal of Biochemistry. 261: 650-8. PMID 10215880 DOI: 10.1046/J.1432-1327.1999.00302.X  0.411
1999 Doering T, Proia RL, Sandhoff K. Accumulation of protein-bound epidermal glucosylceramides in beta-glucocerebrosidase deficient type 2 Gaucher mice. Febs Letters. 447: 167-70. PMID 10214939 DOI: 10.1016/S0014-5793(99)00274-4  0.448
1999 Utku N, Bulwin GC, Beinke S, Heinemann T, Beato F, Randall J, Schnieders B, Sandhoff K, Volk HD, Milford E, Gullans SR. The human homolog of Drosophila cornichon protein is differentially expressed in alloactivated T-cells. Biochimica Et Biophysica Acta. 1449: 203-10. PMID 10209299 DOI: 10.1016/S0167-4889(99)00026-9  0.39
1999 Doering T, Holleran WM, Potratz A, Vielhaber G, Elias PM, Suzuki K, Sandhoff K. Sphingolipid activator proteins are required for epidermal permeability barrier formation. The Journal of Biological Chemistry. 274: 11038-45. PMID 10196186 DOI: 10.1074/Jbc.274.16.11038  0.467
1999 Liu Y, Wada R, Kawai H, Sango K, Deng C, Tai T, McDonald MP, Araujo K, Crawley JN, Bierfreund U, Sandhoff K, Suzuki K, Proia RL. A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder. The Journal of Clinical Investigation. 103: 497-505. PMID 10021458 DOI: 10.1172/Jci5542  0.376
1999 Bierfreund U, Lemm T, Hoffmann A, Uhlhorn-Dierks G, Childs RA, Yuen CT, Feizi T, Sandhoff K. Recombinant GM2-activator protein stimulates in vivo degradation of GA2 in GM2 gangliosidosis AB variant fibroblasts but exhibits no detectable binding of GA2 in an in vitro assay. Neurochemical Research. 24: 295-300. PMID 9972878 DOI: 10.1023/A:1022526407855  0.387
1999 Sakuraba H, Itoh K, Shimmoto M, Utsumi K, Kase R, Hashimoto Y, Ozawa T, Ohwada Y, Imataka G, Eguchi M, Furukawa T, Schepers U, Sandhoff K. GM2 gangliosidosis AB variant: clinical and biochemical studies of a Japanese patient. Neurology. 52: 372-7. PMID 9932959 DOI: 10.1212/Wnl.52.2.372  0.321
1999 Bektas M, Dullin Y, Wieder T, Kolter T, Sandhoff K, Brossmer R, Ihrig P, Orfanos CE, Geilen CC. Induction of apoptosis by synthetic ceramide analogues in the human keratinocyte cell line HaCaT. Experimental Dermatology. 7: 342-9. PMID 9858137 DOI: 10.1111/J.1600-0625.1998.Tb00334.X  0.386
1999 Kolter T, Sandhoff K. Sphingolipide – ihre Stoffwechselwege und die Pathobiochemie neurodegenerativer Erkrankungen Angewandte Chemie. 111: 1632-1670. DOI: 10.1002/(Sici)1521-3757(19990601)111:11<1632::Aid-Ange1632>3.0.Co;2-A  0.322
1998 Brenner B, Ferlinz K, Grassmé H, Weller M, Koppenhoefer U, Dichgans J, Sandhoff K, Lang F, Gulbins E. Fas/CD95/Apo-I activates the acidic sphingomyelinase via caspases. Cell Death and Differentiation. 5: 29-37. PMID 10200443 DOI: 10.1038/Sj.Cdd.4400307  0.365
1998 Wilkening G, Linke T, Sandhoff K. Lysosomal degradation on vesicular membrane surfaces. Enhanced glucosylceramide degradation by lysosomal anionic lipids and activators. The Journal of Biological Chemistry. 273: 30271-8. PMID 9804787 DOI: 10.1074/Jbc.273.46.30271  0.457
1998 Bartelsen O, Lansmann S, Nettersheim M, Lemm T, Ferlinz K, Sandhoff K. Expression of recombinant human acid sphingomyelinase in insect Sf21 cells: purification, processing and enzymatic characterization. Journal of Biotechnology. 63: 29-40. PMID 9764481 DOI: 10.1016/S0168-1656(98)00070-4  0.433
1998 Kolter T, Sandhoff K. Glycosphingolipid degradation and animal models of GM2-gangliosidoses. Journal of Inherited Metabolic Disease. 21: 548-63. PMID 9728335 DOI: 10.1023/A:1005419122018  0.364
1998 Hiesberger T, Hüttler S, Rohlmann A, Schneider W, Sandhoff K, Herz J. Cellular uptake of saposin (SAP) precursor and lysosomal delivery by the low density lipoprotein receptor-related protein (LRP). The Embo Journal. 17: 4617-25. PMID 9707421 DOI: 10.1093/Emboj/17.16.4617  0.384
1998 Landrieu P, Blanche S, Vanier MT, Metral S, Husson B, Sandhoff K, Fischer A. Bone marrow transplantation in metachromatic leukodystrophy caused by saposin-B deficiency: a case report with a 3-year follow-up period. The Journal of Pediatrics. 133: 129-32. PMID 9672525 DOI: 10.1016/S0022-3476(98)70191-X  0.324
1998 Sandhoff K, Kolter T, Van Echten-Deckert G. Sphingolipid metabolism. Sphingoid analogs, sphingolipid activator proteins, and the pathology of the cell. Annals of the New York Academy of Sciences. 845: 139-51. PMID 9668348 DOI: 10.1111/J.1749-6632.1998.TB09667.X  0.378
1998 Li CM, Hong SB, Kopal G, He X, Linke T, Hou WS, Koch J, Gatt S, Sandhoff K, Schuchman EH. Cloning and characterization of the full-length cDNA and genomic sequences encoding murine acid ceramidase Genomics. 50: 267-274. PMID 9653654 DOI: 10.1006/Geno.1998.5334  0.388
1998 Norflus F, Tifft CJ, McDonald MP, Goldstein G, Crawley JN, Hoffmann A, Sandhoff K, Suzuki K, Proia RL. Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice. The Journal of Clinical Investigation. 101: 1881-8. PMID 9576752 DOI: 10.1172/Jci2127  0.381
1998 Schütte CG, Lemm T, Glombitza GJ, Sandhoff K. Complete localization of disulfide bonds in GM2 activator protein. Protein Science : a Publication of the Protein Society. 7: 1039-45. PMID 9568910 DOI: 10.1002/Pro.5560070421  0.389
1998 Breen KC, Potratz A, Georgopoulou N, Sandhoff K. The generation and characterization of a rat neural cell line overexpressing the alpha2,6(N) sialyltransferase. Glycoconjugate Journal. 15: 199-202. PMID 9557882 DOI: 10.1023/A:1006980608983  0.424
1998 Liu Y, Suzuki K, Reed JD, Grinberg A, Westphal H, Hoffmann A, Döring T, Sandhoff K, Proia RL. Mice with type 2 and 3 gaucher disease point mutations generated by a single insertion mutagenesis procedure (SIMP) Proceedings of the National Academy of Sciences of the United States of America. 95: 2503-2508. PMID 9482915 DOI: 10.1073/Pnas.95.5.2503  0.38
1998 Kolter T, Sandhoff K. Recent advances in the biochemistry of sphingolipidoses. Brain Pathology (Zurich, Switzerland). 8: 79-100. PMID 9458169 DOI: 10.1111/J.1750-3639.1998.Tb00138.X  0.471
1998 Sandhoff K, Kolter T. Biochemistry of glycosphingolipid degradation. Clinica Chimica Acta; International Journal of Clinical Chemistry. 266: 51-61. PMID 9435988 DOI: 10.1016/S0009-8981(97)00166-6  0.468
1998 Van Echten-Deckert G, Giannis A, Schwarz A, Futerman AH, Sandhoff K. 1-Methylthiodihydroceramide, a novel analog of dihydroceramide, stimulates sphinganine degradation resulting in decreased de novo sphingolipid biosynthesis Journal of Biological Chemistry. 273: 1184-1191. PMID 9422785 DOI: 10.1074/Jbc.273.2.1184  0.659
1998 Kolter T, Sandhoff K. Sialic acids--why always alpha-linked? Glycobiology. 7: vii-ix. PMID 9363428 DOI: 10.1093/Glycob/7.7.873  0.409
1998 Kolter T, Sandhoff K. Enzymology of Lysosomal Glycolipid Catabolism. Trends in Glycoscience and Glycotechnology. 10: 455-468. DOI: 10.4052/Tigg.10.455  0.415
1998 Sandhoff K, Kolter T. Processing of sphingolipid activator proteins and the topology of lysosomal digestion. Acta Biochimica Polonica. 45: 373-384. DOI: 10.18388/Abp.1998_4232  0.48
1998 Uhlhorn-Dierks G, Kolter T, Sandhoff K. Wie werden Schwefelsäureester in der Natur gespalten? Eine neuartige posttranslationale Proteinmodifikation bei Sulfatasen Angewandte Chemie. 110: 2591-2594. DOI: 10.1002/(Sici)1521-3757(19980918)110:18<2591::Aid-Ange2591>3.0.Co;2-D  0.315
1997 Grassmé H, Gulbins E, Brenner B, Ferlinz K, Sandhoff K, Harzer K, Lang F, Meyer TF. Acidic sphingomyelinase mediates entry of N. gonorrhoeae into nonphagocytic cells. Cell. 91: 605-15. PMID 9393854 DOI: 10.1016/S0092-8674(00)80448-1  0.406
