| Year |
Citation |
Score |
| 2021 |
Prabhu AV, Kang I, De Pace R, Wassif CA, Fujiwara H, Kell P, Jiang X, Ory DS, Bonifacino JS, Ward ME, Porter FD. A human iPSC-derived inducible neuronal model of Niemann-Pick disease, type C1. Bmc Biology. 19: 218. PMID 34592985 DOI: 10.1186/s12915-021-01133-x |
0.386 |
|
| 2021 |
Pipalia NH, Saad SZ, Subramanian K, Cross A, Al-Motawa A, Garg K, Blagg BSJ, Neckers L, Helquist P, Wiest O, Ory DS, Maxfield FR. HSP90 inhibitors reduce cholesterol storage in Niemann-Pick type C1 mutant fibroblasts. Journal of Lipid Research. 100114. PMID 34481829 DOI: 10.1016/j.jlr.2021.100114 |
0.378 |
|
| 2021 |
Krishnan K, Qian M, Feltes M, Chen ZW, Gale S, Wang L, Sugasawa Y, Reichert DE, Schaffer JE, Ory DS, Evers AS, Covey DF. Validation of Trifluoromethylphenyl Diazirine Cholesterol Analogues As Cholesterol Mimetics and Photolabeling Reagents. Acs Chemical Biology. PMID 34355883 DOI: 10.1021/acschembio.1c00364 |
0.374 |
|
| 2021 |
Kutchukian C, Vivas O, Casas M, Jones JG, Tiscione SA, Simó S, Ory DS, Dixon RE, Dickson EJ. NPC1 regulates the distribution of phosphatidylinositol 4-kinases at Golgi and lysosomal membranes. The Embo Journal. e105990. PMID 34019311 DOI: 10.15252/embj.2020105990 |
0.426 |
|
| 2021 |
Xiao X, Kim Y, Romartinez-Alonso B, Sirvydis K, Ory DS, Schwabe JWR, Jung ME, Tontonoz P. Selective Aster inhibitors distinguish vesicular and nonvesicular sterol transport mechanisms. Proceedings of the National Academy of Sciences of the United States of America. 118. PMID 33376205 DOI: 10.1073/pnas.2024149118 |
0.302 |
|
| 2020 |
Bradbury AM, Bagel JH, Nguyen D, Lykken EA, Pesayco Salvador J, Jiang X, Swain GP, Assenmacher CA, Hendricks IJ, Miyadera K, Hess RS, Ostrager A, ODonnell P, Sands MS, Ory DS, et al. Krabbe disease successfully treated via monotherapy of intrathecal gene therapy. The Journal of Clinical Investigation. PMID 32773406 DOI: 10.1172/Jci133953 |
0.3 |
|
| 2020 |
Peterson LR, Jiang X, Chen L, Goldberg AC, Farmer MS, Ory DS, Schaffer JE. Alterations in plasma triglycerides and ceramides: links with cardiac function in humans with type 2 diabetes. Journal of Lipid Research. PMID 32393551 DOI: 10.1194/Jlr.Ra120000669 |
0.35 |
|
| 2020 |
Sidhu R, Kell P, Dietzen DJ, Farhat NY, Do AND, Porter FD, Berry-Kravis E, Vite CH, Reunert J, Marquardt T, Giugliani R, Lourenço CM, Bodamer O, Wang RY, Plummer E, ... ... Ory DS, et al. Application of N-palmitoyl-O-phosphocholineserine for diagnosis and assessment of response to treatment in Niemann-Pick type C disease. Molecular Genetics and Metabolism. PMID 32033912 DOI: 10.1016/J.Ymgme.2020.01.007 |
0.354 |
|
| 2020 |
Feltes M, Gale SE, Moores S, Ory DS, Schaffer JE. Monitoring the itinerary of lysosomal cholesterol in Niemann-Pick Type C1-deficient cells after cyclodextrin treatment. Journal of Lipid Research. PMID 31988149 DOI: 10.1194/Jlr.Ra119000571 |
0.52 |
|
| 2020 |
Ou L, Przybilla MJ, Tăbăran AF, Overn P, O'Sullivan MG, Jiang X, Sidhu R, Kell PJ, Ory DS, Whitley CB. A novel gene editing system to treat both Tay-Sachs and Sandhoff diseases. Gene Therapy. PMID 31896760 DOI: 10.1038/S41434-019-0120-5 |
0.304 |
|
| 2019 |
Corado CR, Pinkstaff J, Jiang X, Galban EM, Fisher SJ, Scholler O, Russell C, Bagel JH, ODonnell PA, Ory DS, Vite CH, Bradbury AM. Cerebrospinal fluid and serum glycosphingolipid biomarkers in canine globoid cell leukodystrophy (Krabbe Disease). Molecular and Cellular Neurosciences. 103451. PMID 31794880 DOI: 10.1016/J.Mcn.2019.103451 |
0.332 |
|
| 2019 |
Aflaki E, Stubblefield BK, McGlinchey RP, McMahon B, Ory DS, Sidransky E. A characterization of Gaucher iPS-derived astrocytes: Potential implications for Parkinson's disease. Neurobiology of Disease. 104647. PMID 31669751 DOI: 10.1016/J.Nbd.2019.104647 |
0.322 |
|
| 2019 |
Tiscione SA, Vivas O, Ginsburg KS, Bers DM, Ory DS, Santana LF, Dixon RE, Dickson EJ. Disease-associated mutations in Niemann-Pick type C1 alter ER calcium signaling and neuronal plasticity. The Journal of Cell Biology. PMID 31601621 DOI: 10.1083/Jcb.201903018 |
0.33 |
|
| 2019 |
Lim CY, Davis OB, Shin HR, Zhang J, Berdan CA, Jiang X, Counihan JL, Ory DS, Nomura DK, Zoncu R. ER-lysosome contacts enable cholesterol sensing by mTORC1 and drive aberrant growth signalling in Niemann-Pick type C. Nature Cell Biology. PMID 31548609 DOI: 10.1038/S41556-019-0391-5 |
0.517 |
|
| 2019 |
Petersen D, Reinholdt P, Szomek M, Hansen SK, Poongavanam V, Dupont A, Heegaard CW, Krishnan K, Fujiwara H, Covey DF, Ory DS, Kongsted J, Wüstner D. Binding and intracellular transport of 25-hydroxycholesterol by Niemann-Pick C2 protein. Biochimica Et Biophysica Acta. Biomembranes. 183063. PMID 31521631 DOI: 10.1016/J.Bbamem.2019.183063 |
0.437 |
|
| 2019 |
Sidhu R, Mondjinou Y, Qian M, Song H, Kumar AB, Hong X, Hsu FF, Dietzen DJ, Yanjanin NM, Porter FD, Berry-Kravis E, Vite CH, Gelb MH, Schaffer JE, Ory DS, et al. N-Acyl-O-phosphocholineserines: structures of a novel class of lipids that are biomarkers for Niemann-Pick C1 disease. Journal of Lipid Research. PMID 31201291 DOI: 10.1194/Jlr.Ra119000157 |
0.409 |
|
| 2019 |
Vivas O, Tiscione SA, Dixon RE, Ory DS, Dickson EJ. Niemann-Pick Type C Disease Reveals a Link between Lysosomal Cholesterol and PtdIns(4,5)P That Regulates Neuronal Excitability. Cell Reports. 27: 2636-2648.e4. PMID 31141688 DOI: 10.1016/J.Celrep.2019.04.099 |
0.402 |
|
| 2019 |
Murphy JT, Liu H, Ma X, Shaver A, Egan BM, Oh C, Boyko A, Mazer T, Ang S, Khopkar R, Javaheri A, Kumar S, Jiang X, Ory D, Mani K, et al. Simple nutrients bypass the requirement for HLH-30 in coupling lysosomal nutrient sensing to survival. Plos Biology. 17: e3000245. PMID 31086360 DOI: 10.1371/Journal.Pbio.3000245 |
0.351 |
|
| 2019 |
Davidson J, Molitor E, Moores S, Gale SE, Subramanian K, Jiang X, Sidhu R, Kell P, Zhang J, Fujiwara H, Davidson C, Helquist P, Melancon BJ, Grigalunas M, Liu G, ... ... Ory DS, et al. 2-Hydroxypropyl-β-cyclodextrin is the active component in a triple combination formulation for treatment of Niemann-Pick C1 disease. Biochimica Et Biophysica Acta. Molecular and Cell Biology of Lipids. PMID 31051283 DOI: 10.1016/J.Bbalip.2019.04.011 |
0.405 |
|
| 2019 |