1997 Michel C, Van Echten-Deckert G, Rother J, Sandhoff K, Wang E, Merrill AH. Characterization of ceramide synthesis. A dihydroceramide desaturase introduces the 4,5-trans-double bond of sphingosine at the level of dihydroceramide Journal of Biological Chemistry. 272: 22432-22437. PMID 9312549 DOI: 10.1074/Jbc.272.36.22432  0.337
1997 Wieder T, Geilen CC, Kolter T, Sadeghlar F, Sandhoff K, Brossmer R, Ihrig P, Perry D, Orfanos CE, Hannun YA. Bcl-2 antagonizes apoptotic cell death induced by two new ceramide analogues. Febs Letters. 411: 260-4. PMID 9271217 DOI: 10.1016/S0014-5793(97)00717-5  0.368
1997 Liu Y, Hoffmann A, Grinberg A, Westphal H, McDonald MP, Miller KM, Crawley JN, Sandhoff K, Suzuki K, Proia RL. Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment. Proceedings of the National Academy of Sciences of the United States of America. 94: 8138-43. PMID 9223328 DOI: 10.1073/Pnas.94.15.8138  0.418
1997 van Echten-Deckert G, Zschoche A, Bär T, Schmidt RR, Raths A, Heinemann T, Sandhoff K. cis-4-Methylsphingosine decreases sphingolipid biosynthesis by specifically interfering with serine palmitoyltransferase activity in primary cultured neurons. The Journal of Biological Chemistry. 272: 15825-33. PMID 9188480 DOI: 10.1074/Jbc.272.25.15825  0.397
1997 Albrecht B, Pohlentz G, Sandhoff K, Schwarzmann G. Synthesis and mass spectrometric characterization of digoxigenin and biotin labeled ganglioside GM1 and their uptake by and metabolism in cultured cells Chemistry and Physics of Lipids. 86: 37-50. PMID 9149388 DOI: 10.1016/S0009-3084(97)02658-3  0.403
1997 Pennybacker M, Schuette CG, Liessem B, Hepbildikler ST, Kopetka JA, Ellis MR, Myerowitz R, Sandhoff K, Proia RL. Evidence for the involvement of Glu-355 in the catalytic action of human beta-hexosaminidase B. The Journal of Biological Chemistry. 272: 8002-6. PMID 9065471 DOI: 10.1074/Jbc.272.12.8002  0.393
1997 Ferlinz K, Hurwitz R, Moczall H, Lansmann S, Schuchman EH, Sandhoff K. Functional characterization of the N-glycosylation sites of human acid sphingomyelinase by site-directed mutagenesis European Journal of Biochemistry. 243: 511-517. PMID 9030779 DOI: 10.1111/J.1432-1033.1997.511_1A.X  0.4
1997 Glombitza GJ, Becker E, Kaiser HW, Sandhoff K. Biosynthesis, processing, and intracellular transport of GM2 activator protein in human epidermal keratinocytes. The lysosomal targeting of the GM2 activator is independent of a mannose-6-phosphate signal. The Journal of Biological Chemistry. 272: 5199-207. PMID 9030589 DOI: 10.1074/Jbc.272.8.5199  0.436
1997 Lansmann S, Ferlinz K, Hurwitz R, Bartelsen O, Glombitza G, Sandhoff K. Purification of acid sphingomyelinase from human placenta: characterization and N-terminal sequence. Febs Letters. 399: 227-31. PMID 8985151 DOI: 10.1016/S0014-5793(96)01331-2  0.43
1997 Vielhaber G, Hurwitz R, Sandhoff K. Biosynthesis, processing, and targeting of sphingolipid activator protein (SAP )precursor in cultured human fibroblasts. Mannose 6-phosphate receptor-independent endocytosis of SAP precursor. The Journal of Biological Chemistry. 271: 32438-46. PMID 8943309 DOI: 10.1074/Jbc.271.50.32438  0.458
1997 Müller E, Giehl A, Schwarzmann G, Sandhoff K, Blume A. Oriented 1,2-dimyristoyl-sn-glycero-3-phosphorylcholine/ganglioside membranes: a Fourier transform infrared attenuated total reflection spectroscopic study. Band assignments; orientational, hydrational, and phase behavior; and effects of Ca2+ binding. Biophysical Journal. 71: 1400-21. PMID 8874015 DOI: 10.1016/S0006-3495(96)79342-6  0.383
1997 Schick A, Schwarzmann G, Kolter T, Sandhoff K. Synthesis of tritium labelled phosphonate analogues of sphinganine-1-phosphate Journal of Labelled Compounds and Radiopharmaceuticals. 39: 441-451. DOI: 10.1002/(Sici)1099-1344(199705)39:5<441::Aid-Jlcr988>3.0.Co;2-D  0.382
1996 Koch J, Gärtner S, Li CM, Quintern LE, Bernardo K, Levran O, Schnabel D, Desnick RJ, Schuchman EH, Sandhoff K. Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase: Identification of the first molecular lesion causing farber disease Journal of Biological Chemistry. 271: 33110-33115. PMID 8955159 DOI: 10.1074/Jbc.271.51.33110  0.428
1996 Sango K, McDonald MP, Crawley JN, Mack ML, Tifft CJ, Skop E, Starr CM, Hoffmann A, Sandhoff K, Suzuki K, Proia RL. Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis. Nature Genetics. 14: 348-52. PMID 8896570 DOI: 10.1038/Ng1196-348  0.367
1996 Kase R, Bierfreund U, Klein A, Kolter T, Itoh K, Suzuki M, Hashimoto Y, Sandhoff K, Sakuraba H. Only sphingolipid activator protein B (SAP-B or saposin B) stimulates the degradation of globotriaosylceramide by recombinant human lysosomal alpha-galactosidase in a detergent-free liposomal system. Febs Letters. 393: 74-6. PMID 8804427 DOI: 10.1016/0014-5793(96)00863-0  0.402
1996 Arni S, Ilangumaran S, van Echten-Deckert G, Sandhoff K, Poincelet M, Briol A, Rungger-Brändle E, Hoessli DC. Differential regulation of Src-family protein tyrosine kinases in GPI domains of T lymphocyte plasma membranes. Biochemical and Biophysical Research Communications. 225: 801-7. PMID 8780693 DOI: 10.1006/Bbrc.1996.1254  0.399
1996 Fujita N, Suzuki K, Vanier MT, Popko B, Maeda N, Klein A, Henseler M, Sandhoff K, Nakayasu H, Suzuki K. Targeted disruption of the mouse sphingolipid activator protein gene: a complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids. Human Molecular Genetics. 5: 711-25. PMID 8776585 DOI: 10.1093/Hmg/5.6.711  0.439
1996 Zacharias C, Van Echten-Deckert G, Wang E, Merrill AH, Sandhoff K. The effect of fumonisin B1 on developing chick embryos: Correlation between de novo sphingolipid biosynthesis and gross morphological changes Glycoconjugate Journal. 13: 167-175. PMID 8737241 DOI: 10.1007/Bf00731491  0.373
1996 Pennybacker M, Liessem B, Moczall H, Tifft CJ, Sandhoff K, Proia RL. Identification of domains in human beta-hexosaminidase that determine substrate specificity. The Journal of Biological Chemistry. 271: 17377-82. PMID 8663217 DOI: 10.1074/Jbc.271.29.17377  0.395
1996 Henseler M, Klein A, Glombitza GJ, Suziki K, Sandhoff K. Expression of the three alternative forms of the sphingolipid activator protein precursor in baby hamster kidney cells and functional assays in a cell culture system. The Journal of Biological Chemistry. 271: 8416-23. PMID 8626540 DOI: 10.1074/Jbc.271.14.8416  0.467
1996 Hoffmann A, Okuda Y, Grinberg A, McDonald MP, Sandhoff K, Crawley JN, Westphal H, Proia RL. Proceedings of the Japanese Society of Animal Models For Human Diseases. 12: 52-52. DOI: 10.1538/expanim1992.12.52  0.266
1996 Suzuki K, Hoffmann A, Sandhoff K, Starr CM, Skop E, Sango K, Proja RL. MICE TOTALLY DEFICIENT IN β-HEXOSAMINIDASE α AND β SUBUNITS SHOW AN ADDITIONAL MUCOPOLYSACCHARIDOSIS PHENOTYPE Journal of Neuropathology and Experimental Neurology. 55: 620. DOI: 10.1097/00005072-199605000-00073  0.221
1996 Kolter T, Sandhoff K. Inhibitors of glycosphingolipid biosythesis Chemical Society Reviews. 25: 371. DOI: 10.1039/Cs9962500371  0.327
1996 Schick A, Kolter T, Giannis A, Sandhoff K. Synthesis of phosphoramide analogues of sphinganine-1-phosphate Tetrahedron. 52: 2945-2956. DOI: 10.1016/0040-4020(95)01119-6  0.669
1995 Haltia M, Tyynelä J, Baumann M, Henseler M, Sandhoff K. Immunological studies on sphingolipid activator proteins in the neuronal ceroid-lipofuscinoses. Gerontology. 41: 239-48. PMID 8821335 DOI: 10.1159/000213746  0.3