Feltes M, Moores S, Gale SE, Krishnan K, Mydock-McGrane L, Covey DF, Ory DS, Schaffer JE. Synthesis and characterization of diazirine alkyne probes for the study of intracellular cholesterol trafficking. Journal of Lipid Research. PMID 30617147 DOI: 10.1194/Jlr.D091470 |
0.546 |
|
| 2018 |
Galassi TV, Jena PV, Shah J, Ao G, Molitor E, Bram Y, Frankel A, Park J, Jessurun J, Ory DS, Haimovitz-Friedman A, Roxbury D, Mittal J, Zheng M, Schwartz RE, et al. An optical nanoreporter of endolysosomal lipid accumulation reveals enduring effects of diet on hepatic macrophages in vivo. Science Translational Medicine. 10. PMID 30282694 DOI: 10.1126/Scitranslmed.Aar2680 |
0.438 |
|
| 2018 |
Vu M, Li R, Baskfield A, Lu B, Farkhondeh A, Gorshkov K, Motabar O, Beers J, Chen G, Zou J, Espejo-Mojica AJ, Rodríguez-López A, Alméciga-Díaz CJ, Barrera LA, Jiang X, ... Ory DS, et al. Neural stem cells for disease modeling and evaluation of therapeutics for Tay-Sachs disease. Orphanet Journal of Rare Diseases. 13: 152. PMID 30220252 DOI: 10.1186/S13023-018-0886-3 |
0.314 |
|
| 2018 |
Ban N, Lee TJ, Sene A, Choudhary M, Lekwuwa M, Dong Z, Santeford A, Lin JB, Malek G, Ory DS, Apte RS. Impaired monocyte cholesterol clearance initiates age-related retinal degeneration and vision loss. Jci Insight. 3. PMID 30185655 DOI: 10.1172/Jci.Insight.120824 |
0.446 |
|
| 2018 |
Jiang X, Sidhu R, Orsini JJ, Farhat NY, Porter FD, Berry-Kravis E, Schaffer JE, Ory DS. Diagnosis of niemann-pick C1 by measurement of bile acid biomarkers in archived newborn dried blood spots. Molecular Genetics and Metabolism. PMID 30172462 DOI: 10.1016/J.Ymgme.2018.08.007 |
0.336 |
|
| 2018 |
Ban N, Lee TJ, Sene A, Dong Z, Santeford A, Lin JB, Ory DS, Apte RS. Disrupted Cholesterol Metabolism Promotes Age-Related Photoreceptor Neurodegeneration. Journal of Lipid Research. PMID 29946056 DOI: 10.1194/Jlr.M084442 |
0.416 |
|
| 2018 |
Lin JB, Sene A, Santeford A, Fujiwara H, Sidhu R, Ligon MM, Shankar VA, Ban N, Mysorekar IU, Ory DS, Apte RS. Oxysterol Signatures Distinguish Age-Related Macular Degeneration from Physiologic Aging. Ebiomedicine. PMID 29903570 DOI: 10.1016/J.Ebiom.2018.05.035 |
0.315 |
|
| 2018 |
Pugach EK, Feltes M, Kaufman RJ, Ory DS, Bang AG. High-content screen for modifiers of Niemann-Pick Type C disease in patient cells. Human Molecular Genetics. PMID 29659804 DOI: 10.1093/Hmg/Ddy117 |
0.462 |
|
| 2018 |
Zhao K, van der Spoel A, Castiglioni C, Gale S, Fujiwara H, Ory DS, Ridgway ND. 19q13.12 microdeletion syndrome fibroblasts display abnormal storage of cholesterol and sphingolipids in the endo-lysosomal system. Biochimica Et Biophysica Acta. PMID 29580926 DOI: 10.1016/J.Bbadis.2018.03.020 |
0.464 |
|
| 2018 |
Berry-Kravis E, Chin J, Hoffmann A, Winston A, Stoner R, LaGorio L, Friedmann K, Hernandez M, Ory DS, Porter FD, O'Keefe JA. Long-Term Treatment of Niemann-Pick Type C1 Disease With Intrathecal 2-Hydroxypropyl-Β-Cyclodextrin. Pediatric Neurology. PMID 29429782 DOI: 10.1016/J.Pediatrneurol.2017.12.014 |
0.399 |
|
| 2018 |