1995 Bernardo K, Hurwitz R, Zenk T, Desnick RJ, Ferlinz K, Schuchman EH, Sandhoff K. Purification, characterization, and biosynthesis of human acid ceramidase Journal of Biological Chemistry. 270: 11098-11102. PMID 7744740 DOI: 10.1074/Jbc.270.19.11098  0.446
1995 Horinouchi K, Erlich S, Perl DP, Ferlinz K, Bisgaier CL, Sandhoff K, Desnick RJ, Stewart CL, Schuchman EH. Acid sphingomyelinase deficient mice: a model of types A and B Niemann-Pick disease. Nature Genetics. 10: 288-93. PMID 7670466 DOI: 10.1038/Ng0795-288  0.407
1995 Tyynelä J, Baumann M, Henseler M, Sandhoff K, Haltia M. Sphingolipid activator proteins (SAPs) are stored together with glycosphingolipids in the infantile neuronal ceroid-lipofuscinosis (INCL). American Journal of Medical Genetics. 57: 294-7. PMID 7668348 DOI: 10.1002/Ajmg.1320570236  0.423
1995 Tyynelä J, Baumann M, Henseler M, Sandhoff K, Haltia M. Sphingolipid activator proteins in the neuronal ceroid-lipofuscinoses: an immunological study. Acta Neuropathologica. 89: 391-8. PMID 7618436 DOI: 10.1007/Bf00307641  0.414
1995 Liessem B, Glombitza GJ, Knoll F, Lehmann J, Kellermann J, Lottspeich F, Sandhoff K. Photoaffinity labeling of human lysosomal beta-hexosaminidase B. Identification of Glu-355 at the substrate binding site. The Journal of Biological Chemistry. 270: 23693-9. PMID 7559539 DOI: 10.1074/Jbc.270.40.23693  0.405
1995 Sango K, Yamanaka S, Hoffmann A, Okuda Y, Grinberg A, Westphal H, McDonald MP, Crawley JN, Sandhoff K, Suzuki K, Proia RL. Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism. Nature Genetics. 11: 170-6. PMID 7550345 DOI: 10.1038/Ng1095-170  0.393
1995 Schick A, Kolter T, Giannis A, Sandhoff K. Synthesis of phosphonate analogues of sphinganine-1-phosphate and sphingosine-1-phosphate Tetrahedron. 51: 11207-11218. DOI: 10.1016/0040-4020(95)00688-5  0.668
1995 Sandhoff K, Kolter T. Glykolipide der Zelloberfl�che ? Biochemie ihres Abbaus Naturwissenschaften. 82: 403-413. DOI: 10.1007/S001140050208  0.457
1995 Sandhoff K, Kolter T. Glykolipide der Zelloberfl�che ?Biochemie ihres Abbaus Naturwissenschaften. 82: 403-413. DOI: 10.1007/Bf01133673  0.303
1995 Kolter T, Sandhoff K. New Brain Lipids that Induce Sleep Angewandte Chemie International Edition in English. 34: 2363-2364. DOI: 10.1002/Anie.199523631  0.332
1995 Kolter T, Sandhoff K. Neue Gehirnlipide mit schlafinduzierender Wirkung Angewandte Chemie. 107: 2559-2560. DOI: 10.1002/Ange.19951072106  0.304
1994 Swallow DM, Islam I, Fox MF, Povey S, Klima H, Schepers U, Sandhoff K. Regional localization of the gene coding for the GM2 activator protein (GM2A) to chromosome 5q32-33 and confirmation of the assignment of GM2AP to chromosome 3. Annals of Human Genetics. 57: 187-93. PMID 8257088 DOI: 10.1111/j.1469-1809.1993.tb01594.x  0.212
1994 Schröder M, Schnabel D, Hurwitz R, Young E, Suzuki K, Sandhoff K. Molecular genetics of GM2-gangliosidosis AB variant: a novel mutation and expression in BHK cells. Human Genetics. 92: 437-40. PMID 8244332 DOI: 10.1007/Bf00216446  0.426
1994 Sandhoff K, Klein A. Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion. Febs Letters. 346: 103-7. PMID 8206147 DOI: 10.1016/0014-5793(94)00282-7  0.438
1994 Zschoche A, Fürst W, Schwarzmann G, Sandhoff K. Hydrolysis of lactosylceramide by human galactosylceramidase and GM1‐β‐galactosidase in a detergent‐free system and its stimulation by sphingolipid activator proteins, sap‐B and sap‐C Activator proteins stimulate lactosylceramide hydrolysis Febs Journal. 222: 83-90. PMID 8200356 DOI: 10.1111/J.1432-1033.1994.Tb18844.X  0.422
1994 Klein A, Henseler M, Klein C, Suzuki K, Harzer K, Sandhoff K. Sphingolipid activator protein D (sap-D) stimulates the lysosomal degradation of ceramide in vivo. Biochemical and Biophysical Research Communications. 200: 1440-8. PMID 8185598 DOI: 10.1006/Bbrc.1994.1612  0.415
1994 Ferlinz K, Hurwitz R, Vielhaber G, Suzuki K, Sandhoff K. Occurrence of two molecular forms of human acid sphingomyelinase. The Biochemical Journal. 855-62. PMID 8053910 DOI: 10.1042/Bj3010855  0.453
1994 Sandhoff K, van Echten G. Ganglioside metabolism: enzymology, topology and regulation. Progress in Brain Research. 101: 17-29. PMID 8029449 DOI: 10.1016/S0079-6123(08)61937-8  0.422
1994 Hurwitz R, Ferlinz K, Sandhoff K. The tricyclic antidepressant desipramine causes proteolytic degradation of lysosomal sphingomyelinase in human fibroblasts. Biological Chemistry Hoppe-Seyler. 375: 447-50. PMID 7945993 DOI: 10.1515/Bchm3.1994.375.7.447  0.427
1994 Sandhoff K, Echten Gv. Chapter 3 – Metabolism of Gangliosides: Topology, Pathobiochemistry, and Sphingolipid Activator Proteins Current Topics in Membranes. 40: 75-91. DOI: 10.1016/S0070-2161(08)60977-6  0.396
1994 Kolter T, van Echten-Deckert G, Sandhoff K. Synthesis of sphinganine analogues modified in the head group Tetrahedron. 50: 13425-13432. DOI: 10.1016/S0040-4020(01)89349-2  0.364
1993 Klima H, Klein A, van Echten G, Schwarzmann G, Suzuki K, Sandhoff K. Over-expression of a functionally active human GM2-activator protein in Escherichia coli. The Biochemical Journal. 571-6. PMID 8503891 DOI: 10.1042/Bj2920571  0.331
1993 Vanier MT, Ferlinz K, Rousson R, Duthel S, Louisot P, Sandhoff K, Suzuki K. Deletion of arginine (608) in acid sphingomyelinase is the prevalent mutation among Niemann-Pick disease type B patients from northern Africa. Human Genetics. 92: 325-30. PMID 8225311 DOI: 10.1007/Bf01247328  0.38
1993 Liessem B, Giannis A, Sandhoff K, Nieger M. Synthese von 2-acetamido-1,4-imino-1,2,4-tridesoxy-d- galaktitol und kompetitive Inhibition der humanen lysosomalen β-hexosaminidase A Carbohydrate Research. 250: 19-30. PMID 8143290 DOI: 10.1016/0008-6215(93)84151-U  0.659
1993 Akli S, Boue J, Sandhoff K, Kleijer W, Vamos E, Young E, Gatti R, Di Natale P, Motte J, Vanier MT. Collaborative study of the molecular epidemiology of Tay-Sachs disease in Europe. European Journal of Human Genetics : Ejhg. 1: 229-38. PMID 8044648 DOI: 10.1159/000472416  0.274
1993 Antes P, Schwarzmann G, Sandhoff K. Detection of protein mediated glycosphingolipid clustering by the use of resonance energy transfer between fluorescent labelled lipids. A method established by applying the system ganglioside GM1 and cholera toxin B subunit. Chemistry and Physics of Lipids. 62: 269-80. PMID 1468126 DOI: 10.1016/0009-3084(92)90064-V  0.383
1993 Zdebska E, Antoniewicz J, Nilsson B, Sandhoff K, Fürst W, Janik P, Kościelak J. Ganglioside binding proteins of calf brain with ubiquitin-like N-terminals. European Journal of Biochemistry. 210: 483-9. PMID 1333954 DOI: 10.1111/J.1432-1033.1992.Tb17446.X  0.399
1993 Sandhoff K. S7.1 Topology and regulation of glycolipid metabolism Glycoconjugate Journal. 10: 260-261. DOI: 10.1007/Bf01209928  0.339
1993 Bär T, Kratzer B, Wild R, Sandhoff K, Schmidt RR. Synthesis of Sphingosines, Part 8. Synthesis of Methyl-branched Sphingosines Liebigs Annalen Der Chemie. 1993: 419-426. DOI: 10.1002/Jlac.199319930168  0.483
1993 Birk R, Sandhoff K, Schmidt RR. Epoxides of Phytosphingosine and Derivatives, Potential Inhibitors of Sphingosine Biosynthesis Liebigs Annalen Der Chemie. 1993: 71-75. DOI: 10.1002/Jlac.199319930112  0.487
1992 Conzelmann E, Sandhoff K. Biochemical basis of late-onset neurolipidoses. Developmental Neuroscience. 13: 197-204. PMID 1817024 DOI: 10.1159/000112160  0.304