Bradbury AM, Rafi MA, Bagel J, Brisson BK, Marshall MS, Pesayco Salvador J, Jiang X, Swain GP, Prociuk ML, O'Donnell P, Fitzgerald C, Ory DS, Bongarzone ER, Shelton GD, Wenger DA, et al. AAVrh10 gene therapy ameliorates central and peripheral nervous system disease in canine globoid cell leukodystrophy (Krabbe disease). Human Gene Therapy. PMID 29316812 DOI: 10.1089/Hum.2017.151 |
0.323 |
|
| 2017 |
Nåbo LJ, Modzel M, Krishnan K, Covey DF, Fujiwara H, Ory DS, Szomek M, Khandelia H, Wüstner D, Kongsted J. Structural design of intrinsically fluorescent oxysterols. Chemistry and Physics of Lipids. PMID 29287905 DOI: 10.1016/J.Chemphyslip.2017.12.005 |
0.305 |
|
| 2017 |
Li MW, Sletten AC, Lee J, Pyles KD, Matkovich SJ, Ory DS, Schaffer JE. Nuclear export factor 3 regulates localization of small nucleolar RNAs. The Journal of Biological Chemistry. PMID 29021253 DOI: 10.1074/Jbc.M117.818146 |
0.31 |
|
| 2017 |
Gray-Edwards HL, Jiang X, Randle AN, Taylor AR, Voss TL, Johnson AK, McCurdy VJ, Sena-Esteves M, Ory DS, Martin DR. Lipidomic Evaluation of Feline Neurologic Disease after AAV Gene Therapy. Molecular Therapy. Methods & Clinical Development. 6: 135-142. PMID 28808666 DOI: 10.1016/J.Omtm.2017.07.005 |
0.385 |
|
| 2017 |
Ory DS, Ottinger EA, Farhat NY, King KA, Jiang X, Weissfeld L, Berry-Kravis E, Davidson CD, Bianconi S, Keener LA, Rao R, Soldatos A, Sidhu R, Walters KA, Xu X, et al. Intrathecal 2-hydroxypropyl-β-cyclodextrin decreases neurological disease progression in Niemann-Pick disease, type C1: a non-randomised, open-label, phase 1-2 trial. Lancet (London, England). PMID 28803710 DOI: 10.1016/S0140-6736(17)31465-4 |
0.324 |
|
| 2017 |
Li R, Hao J, Fujiwara H, Xu M, Yang S, Dai S, Long Y, Swaroop M, Li C, Vu M, Marugan JJ, Ory DS, Zheng W. Analytical Characterization of Methyl-β-Cyclodextrin for Pharmacological Activity to Reduce Lysosomal Cholesterol Accumulation in Niemann-Pick Disease Type C1 Cells. Assay and Drug Development Technologies. 15: 154-166. PMID 28631941 DOI: 10.1089/Adt.2017.774 |
0.406 |
|
| 2017 |
Dai S, Dulcey AE, Hu X, Wassif CA, Porter FD, Austin CP, Ory DS, Marugan J, Zheng W. Methyl-β-cyclodextrin restores impaired autophagy flux in Niemann-Pick C1-deficient cells through activation of AMPK. Autophagy. 0. PMID 28613987 DOI: 10.1080/15548627.2017.1329081 |
0.424 |
|
| 2017 |
Castellano BM, Thelen AM, Moldavski O, Feltes M, van der Welle RE, Mydock-McGrane L, Jiang X, van Eijkeren RJ, Davis OB, Louie SM, Perera RM, Covey DF, Nomura DK, Ory DS, Zoncu R. Lysosomal cholesterol activates mTORC1 via an SLC38A9-Niemann-Pick C1 signaling complex. Science (New York, N.Y.). 355: 1306-1311. PMID 28336668 DOI: 10.1126/Science.Aag1417 |
0.469 |
|
| 2016 |
Munkacsi AB, Hammond N, Schneider RT, Senanayake DS, Higaki K, Lagutin K, Bloor SJ, Ory DS, Maue RA, Chen FW, Hernandez-Ono A, Dahlson N, Repa JJ, Ginsberg HN, Ioannou YA, et al. Normalization of Hepatic Homeostasis in the Npc1nmf164 Mouse Model of Niemann-Pick Type C Disease Treated with the Histone Deacetylase Inhibitor Vorinostat. The Journal of Biological Chemistry. PMID 28031458 DOI: 10.1074/Jbc.M116.770578 |
0.404 |
|
| 2016 |