1992 Schmid B, Paton BC, Sandhoff K, Harzer K. Metabolism of GM1 ganglioside in cultured skin fibroblasts: anomalies in gangliosidoses, sialidoses, and sphingolipid activator protein (SAP, saposin) 1 and prosaposin deficient disorders. Human Genetics. 89: 513-8. PMID 1634229 DOI: 10.1007/Bf00219176  0.444
1992 Iber H, Zacharias C, Sandhoff K. The c-series gangliosides GT3, GT2 and GP1c are formed in rat liver Golgi by the same set of glycosyltransferases that catalyse the biosynthesis of asialo-, a- and b-series gangliosides. Glycobiology. 2: 137-42. PMID 1606358 DOI: 10.1093/Glycob/2.2.137  0.329
1992 Fürst W, Sandhoff K. Activator proteins and topology of lysosomal sphingolipid catabolism. Biochimica Et Biophysica Acta. 1126: 1-16. PMID 1606169 DOI: 10.1016/0005-2760(92)90210-M  0.431
1992 Iber H, van Echten G, Sandhoff K. Fractionation of primary cultured cerebellar neurons: distribution of sialyltransferases involved in ganglioside biosynthesis. Journal of Neurochemistry. 58: 1533-7. PMID 1548486 DOI: 10.1111/J.1471-4159.1992.Tb11375.X  0.399
1992 Rother J, van Echten G, Schwarzmann G, Sandhoff K. Biosynthesis of sphingolipids: dihydroceramide and not sphinganine is desaturated by cultured cells. Biochemical and Biophysical Research Communications. 189: 14-20. PMID 1449467 DOI: 10.1016/0006-291X(92)91518-U  0.392
1992 Sandhoff K, van Echten G, Schröder M, Schnabel D, Suzuki K. Metabolism of glycolipids: the role of glycolipid-binding proteins in the function and pathobiochemistry of lysosomes. Biochemical Society Transactions. 20: 695-9. PMID 1426613 DOI: 10.1042/Bst0200695  0.379
1992 Kuhn CS, Lehmann J, Sandhoff K. Efficient photoaffinity labeling of human beta-hexosaminidase A. Synthesis and application of 3-azi-1-[(2-acetamido-2-deoxy-1-beta- D-glucopyranosyl)thio]- and -galactopyranosyl)thio]butane. Bioconjugate Chemistry. 3: 230-3. PMID 1387803 DOI: 10.1021/Bc00015A005  0.389
1992 Leinekugel P, Michel S, Conzelmann E, Sandhoff K. Quantitative correlation between the residual activity of beta-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease. Human Genetics. 88: 513-23. PMID 1348043 DOI: 10.1007/BF00219337  0.335
1992 Zeitler R, Giannis A, Danneschewski S, Henk E, Henk T, Bauer C, Reutter W, Sandhoff K. Inhibition of N-acetylglucosamine kinase and N-acetylmannosamine kinase by 3-O-methyl-N-acetyl-D-glucosamine in vitro European Journal of Biochemistry. 204: 1165-1168. PMID 1312935 DOI: 10.1111/J.1432-1033.1992.Tb16743.X  0.658
1992 Plewe M, Sandhoff K, Schmidt RR. Synthesis of a 4-Deoxy-4-C-Methylene Analog of Glucosylceramide Journal of Carbohydrate Chemistry. 11: 881-890. DOI: 10.1080/07328309208018276  0.493
1992 Brenner-Weiß G, Giannis A, Sandhoff K. Synthesis of potential inhibitors of the glycosphingolipid biosynthesis Tetrahedron. 48: 5855-5860. DOI: 10.1016/S0040-4020(01)90177-2  0.664
1992 Sandhoff K. Topology and regulation of ganglioside synthesis Neurochemistry International. 21: Q5. DOI: 10.1016/0197-0186(92)91804-6  0.235
1992 Plewe M, Sandhoff K, Schmidt RR. Synthesis of 4-C-methyl analogues of glucosylceramide Carbohydrate Research. 235: 151-161. DOI: 10.1016/0008-6215(92)80085-F  0.504
1992 Sandhoff K. Glycolipids of the cell surface: Biosynthesis and topology of metabolism Fresenius' Journal of Analytical Chemistry. 343: 44-45. DOI: 10.1007/Bf00331989  0.354
1992 Kuhn C, Lehmann J, Sandhoff K. Irreversible Desaktivierung von menschlicher β-Hexosaminidase A mit dem alkylierenden Pseudodisaccharid (3,4,6/5)-6-(2-Acetamido-2-desoxy-β-D-glucopyranosylthio)-3,4-epoxy-5-hydroxycyclohexen Liebigs Annalen Der Chemie. 1992: 407-409. DOI: 10.1002/Jlac.199219920172  0.344
1992 Henk T, Giannis A, Sandhoff K. Wittig Olefination of Unprotected Carbohydrates with a Semistabilized Ylide Liebigs Annalen Der Chemie. 1992: 167-168. DOI: 10.1002/Jlac.199219920130  0.645
1992 Plewe M, Sandhoff K, Schmidt RR. Synthesis of 4-Epoxy-4-c-methyleneglycosylceramides, Potential Glycosyltransferase Inhibitors Liebigs Annalen Der Chemie. 1992: 699-708. DOI: 10.1002/Jlac.1992199201118  0.495
1991 Schwarzmann G, Sandhoff K. Metabolism and intracellular transport of glycosphingolipids. Biochemistry. 29: 10865-71. PMID 2271686 DOI: 10.1021/Bi00501A001  0.335
1991 Meier EM, Schummer D, Sandhoff K. Evidence for the presence of water within the hydrophobic core of membranes. Chemistry and Physics of Lipids. 55: 103-13. PMID 2090357 DOI: 10.1016/0009-3084(90)90071-X  0.386
1991 Schnabel D, Schröder M, Sandhoff K. Mutation in the sphingolipid activator protein 2 in a patient with a variant of Gaucher disease. Febs Letters. 284: 57-9. PMID 2060627 DOI: 10.1016/0014-5793(91)80760-Z  0.416
1991 Quintern LE, Sandhoff K. Human acid sphingomyelinase from human urine. Methods in Enzymology. 197: 536-40. PMID 2051941 DOI: 10.1016/0076-6879(91)97180-7  0.252
1991 Holtschmidt H, Sandhoff K, Fürst W, Kwon HY, Schnabel D, Suzuki K. The organization of the gene for the human cerebroside sulfate activator protein. Febs Letters. 280: 267-70. PMID 2013321 DOI: 10.1016/0014-5793(91)80308-P  0.382
1991 Iber H, van Echten G, Sandhoff K. Substrate specificity of alpha 2----3-sialyltransferases in ganglioside biosynthesis of rat liver golgi. European Journal of Biochemistry. 195: 115-20. PMID 1991463 DOI: 10.1111/J.1432-1033.1991.Tb15683.X  0.358
1991 Schröder M, Schnabel D, Suzuki K, Sandhoff K. A mutation in the gene of a glycolipid-binding protein (GM2 activator) that causes GM2-gangliosidosis variant AB. Febs Letters. 290: 1-3. PMID 1915858 DOI: 10.1016/0014-5793(91)81211-P  0.407
1991 Klima H, Tanaka A, Schnabel D, Nakano T, Schröder M, Suzuki K, Sandhoff K. Characterization of full-length cDNAs and the gene coding for the human GM2 activator protein. Febs Letters. 289: 260-4. PMID 1915857 DOI: 10.1016/0014-5793(91)81084-L  0.359
1991 Vogel A, Schwarzmann G, Sandhoff K. Glycosphingolipid specificity of the human sulfatide activator protein. European Journal of Biochemistry. 200: 591-7. PMID 1889421 DOI: 10.1111/J.1432-1033.1991.Tb16222.X  0.449
1991 Van Weely S, Van Leeuwen MB, Jansen IDC, De Bruijn MAC, Brouwer-Kelder EM, Schram A, Sa Miranda MC, Barranger JA, Petersen EM, Goldblatt J, Stotz H, Schwarzmann G, Sandhoff K, Svennerholm L, Erikson A, et al. Clinical phenotype of Gaucher disease in relation to properties of mutant glucocerebrosidase in cultured fibroblasts Bba - Molecular Basis of Disease. 1096: 301-311. PMID 1829642 DOI: 10.1016/0925-4439(91)90066-I  0.644
1991 Ferlinz K, Hurwitz R, Sandhoff K. Molecular basis of acid sphingomyelinase deficiency in a patient with Niemann-Pick disease type A. Biochemical and Biophysical Research Communications. 179: 1187-91. PMID 1718266 DOI: 10.1016/0006-291X(91)91697-B  0.384
1991 Mandon EC, van Echten G, Birk R, Schmidt RR, Sandhoff K. Sphingolipid biosynthesis in cultured neurons. Down-regulation of serine palmitoyltransferase by sphingoid bases. European Journal of Biochemistry. 198: 667-74. PMID 1646717 DOI: 10.1111/J.1432-1033.1991.Tb16065.X  0.552
1991 Birk R, Brenner-Weiß G, Giannis A, Sandhoff K, Schmidt RR. Specific tritium labelling of sphingosines at the 3-position Journal of Labelled Compounds and Radiopharmaceuticals. 29: 289-298. DOI: 10.1002/Jlcr.2580290306  0.478
1990 Fürst W, Schubert J, Machleidt W, Meyer HE, Sandhoff K. The complete amino-acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein. European Journal of Biochemistry. 192: 709-14. PMID 2209618 DOI: 10.1111/J.1432-1033.1990.Tb19280.X  0.429
1990 Röder B, Dabrowski J, Dabrowski U, Egge H, Peter-Katalinić J, Schwarzmann G, Sandhoff K. The determination of phytosphingosine-containing globotriaosylceramide from human kidney in the presence of lactosylceramide. Chemistry and Physics of Lipids. 53: 85-9. PMID 2162261 DOI: 10.1016/0009-3084(90)90136-F  0.389
1990 Singh NP, Schmidt RR, Giannis A, Henk E, Kolter T, Sandhoff K. Synthesis of 2-Carboxy-Substituted Sphingosine Derivatives Journal of Carbohydrate Chemistry. 9: 543-559. DOI: 10.1080/07328309008543852  0.702
1990 Áts S, Lausberg E, Lehmann J, Sandhoff K. Synthese von 4-Acetamido-3,7-anhydro-2-azi-1,2,4-tridesoxy-D-glycero-D-gulo-octitol, einem potentiellen Photoaffinitätsreagens mit guter Affinität zu menschlicher β-Hexosaminidase Liebigs Annalen Der Chemie. 1990: 1261-1264. DOI: 10.1002/Jlac.1990199001226  0.377
1989 van Echten G, Sandhoff K. Modulation of ganglioside biosynthesis in primary cultured neurons. Journal of Neurochemistry. 52: 207-14. PMID 2908888 DOI: 10.1111/J.1471-4159.1989.Tb10918.X  0.412
1989 Hohenschutz C, Friedl W, Schlör KH, Waheed A, Conzelmann E, Sandhoff K, Propping P. Probable metachromatic leukodystrophy/pseudodeficiency compound heterozygote at the arylsulfatase A locus with neurological and psychiatric symptomatology. American Journal of Medical Genetics. 31: 169-75. PMID 2906225 DOI: 10.1002/Ajmg.1320310120  0.366
1989 Iber H, Sandhoff K. Identity of GD1C, GT1a and GQ1b synthase in Golgi vesicles from rat liver. Febs Letters. 254: 124-8. PMID 2776878 DOI: 10.1016/0014-5793(89)81022-1  0.297
1989 Schröder M, Klima H, Nakano T, Kwon H, Quintern LE, Gärtner S, Suzuki K, Sandhoff K. Isolation of a cDNA encoding the human GM2 activator protein. Febs Letters. 251: 197-200. PMID 2753159 DOI: 10.1016/0014-5793(89)81454-1  0.406
1989 Quintern LE, Zenk TS, Sandhoff K. The urine from patients with peritonitis as a rich source for purifying human acid sphingomyelinase and other lysosomal enzymes. Biochimica Et Biophysica Acta. 1003: 121-4. PMID 2543449 DOI: 10.1016/0005-2760(89)90244-0  0.407
1989 Nakano T, Sandhoff K, Stümper J, Christomanou H, Suzuki K. Structure of full-length cDNA coding for sulfatide activator, a Co-beta-glucosidase and two other homologous proteins: two alternate forms of the sulfatide activator. Journal of Biochemistry. 105: 152-4. PMID 2498298 DOI: 10.1093/Oxfordjournals.Jbchem.A122629  0.394
1989 Iber H, Kaufmann R, Pohlentz G, Schwarzmann G, Sandhoff K. Identity of GA1, GM1a and GD1b synthase in Golgi vesicles from rat liver. Febs Letters. 248: 18-22. PMID 2498125 DOI: 10.1016/0014-5793(89)80423-5  0.376
1989 Quintern L, Schuchman E, Levran O, Suchi M, Ferlinz K, Reinke H, Sandhoff K, Desnick R. Isolation of cDNA clones encoding human acid sphingomyelinase: occurrence of alternatively processed transcripts. The Embo Journal. 8: 2469-2473. DOI: 10.1002/J.1460-2075.1989.Tb08382.X  0.368
1989 GIANNIS A, SANDHOFF K. ChemInform Abstract: LiBH4(NaBH4)/Me3SiCl, an Unusually Potent and Versatile Reduction Agent. Cheminform. 20. DOI: 10.1002/CHIN.198918117  0.208
1989 GIANNIS A, MUENSTER P, SANDHOFF K, STEGLICH W. ChemInform Abstract: Fragmentation and Wittig Olefination of Glucosamine Derivatives - A Simple Route to Open Chain Amino Sugars and Chiral Glycerols. Cheminform. 20. DOI: 10.1002/CHIN.198914311  0.253
1989 Giannis A, Sandhoff K. LiBH4(NaBH4)/Me3SiCl, an Unusually Strong and Versatile Reducing Agent Angewandte Chemie International Edition in English. 28: 218-220. DOI: 10.1002/Anie.198902181  0.628
1989 Giannis A, Sandhoff K. LiBH4(NaBH4)/Me3SiCl, ein ungewöhnlich starkes und vielseitig einsetzbares Reduktionsmittel Angewandte Chemie. 101: 220-222. DOI: 10.1002/Ange.19891010231  0.626
1988 Vogel A, Fürst W, Abo-Hashish MA, Lee-Vaupel M, Conzelmann E, Sandhoff K. Identity of the activator proteins for the enzymatic hydrolysis of sulfatide, ganglioside GM1, and globotriaosylceramide. Archives of Biochemistry and Biophysics. 259: 627-38. PMID 3426245 DOI: 10.1016/0003-9861(87)90529-7  0.423
1988 Willibald CJ, Rösner H, Schwarzmann G, Sandhoff K, Rahmann H. Axonal transport of intraocularly injected [3H-Sph]-GD1a in the chicken optic system and the fate of the exogenous ganglioside distributed by blood. Neuroscience Research. 5: 361-79. PMID 3399144 DOI: 10.1016/0168-0102(88)90023-5  0.227
1988 Pohlentz G, Klein D, Schmitz D, Schwarzmann G, Peter-Katalinić J, Sandhoff K. Biosynthesis of gangliosides from asialogangliosides in rat liver Golgi vesicles. Biological Chemistry Hoppe-Seyler. 369: 55-63. PMID 3348889 DOI: 10.1515/Bchm3.1988.369.1.55  0.392
1988 Pohlentz G, Klein D, Schwarzmann G, Schmitz D, Sandhoff K. Both GA2, GM2, and GD2 synthases and GM1b, GD1a, and GT1b synthases are single enzymes in Golgi vesicles from rat liver. Proceedings of the National Academy of Sciences of the United States of America. 85: 7044-8. PMID 3140234 DOI: 10.1073/Pnas.85.19.7044  0.388
1988 Sandhoff K, Quintern L. [Sphingolipid storage diseases of the central nervous system: bases of biochemical and clinical heterogeneity]. Die Naturwissenschaften. 75: 123-31. PMID 3132618 DOI: 10.1007/Bf00405302  0.413
1988 Klein D, Pohlentz G, Hinrichs U, Sandhoff K. Metabolism of ganglioside-amides in cultured human fibroblasts. Biological Chemistry Hoppe-Seyler. 368: 1495-503. PMID 3124867 DOI: 10.1515/Bchm3.1987.368.2.1495  0.368
1988 Fürst W, Machleidt W, Sandhoff K. The precursor of sulfatide activator protein is processed to three different proteins. Biological Chemistry Hoppe-Seyler. 369: 317-28. PMID 3048308 DOI: 10.1515/Bchm3.1988.369.1.317  0.425
1988 Esmann M, Marsh D, Schwarzmann G, Sandhoff K. Ganglioside-protein interactions: spin-label electron spin resonance studies with (Na+,K+)-ATPase membranes. Biochemistry. 27: 2398-403. PMID 2838072 DOI: 10.1021/Bi00407A022  0.38
1988 Giannis A, Münster P, Sandhoff K, Steglich W. Fragmentation and wittig olefination of glucosamine derivatives-a simple route to open chain amino sugars and chiral glycerols Tetrahedron. 44: 7177-7180. DOI: 10.1016/S0040-4020(01)86086-5  0.658
1988 GIANNIS A, SANDHOFF K. ChemInform Abstract: Diastereoselective Synthesis of Functionalized C-α- and C-β-Glycosyl Derivatives of 2-Acetamido-2-deoxy-D-glucose. Cheminform. 19. DOI: 10.1002/CHIN.198828253  0.251
1987 Sarmientos F, Schwarzmann G, Sandhoff K. Specificity of human glucosylceramide beta-glucosidase towards synthetic glucosylsphingolipids inserted into liposomes. Kinetic studies in a detergent-free assay system. European Journal of Biochemistry. 160: 527-35. PMID 3780720 DOI: 10.1111/J.1432-1033.1986.Tb10071.X  0.454
1987 Schwarzmann G, Sandhoff K. Lysogangliosides: synthesis and use in preparing labeled gangliosides. Methods in Enzymology. 138: 319-41. PMID 3600331 DOI: 10.1016/0076-6879(87)38028-0  0.424
1987 Conzelmann E, Sandhoff K. Activator proteins for lysosomal glycolipid hydrolysis. Methods of Biochemical Analysis. 32: 1-23. PMID 3553853 DOI: 10.1002/9780470110539.ch1  0.309
1987 Conzelmann E, Sandhoff K. Glycolipid and glycoprotein degradation. Advances in Enzymology and Related Areas of Molecular Biology. 60: 89-216. PMID 3310533 DOI: 10.1002/9780470123065.Ch3  0.368
1987 Klein D, Pohlentz G, Schwarzmann G, Sandhoff K. Substrate specificity of GM2 and GD3 synthase of Golgi vesicles derived from rat liver. European Journal of Biochemistry. 167: 417-24. PMID 3115774 DOI: 10.1111/J.1432-1033.1987.Tb13354.X  0.421