Walkley SU, Davidson CD, Jacoby J, Marella PD, Ottinger EA, Austin CP, Porter FD, Vite CH, Ory DS. Fostering collaborative research for rare genetic disease: the example of niemann-pick type C disease. Orphanet Journal of Rare Diseases. 11: 161. PMID 27903269 DOI: 10.1186/S13023-016-0540-X |
0.301 |
|
| 2016 |
Wei X, Song H, Yin L, Rizzo MG, Sidhu R, Covey DF, Ory DS, Semenkovich CF. Fatty acid synthesis configures the plasma membrane for inflammation in diabetes. Nature. PMID 27806377 DOI: 10.1038/Nature20117 |
0.405 |
|
| 2016 |
Bradbury AM, Bagel JH, Jiang X, Swain GP, Prociuk ML, Fitzgerald CA, O'Donnell PA, Braund KG, Ory DS, Vite CH. Clinical, electrophysiological, and biochemical markers of peripheral and central nervous system disease in canine globoid cell leukodystrophy (Krabbe's disease). Journal of Neuroscience Research. 94: 1007-17. PMID 27638585 DOI: 10.1002/Jnr.23838 |
0.327 |
|
| 2016 |
Westbroek W, Nguyen M, Siebert M, Lindstrom T, Burnett RA, Aflaki E, Jung O, Tamargo R, Rodriguez-Gil JL, Acosta W, Hendrix A, Behre B, Tayebi N, Fujiwara H, Sidhu R, ... ... Ory DS, et al. A new glucocerebrosidase-deficient neuronal cell model provides a tool to probe pathophysiology and therapeutics for Gaucher disease. Disease Models & Mechanisms. 9: 769-778. PMID 27482815 DOI: 10.1242/Dmm.024588 |
0.316 |
|
| 2016 |
Aflaki E, Borger DK, Moaven N, Stubblefield BK, Rogers SA, Patnaik S, Schoenen FJ, Westbroek W, Zheng W, Sullivan P, Fujiwara H, Sidhu R, Khaliq ZM, Lopez GJ, Goldstein DS, ... Ory DS, et al. A New Glucocerebrosidase Chaperone Reduces α-Synuclein and Glycolipid Levels in iPSC-Derived Dopaminergic Neurons from Patients with Gaucher Disease and Parkinsonism. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 36: 7441-52. PMID 27413154 DOI: 10.1523/Jneurosci.0636-16.2016 |
0.308 |
|
| 2016 |
Ebrahimi-Fakhari D, Wahlster L, Bartz F, Werenbeck-Ueding J, Praggastis M, Zhang J, Joggerst-Thomalla B, Theiss S, Grimm D, Ory DS, Runz H. Reduction of TMEM97 increases NPC1 protein levels and restores cholesterol trafficking in Niemann-Pick type C1 disease cells. Human Molecular Genetics. PMID 27378690 DOI: 10.1093/Hmg/Ddw204 |
0.788 |
|
| 2016 |
Jiang X, Sidhu R, Mydock-McGrane L, Hsu FF, Covey DF, Scherrer DE, Earley B, Gale SE, Farhat NY, Porter FD, Dietzen DJ, Orsini JJ, Berry-Kravis E, Zhang X, Reunert J, ... ... Ory DS, et al. Development of a bile acid-based newborn screen for Niemann-Pick disease type C. Science Translational Medicine. 8: 337ra63. PMID 27147587 DOI: 10.1126/Scitranslmed.Aaf2326 |
0.328 |
|
| 2016 |
Chung C, Puthanveetil P, Ory DS, Lieberman AP. Genetic and pharmacological evidence implicates cathepsins in Niemann-Pick C cerebellar degeneration. Human Molecular Genetics. PMID 26908626 DOI: 10.1093/Hmg/Ddw025 |
0.402 |
|
| 2016 |
Newberry EP, Xie Y, Kennedy S, Graham M, Crooke R, Jiang H, Chen A, Ory DS, Davidson NO. 288 Targeting L-Fabp attenuates Steatosis and Prevents Hepatic Fibrosis in Mice With Liver Specific Microsomal Triglyceride Transfer Protein Knockout (Mttp-LKO) By Changing Lipid Droplet Metabolism and Inducing Autophagy Gastroenterology. 150: S1025-S1026. DOI: 10.1016/S0016-5085(16)33472-2 |