1987 Yusuf HK, Schwarzmann G, Pohlentz G, Sandhoff K. Oligosialogangliosides inhibit GM2- and GD3-synthesis in isolated Golgi vesicles from rat liver. Biological Chemistry Hoppe-Seyler. 368: 455-62. PMID 3113445 DOI: 10.1515/Bchm3.1987.368.1.455  0.425
1987 Novotny JR, Brendler S, Kytzia HJ, Sandhoff K, Gaedicke G. Hexosaminidase I indicates maturation disarrangement in acute leukemias. Haematology and Blood Transfusion. 31: 191-4. PMID 2965081 DOI: 10.1007/978-3-642-72624-8_42  0.185
1987 Conzelmann E, Sandhoff K. Activator proteins for lysosomal glycolipid hydrolysis. Methods in Enzymology. 138: 792-815. PMID 2885714 DOI: 10.1016/0076-6879(87)38068-1  0.435
1987 Propping P, Friedl W, Huschka M, Schlör KH, Reimer F, Lee-Vaupel M, Conzelmann E, Sandhoff K. The influence of low arylsulfatase A activity on neuropsychiatric morbidity: a large-scale screening in patients. Human Genetics. 74: 244-8. PMID 2877931 DOI: 10.1007/Bf00282542  0.352
1987 Quintern LE, Weitz G, Nehrkorn H, Tager JM, Schram AW, Sandhoff K. Acid sphingomyelinase from human urine: purification and characterization. Biochimica Et Biophysica Acta. 922: 323-36. PMID 2825797 DOI: 10.1016/0005-2760(87)90055-5  0.461
1987 Giannis A, Sandhoff K. Diastereoselektive synthese von funktionalisierten C-α- und -β-glykosylderivaten der 2-acetamido-2-desoxy-d-glucose Carbohydrate Research. 171: 201-210. DOI: 10.1016/S0008-6215(00)90887-5  0.651
1987 Klein D, Pohlentz G, Schwarzmann G, Sandhoff K. Substrate specificities of a bacterial sialidase and rat liver ganglioside GM3 sialyltransferase Glycoconjugate Journal. 4: 291-295. DOI: 10.1007/Bf01048435  0.42
1987 Ollmann M, Schwarzmann G, Sandhoff K, Galla HJ. Pyrene-Labeled Gangliosides: Micelle Formation in Aqueous Solution, Lateral Diffusion, and Thermotropic Behavior in Phosphatidylcholine Bilayers? Biochemistry. 26: 181-182. DOI: 10.1007/978-3-642-71932-5_13  0.368
1986 Sandhoff K, Conzelmann E. The biochemical basis of gangliosidoses. Neuropediatrics. 85-92. PMID 6242704 DOI: 10.1055/s-2008-1052387  0.243
1986 Burg J, Banerjee A, Sandhoff K. Molecular forms of GM2-activator protein. A study on its biosynthesis in human skin fibroblasts. Biological Chemistry Hoppe-Seyler. 366: 887-91. PMID 3935131 DOI: 10.1515/Bchm3.1985.366.2.887  0.442
1986 Burg J, Conzelmann E, Sandhoff K, Solomon E, Swallow DM. Mapping of the gene coding for the human GM2 activator protein to chromosome 5. Annals of Human Genetics. 49: 41-5. PMID 3865618 DOI: 10.1111/J.1469-1809.1985.Tb01674.X  0.377
1986 Neuenhofer S, Conzelmann E, Schwarzmann G, Egge H, Sandhoff K. Occurrence of lysoganglioside lyso-GM2 (II3-Neu5Ac-gangliotriaosylsphingosine) in GM2 gangliosidosis brain. Biological Chemistry Hoppe-Seyler. 367: 241-4. PMID 3707714 DOI: 10.1515/Bchm3.1986.367.1.241  0.348
1986 Conzelmann E, Nehrkorn H, Kytzia HJ, Sandhoff K, Macek M, Lehovský M, Elleder M, Jirásek A, Kobilková J. Prenatal diagnosis of GM2 gangliosidosis with high residual hexosaminidase A activity (variant B1; pseudo AB variant). Pediatric Research. 19: 1220-4. PMID 2933632 DOI: 10.1203/00006450-198511000-00022  0.331
1986 Sonderfeld S, Brendler S, Sandhoff K, Galjaard H, Hoogeveen AT. Genetic complementation in somatic cell hybrids of four variants of infantile GM2 gangliosidosis. Human Genetics. 71: 196-200. PMID 2933318 DOI: 10.1007/Bf00284572  0.379
1985 Yusuf HK, Pohlentz G, Sandhoff K. Ganglioside biosynthesis in Golgi apparatus: new perspectives on its mechanism. Journal of Neuroscience Research. 12: 161-78. PMID 6502747 DOI: 10.1002/Jnr.490120205  0.429
1985 Neuenhofer S, Schwarzmann G, Egge H, Sandhoff K. Synthesis of lysogangliosides. Biochemistry. 24: 525-32. PMID 3978090 DOI: 10.1021/Bi00323A042  0.401
1985 Sarmientos F, Schwarzmann G, Sandhoff K. Direct evidence by carbon-13 NMR spectroscopy for the erythro configuration of the sphingoid moiety in Gaucher cerebroside and other natural sphingolipids. European Journal of Biochemistry. 146: 59-64. PMID 3967657 DOI: 10.1111/J.1432-1033.1985.Tb08619.X  0.351
1985 Sonderfeld S, Conzelmann E, Schwarzmann G, Burg J, Hinrichs U, Sandhoff K. Incorporation and metabolism of ganglioside GM2 in skin fibroblasts from normal and GM2 gangliosidosis subjects. European Journal of Biochemistry. 149: 247-55. PMID 3922757 DOI: 10.1111/J.1432-1033.1985.Tb08919.X  0.419
1985 Scheel G, Schwarzmann G, Hoffmann-Bleihauer P, Sandhoff K. The influence of ganglioside insertion into brain membranes on the rate of ganglioside degradation by membrane-bound sialidase. European Journal of Biochemistry. 153: 29-35. PMID 2998792 DOI: 10.1111/J.1432-1033.1985.Tb09262.X  0.41
1985 Driessen M, Weitz G, Brouwer-Kelder EM, Donker-Koopman WE, Bastiaannet J, Sandhoff K, Barranger JA, Tager JM, Schram A. The effect of detergents on immunoprecipitability of lysosomal sphingomyelinase Bba - General Subjects. 841: 97-102. PMID 2990569 DOI: 10.1016/0304-4165(85)90278-8  0.624
1985 Weitz G, Driessen M, Brouwer-Kelder EM, Sandhoff K, Barranger JA, Tager JM, Schram A. Soluble sphingomyelinase from human urine as antigen for obtaining anti-sphingomyelinase antibodies Bba - General Subjects. 838: 92-97. PMID 2981569 DOI: 10.1016/0304-4165(85)90254-5  0.618
1985 Giannis A, Sandhoff K. Stereoselective synthesis of α- C-allyl-glycopyranosides Tetrahedron Letters. 26: 1479-1482. DOI: 10.1016/S0040-4039(00)98529-0  0.664
1985 Sandhoff K. Function and relevance of activator proteins for glycolipid degradation Seikagaku. 57: 87-102. DOI: 10.1016/B978-0-12-079280-1.50007-3  0.392
1985 Neuenhofer S, Sandhoff K. Affinity labelling of the GM2 -activator protein Febs Letters. 185: 112-114. DOI: 10.1016/0014-5793(85)80751-1  0.338
1985 GIANNIS A, SANDHOFF K. ChemInform Abstract: STEREOSELECTIVE SYNTHESIS OF α-C-ALLYLGLYCOPYRANOSIDES Chemischer Informationsdienst. 16. DOI: 10.1002/chin.198530373  0.232
1984 Yusuf HK, Pohlentz G, Schwarzmann G, Sandhoff K. Ganglioside biosynthesis in rat liver golgi apparatus: stimulation by phosphatidylglycerol and inhibition by tunicamycin. Advances in Experimental Medicine and Biology. 174: 227-39. PMID 6741731 DOI: 10.1007/978-1-4684-1200-0_19  0.431
1984 Banerjee A, Burg J, Conzelmann E, Carroll M, Sandhoff K. Enzyme-linked immunosorbent assay for the ganglioside GM2-activator protein. Screening of normal human tissues and body fluids, of tissues of GM2 gangliosidosis, and for its subcellular localization. Hoppe-Seyler's Zeitschrift Fur Physiologische Chemie. 365: 347-56. PMID 6724528 DOI: 10.1515/Bchm2.1984.365.1.347  0.422
1984 Kytzia HJ, Hinrichs U, Sandhoff K. Diagnosis of infantile and juvenile forms of GM2 gangliosidosis variant 0. Residual activities toward natural and different synthetic substrates. Human Genetics. 67: 414-8. PMID 6436167 DOI: 10.1007/Bf00291402  0.393
1984 Conzelmann E, Sandhoff K. Partial enzyme deficiencies: residual activities and the development of neurological disorders. Developmental Neuroscience. 6: 58-71. PMID 6421563 DOI: 10.1159/000112332  0.305
1984 Yusuf HK, Pohlentz G, Sandhoff K. Tunicamycin inhibits ganglioside biosynthesis in rat liver Golgi apparatus by blocking sugar nucleotide transport across the membrane vesicles. Proceedings of the National Academy of Sciences of the United States of America. 80: 7075-9. PMID 6417662 DOI: 10.1073/Pnas.80.23.7075  0.417
1984 Schwarzmann G, Hoffmann-Bleihauer P, Schubert J, Sandhoff K, Marsh D. Incorporation of ganglioside analogues into fibroblast cell membranes. A spin-label study. Biochemistry. 22: 5041-8. PMID 6315058 DOI: 10.1021/Bi00290A025  0.423
1984 Schram AW, Dreissen M, Bastiaannet J, Donker-Koopman WE, Brouwer-Kelder EM, Weitz G, Barranger JA, Sandhoff K, Tager JM. Immunological studies on lysosomal sphingomyelinase: Identification of a 28 000-Da component deficient in urine from patients with Niemann-Pick disease types A and B Bioscience Reports. 4: 1051-1057. PMID 6099155 DOI: 10.1007/Bf01116698  0.608
1984 Schram AW, Weitz G, Driessen M, Brouwer-Kelder EM, Sandhoff K, Barranger JA, Tager JM. Immunological properties of urinary sphingomyelinase Biochemical Society Transactions. 12: 1027-1028. DOI: 10.1042/Bst0121027  0.571
1983 Yusuf HK, Pohlentz G, Schwarzmann G, Sandhoff K. Ganglioside biosynthesis in Golgi apparatus of rat liver. Stimulation by phosphatidylglycerol and inhibition by tunicamycin. European Journal of Biochemistry. 134: 47-54. PMID 6861762 DOI: 10.1111/J.1432-1033.1983.Tb07529.X  0.419
1983 Weitz G, Lindl T, Hinrichs U, Sandhoff K. Release of sphingomyelin phosphodiesterase (acid sphingomyelinase) by ammonium chloride from CL 1D mouse L-cells and human fibroblasts. Partial purification and characterization of the exported enzymes. Hoppe-Seyler's Zeitschrift Fur Physiologische Chemie. 364: 863-71. PMID 6311712 DOI: 10.1515/Bchm2.1983.364.2.863  0.412
1983 Burg J, Banerjee A, Conzelmann E, Sandhoff K. Activating proteins for ganglioside GM2 degradation by beta-hexosaminidase isoenzymes in tissue extracts from different species. Hoppe-Seyler's Zeitschrift Fur Physiologische Chemie. 364: 821-9. PMID 6225718 DOI: 10.1515/Bchm2.1983.364.2.821  0.422
1983 Kytzia H, Hinrichs U, Maire I, Suzuki K, Sandhoff K. Variant of GM2-gangliosidosis with hexosaminidase A having a severely changed substrate specificity. The Embo Journal. 2: 1201-1205. DOI: 10.1002/J.1460-2075.1983.Tb01567.X  0.418
1982 Scheel G, Acevedo E, Conzelmann E, Nehrkorn H, Sandhoff K. Model for the interaction of membrane-bound substrates and enzymes. Hydrolysis of ganglioside GD1a by sialidase of neuronal membranes isolated from calf brain. European Journal of Biochemistry / Febs. 127: 245-53. PMID 7140766 DOI: 10.1111/J.1432-1033.1982.Tb06862.X  0.426
1982 Marsh D, Pellkofer R, Hoffmann-Bleihauer P, Sandhoff K. Incorporation of lipids into cellular membranes--a spin-label assay. Analytical Biochemistry. 122: 206-12. PMID 6285763 DOI: 10.1016/0003-2697(82)90272-X  0.393
1982 Pellkofer R, Marsh D, Hoffmann-Bleihauer P, Sandhoff K. Melittin stimulates incorporation and degradation of sphingomyelin in synaptosomal plasma membranes. Journal of Neurochemistry. 38: 1230-5. PMID 6278088 DOI: 10.1111/J.1471-4159.1982.Tb07895.X  0.395
1982 Hasilik A, von Figura K, Conzelmann E, Nehrkorn H, Sandhoff K. Lysosomal enzyme precursors in human fibroblasts. Activation of cathepsin D precursor in vitro and activity of beta-hexosaminidase A precursor towards ganglioside GM2. European Journal of Biochemistry. 125: 317-21. PMID 6214395 DOI: 10.1111/J.1432-1033.1982.Tb06685.X  0.426
1982 Conzelmann E, Burg J, Stephan G, Sandhoff K. Complexing of glycolipids and their transfer between membranes by the activator protein for degradation of lysosomal ganglioside GM2. European Journal of Biochemistry. 123: 455-64. PMID 6210531 DOI: 10.1111/J.1432-1033.1982.Tb19789.X  0.442
1981 Schaub J, Janka GE, Christomanou H, Sandhoff K, Permanetter W, Hübner G, Meister P. Wolman's disease: clinical, biochemical and ultrastructural studies in an unusual case without striking adrenal calcification. European Journal of Pediatrics. 135: 45-53. PMID 7449788 DOI: 10.1007/BF00445892  0.27
1981 Erzberger A, Conzelmann E, Sandhoff K. Assay of ganglioside GM2-N-acetyl-beta-D-galactosaminidase activity in human fibroblasts employing the natural activator protein--diagnosis of variant forms of GM2 gangliosidosis. Clinica Chimica Acta; International Journal of Clinical Chemistry. 108: 361-8. PMID 6781795 DOI: 10.1016/0009-8981(80)90342-3  0.429
1980 Sandhoff K. [Lipid-protein interactions: mechanisms of enzymatic glycolipid catabolism and their genetic restrictive escapes]. Die Naturwissenschaften. 67: 431-41. PMID 7422009 DOI: 10.1007/Bf00405636  0.451
1980 Pallmann B, Sandhoff K, Berra B, Miyatake T. Sialidase in brain and fibroblasts in three patients with different types of sialidosis. Advances in Experimental Medicine and Biology. 125: 401-14. PMID 7361622 DOI: 10.1007/978-1-4684-7844-0_37  0.365
1980 Conzelmann E, Sandhoff K. The specificity of human N-acetyl-beta-D-hexosaminidases towards glycosphincolipids is determined by an activator protein. Advances in Experimental Medicine and Biology. 125: 295-306. PMID 6444774 DOI: 10.1007/978-1-4684-7844-0_27  0.471
1980 Pellkofer R, Sandhoff K. Halothane increases membrane fluidity and stimulates sphingomyelin degradation by membrane-bound neutral sphingomyelinase of synaptosomal plasma membranes from calf brain already at clinical concentrations. Journal of Neurochemistry. 34: 988-92. PMID 6244368 DOI: 10.1111/J.1471-4159.1980.Tb09675.X  0.39
1980 Conzelmann E, Sandhoff K. Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A. Hoppe-Seyler's Zeitschrift Fur Physiologische Chemie. 360: 1837-49. PMID 527942 DOI: 10.1515/Bchm2.1979.360.2.1837  0.456
1980 Sandhoff K, Christomanou H. Biochemistry and genetics of gangliosidoses. Human Genetics. 50: 107-43. PMID 116955 DOI: 10.1007/Bf00390234  0.449
1979 Miyatake T, Yamada T, Suzuki M, Pallmann B, Sandhoff K, Ariga T, Atsumi T. Sialidase deficiency in adult-type neuronal storage disease. Febs Letters. 97: 257-9. PMID 761631 DOI: 10.1016/0014-5793(79)80097-6  0.333
1979 Christomanou H, Cáp C, Sandhoff K. Prenatal diagnosis of Tay-Sachs disease in cell-free amniotic fluid. Klinische Wochenschrift. 56: 1133-5. PMID 713433 DOI: 10.1007/Bf01477136  0.365
1979 Sandhoff K, Conzelmann E. Activation of lysosomal hydrolysis of complex glycolipids by non-enzymic proteins Trends in Biochemical Sciences. 4: 231-233. DOI: 10.1016/0968-0004(79)90125-7  0.455
1979 Sandhoff K, Christomanou H. Biochemistry and genetics of gangliosidoses Human Genetics. 52: 353-353. DOI: 10.1007/BF00278685  0.183
1978 Sandhoff K, Pallmann B, Wiegandt H, Ziegler W. Studies on bovine brain membrane-bound neuraminidase (sialidase). Advances in Experimental Medicine and Biology. 101: 463-74. PMID 665379 DOI: 10.1007/978-1-4615-9071-2_42  0.415
1978 Sandhoff K, Pallmann B. Membrane-bound neuraminidase from calf brain: regulation of oligosialoganglioside degradation by membrane fluidity and membrane components. Proceedings of the National Academy of Sciences of the United States of America. 75: 122-6. PMID 272627 DOI: 10.1073/pnas.75.1.122  0.327
1978 Conzelmann E, Sandhoff K. AB variant of infantile GM2 gangliosidosis: deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2. Proceedings of the National Academy of Sciences of the United States of America. 75: 3979-83. PMID 99746 DOI: 10.1073/pnas.75.8.3979  0.331
1978 Sandhoff K, Conzelmann E, Nehrkorn H. Substrate specificity of hexosaminidase A isolated from the liver of a patient with a rare form (AB variant) of infantile GM2 gangliosidosis and control tissues. Advances in Experimental Medicine and Biology. 101: 727-30. PMID 96668 DOI: 10.1007/978-1-4615-9071-2_67  0.428