0.321 |
|
| 2015 |
Iaea DB, Gale SE, Bielska AA, Krishnan K, Fujiwara H, Jiang H, Maxfield FR, Schlesinger PH, Covey DF, Schaffer JE, Ory DS. A novel intrinsically fluorescent probe for study of uptake and trafficking of 25-hydroxycholesterol. Journal of Lipid Research. PMID 26497473 DOI: 10.1194/Jlr.D064287 |
0.816 |
|
| 2015 |
Praggastis M, Tortelli B, Zhang J, Fujiwara H, Sidhu R, Chacko A, Chen Z, Chung C, Lieberman AP, Sikora J, Davidson C, Walkley SU, Pipalia NH, Maxfield FR, Schaffer JE, ... Ory DS, et al. A Murine Niemann-Pick C1 I1061T Knock-In Model Recapitulates the Pathological Features of the Most Prevalent Human Disease Allele. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 35: 8091-106. PMID 26019327 DOI: 10.1523/Jneurosci.4173-14.2015 |
0.757 |
|
| 2015 |
Jinn S, Brandis KA, Ren A, Chacko A, Dudley-Rucker N, Gale SE, Sidhu R, Fujiwara H, Jiang H, Olsen BN, Schaffer JE, Ory DS. snoRNA U17 regulates cellular cholesterol trafficking. Cell Metabolism. 21: 855-67. PMID 25980348 DOI: 10.1016/J.Cmet.2015.04.010 |
0.783 |
|
| 2015 |
Hawkins-Salsbury JA, Shea L, Jiang X, Hunter DA, Guzman AM, Reddy AS, Qin EY, Li Y, Gray SJ, Ory DS, Sands MS. Mechanism-based combination treatment dramatically increases therapeutic efficacy in murine globoid cell leukodystrophy. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 35: 6495-505. PMID 25904800 DOI: 10.1523/Jneurosci.4199-14.2015 |
0.355 |
|
| 2015 |
Wassif CA, Cross JL, Iben J, Sanchez-Pulido L, Cougnoux A, Platt FM, Ory DS, Ponting CP, Bailey-Wilson JE, Biesecker LG, Porter FD. High incidence of unrecognized visceral/neurological late-onset Niemann-Pick disease, type C1, predicted by analysis of massively parallel sequencing data sets. Genetics in Medicine : Official Journal of the American College of Medical Genetics. PMID 25764212 DOI: 10.1038/Gim.2015.25 |
0.349 |
|
| 2015 |
Vite CH, Bagel JH, Swain GP, Prociuk M, Sikora TU, Stein VM, O'Donnell P, Ruane T, Ward S, Crooks A, Li S, Mauldin E, Stellar S, De Meulder M, Kao ML, ... Ory DS, et al. Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease. Science Translational Medicine. 7: 276ra26. PMID 25717099 DOI: 10.1126/Scitranslmed.3010101 |
0.393 |
|
| 2015 |
Jinn S, Brandis KA, Ren A, Chacko A, Dudley-Rucker N, Gale SE, Sidhu R, Fujiwara H, Jiang H, Olsen BN, Schaffer JE, Ory DS. SnoRNA U17 regulates cellular cholesterol trafficking Cell Metabolism. 21: 855-867. DOI: 10.1016/j.cmet.2015.04.010 |
0.797 |
|
| 2014 |
Tortelli B, Fujiwara H, Bagel JH, Zhang J, Sidhu R, Jiang X, Yanjanin NM, Shankar RK, Carillo-Carasco N, Heiss J, Ottinger E, Porter FD, Schaffer JE, Vite CH, Ory DS. Cholesterol homeostatic responses provide biomarkers for monitoring treatment for the neurodegenerative disease Niemann-Pick C1 (NPC1). Human Molecular Genetics. 23: 6022-33. PMID 24964810 DOI: 10.1093/Hmg/Ddu331 |
0.394 |
|
| 2014 |
Daily MD, Olsen BN, Schlesinger PH, Ory DS, Baker NA. Improved Coarse-Grained Modeling of Cholesterol-Containing Lipid Bilayers. Journal of Chemical Theory and Computation. 10: 2137-2150. PMID 24910542 DOI: 10.1021/Ct401028G |