1978 Conzelmann E, Sandhoff K, Nehrkorn H, Geiger B, Arnon R. Purification, biochemical and immunological characterisation of hexosaminidase A from variant AB of infantile GM2 gangliosidosis. European Journal of Biochemistry. 84: 27-33. PMID 25769 DOI: 10.1111/J.1432-1033.1978.Tb12137.X  0.412
1977 Sandhoff K, Conzelmann E, Nehrkorn H. Specificity of human liver hexosaminidases A and B against glycosphingolipids GM2 and GA2. Purification of the enzymes by affinity chromatography employing specific elution. Hoppe-Seyler's Zeitschrift Fur Physiologische Chemie. 358: 779-87. PMID 892710 DOI: 10.1515/Bchm2.1977.358.2.779  0.379
1977 Schraven J, Cáp C, Nowoczek G, Sandhoff K. A radiometric assay for sialidase acting on ganglioside GD1a. Analytical Biochemistry. 78: 333-9. PMID 851209 DOI: 10.1016/0003-2697(77)90094-X  0.379
1977 Christomanou H, Cáp C, Sandhoff K. Isoelectric focusing pattern of acid hydrolases in cultured fibroblasts, leucocytes and cell-free amniotic fluid. Neuropadiatrie. 8: 238-52. PMID 578295 DOI: 10.1055/s-0028-1091520  0.282
1977 Sandhoff K. The biochemistry of sphingolipid storage diseases. Angewandte Chemie (International Ed. in English). 16: 273-85. PMID 406814 DOI: 10.1002/Anie.197702733  0.433
1977 Christomanou H, Sandhoff K. A sensitive fluorescence assay for the simultaneous and separate determination of arylsulphatases A and B. Clinica Chimica Acta; International Journal of Clinical Chemistry. 79: 527-31. PMID 19184 DOI: 10.1016/0009-8981(77)90172-3  0.361
1977 Sandhoff K. Biochemie der Erbkrankheiten Chemie in Unserer Zeit. 11: 1-13. DOI: 10.1002/ciuz.19770110102  0.19
1977 Sandhoff K. Biochemie der Sphingolipidspeicherkrankheiten Angewandte Chemie. 89: 283-295. DOI: 10.1002/Ange.19770890504  0.382
1976 Sandhoff K, Schraven J, Nowoczek G. Effect of xenon, nitrous oxide and halothane on membrane-bound sialidase from calf brain. Febs Letters. 62: 284-7. PMID 1278370 DOI: 10.1016/0014-5793(76)80076-2  0.354
1976 Jatzkewitz H, Sandhoff K. [Sphingolipid storage disease as an example of a molecular neuropathology (author's transl)]. Archiv Fur Psychiatrie Und Nervenkrankheiten. 221: 213-25. PMID 822805 DOI: 10.1007/Bf00418481  0.66
1974 Zerfowski J, Sandhoff K. [Juvenile GM2 gangliosidosis with altered substrate specificity of hexosaminidase A (author's transl)]. Acta Neuropathologica. 27: 225-32. PMID 4210440 DOI: 10.1007/Bf00687632  0.412
1974 Sandhoff K. Sphingolipidoses Journal of Clinical Pathology. 27: 94-105. DOI: 10.1136/jcp.27.Suppl_8.94  0.232
1972 Harzer K, Sandhoff K, Schall H, Kollmann F. [Enzymatic studies on the blood of carriers of a Tay-Sachs disease variant (variant O)]. Klinische Wochenschrift. 49: 1189-91. PMID 5124584 DOI: 10.1007/BF01732464  0.231
1972 Harzer K, Sandhoff K. Age-dependent variations of the human N-acetyl- -D-hexosaminidases. Journal of Neurochemistry. 18: 2041-50. PMID 4256910 DOI: 10.1111/J.1471-4159.1971.Tb05063.X  0.407
1971 Sandhoff K, Harzer K, Wässle W, Jatzkewitz H. Enzyme alterations and lipid storage in three variants of Tay-Sachs disease. Journal of Neurochemistry. 18: 2469-89. PMID 5135907 DOI: 10.1111/J.1471-4159.1971.Tb00204.X  0.681
1971 SANDHOFF K, WÄSSLE W. Anreicherung und Charakterisierung zweier Formen der menschlichen N-Acetyl-β-D-hexosaminidase Hoppe-Seyler´S Zeitschrift FüR Physiologische Chemie. 352: 1119-1133. DOI: 10.1515/Bchm2.1971.352.2.1119  0.343
1970 Jatzkewitz H, Mehl E, Sandhoff K. Blocks in the catabolism of sulphatides and gangliosides as a cause of human neurological diseases. The Biochemical Journal. 117: 6P-8P. PMID 4911751  0.616
1970 Wässle W, Sandhoff K. Einfaches Verfahren zur kontinuierlichen Verfolgung von hydrolytischen Enzymreaktionen Fresenius' Zeitschrift FüR Analytische Chemie. 252: 194-197. DOI: 10.1007/Bf00546382  0.349
1969 Harzer K, Jatzkewitz H, Sandhoff K. Incorporation of labelled glucose into the individual major gangliosides of the brain of young rats. Journal of Neurochemistry. 16: 1279-82. PMID 5803801 DOI: 10.1111/J.1471-4159.1969.Tb05977.X  0.623
1969 Sandhoff K, Jatzkewitz H, Peters G. [Infantile amaurotic idiocy and related forms as ganglioside storage diseases]. Die Naturwissenschaften. 56: 356-62. PMID 5360899 DOI: 10.1007/Bf00596925  0.638
1968 Sandhoff K, Andreae U, Jatzkewitz H. Deficient hexosaminidase activity in an exceptional case of Tay-Sachs disease with additional storage of kidney globoside in visceral organs. Pathologia Europaea. 3: 278-85. PMID 5688464  0.613
1968 Pilz H, Müller D, Sandhoff K, ter Meulen V. [Tay-Sachs disease with hexosaminidase deficiency. Clinical, morphological and biochemical findings in a case with visceral storage of renal globosides]. Deutsche Medizinische Wochenschrift (1946). 93: 1833-9 passim. PMID 5679107 DOI: 10.1055/s-0028-1110836  0.225
1968 Wässle W, Sandhoff K. [Appratus for the loading of samples on to thin layer chromatography plates]. Journal of Chromatography. 34: 357-63. PMID 5655904 DOI: 10.1016/0021-9673(68)80067-6  0.182
1968 Sandhoff K, Andreae U, Jatzkewitz H. Deficient hexozaminidase activity in an exceptional case of Tay-Sachs disease with additional storage of kidney globoside in visceral organs. Life Sciences. 7: 283-8. PMID 5651108 DOI: 10.1016/0024-3205(68)90024-6  0.642
1968 SANDHOFF K. Auftrennung der Säuger-N-Acetyl-β-D-hexosaminidase in multiple Formen durch Elektrofokussierung Hoppe-Seyler´S Zeitschrift FüR Physiologische Chemie. 349: 1095-1098. DOI: 10.1515/Bchm2.1968.349.2.1095  0.344
1968 SANDHOFF K, HARZER K, JATZKEWITZ H. Densitometrische Mikrobestimmung von Gangliosiden aus dem Gesamtlipidextrakt nach Dünnschichtchromatographie Hoppe-Seyler´S Zeitschrift FüR Physiologische Chemie. 349: 283-287. DOI: 10.1515/Bchm2.1968.349.1.283  0.603
1968 Harzer K, Wässle W, Sandhoff K, Jatzkewitz H. Densitometrische Mikrobestimmung von Lipiden nach Dünnschicht-Chromatographie des Gesamtlipidextrakts Fresenius' Zeitschrift FüR Analytische Chemie. 243: 527-536. DOI: 10.1007/Bf00530741  0.608
1967 Sandhoff K, Jatkewitz H. A particle-bound sialyl lactosidoceramide splitting mammalian sialidase. Biochimica Et Biophysica Acta. 141: 442-4. PMID 6048332 DOI: 10.1016/0304-4165(67)90124-9  0.189
1966 Pilz H, Sandhoff K, Jatzkewitz H. [A disorder of ganglioside metabolism with storage of ceramide lactoside, monosialo ceramide lactoside and Tay-Sachs ganglioside in the brain]. Journal of Neurochemistry. 13: 1273-82. PMID 4164111 DOI: 10.1111/J.1471-4159.1966.Tb04290.X  0.635
1965 JATZKEWITZ H, PILZ H, SANDHOFF K. [THE QUANTITATIVE DETERMINATION OF GANGLIOSIDES AND THEIR NEURAMINIC ACID-FREE DERIVATIVES IN INFANTILE, JUVENILE AND FORMS OF AMAUROTIC IDIOCY AND AN UNUSUAL LATE INFANTILE BIOCHEMICAL FORM]. Journal of Neurochemistry. 12: 135-44. PMID 14272650 DOI: 10.1111/J.1471-4159.1965.Tb06749.X  0.66
1964 SANDHOFF K, JATZKEWITZ H. [ON THE ENZYMATIC BREAKDOWN OF N-ACETYLNEURAMINOACID-FREE GANGLIOSIDE RESIDUES (CEREMIDE OLIGOSACCHARIDES)]. Hoppe-Seyler's Zeitschrift Fur Physiologische Chemie. 338: 281-5. PMID 14330405 DOI: 10.1515/Bchm2.1964.338.1-2.281  0.64
1963 JATZKEWITZ H, SANDHOFF K. On a biochemically special form of infantile amaturotic idiocy. Biochimica Et Biophysica Acta. 70: 354-6. PMID 13957544 DOI: 10.1016/0006-3002(63)90764-9  0.642
1963 Jatzkewitz H, Sandhoff K. Preliminary noteOn a biochemically special form of infantile amaurotic idiocy Biochimica Et Biophysica Acta. 70. DOI: 10.1016/0926-6542(63)90053-2  0.642
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