0.49 |
|
| 2014 |
Bielska AA, Olsen BN, Gale SE, Mydock-McGrane L, Krishnan K, Baker NA, Schlesinger PH, Covey DF, Ory DS. Side-chain oxysterols modulate cholesterol accessibility through membrane remodeling. Biochemistry. 53: 3042-51. PMID 24758724 DOI: 10.1021/Bi5000096 |
0.807 |
|
| 2014 |
Peyrot SM, Nachtergaele S, Luchetti G, Mydock-McGrane LK, Fujiwara H, Scherrer D, Jallouk A, Schlesinger PH, Ory DS, Covey DF, Rohatgi R. Tracking the subcellular fate of 20(s)-hydroxycholesterol with click chemistry reveals a transport pathway to the Golgi. The Journal of Biological Chemistry. 289: 11095-110. PMID 24596093 DOI: 10.1074/Jbc.M113.540351 |
0.447 |
|
| 2014 |
Hung YH, Faux NG, Killilea DW, Yanjanin N, Firnkes S, Volitakis I, Ganio G, Walterfang M, Hastings C, Porter FD, Ory DS, Bush AI. Altered transition metal homeostasis in Niemann-Pick disease, type C1. Metallomics : Integrated Biometal Science. 6: 542-53. PMID 24343124 DOI: 10.1039/C3Mt00308F |
0.341 |
|
| 2014 |
Ottinger EA, Kao ML, Carrillo-Carrasco N, Yanjanin N, Shankar RK, Janssen M, Brewster M, Scott I, Xu X, Cradock J, Terse P, Dehdashti SJ, Marugan J, Zheng W, Portilla L, ... ... Ory DS, et al. Collaborative development of 2-hydroxypropyl-β-cyclodextrin for the treatment of Niemann-Pick type C1 disease. Current Topics in Medicinal Chemistry. 14: 330-9. PMID 24283970 DOI: 10.2174/1568026613666131127160118 |
0.353 |
|
| 2014 |
Speak AO, Te Vruchte D, Davis LC, Morgan AJ, Smith DA, Yanjanin NM, Simmons L, Hartung R, Runz H, Mengel E, Beck M, Imrie J, Jacklin E, Wraith JE, Hendriksz C, ... ... Ory DS, et al. Altered distribution and function of natural killer cells in murine and human Niemann-Pick disease type C1. Blood. 123: 51-60. PMID 24235134 DOI: 10.1182/Blood-2013-03-488692 |
0.352 |
|
| 2014 |
Tortelli B, Fujiwara H, Bagel JH, Zhang J, Sidhu R, Jiang X, Yanjanin NM, Shankar RK, Carillo-Carasco N, Heiss J, Ottinger E, Porter FD, Schaffer JE, Vite CH, Ory DS. Cholesterol homeostatic responses provide biomarkers for monitoring treatment for the neurodegenerative disease Niemann-Pick C1 (NPC1) Human Molecular Genetics. 23: 6022-6033. DOI: 10.1093/hmg/ddu331 |
0.309 |
|
| 2013 |
Brandis KA, Gale S, Jinn S, Langmade SJ, Dudley-Rucker N, Jiang H, Sidhu R, Ren A, Goldberg A, Schaffer JE, Ory DS. Box C/D small nucleolar RNA (snoRNA) U60 regulates intracellular cholesterol trafficking. The Journal of Biological Chemistry. 288: 35703-13. PMID 24174535 DOI: 10.1074/Jbc.M113.488577 |
0.801 |
|
| 2013 |
Olsen BN, Bielska AA, Lee T, Daily MD, Covey DF, Schlesinger PH, Baker NA, Ory DS. The structural basis of cholesterol accessibility in membranes. Biophysical Journal. 105: 1838-47. PMID 24138860 DOI: 10.1016/J.Bpj.2013.08.042 |
0.812 |
|
| 2013 |
Hawkins-Salsbury JA, Parameswar AR, Jiang X, Schlesinger PH, Bongarzone E, Ory DS, Demchenko AV, Sands MS. Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture. Journal of Lipid Research. 54: 3303-11. PMID 24006512 DOI: 10.1016/J.Ymgme.2012.11.108 |
0.431 |
|
| 2013 |
Fan M, Sidhu R, Fujiwara H, Tortelli B, Zhang J, Davidson C, Walkley SU, Bagel JH, Vite C, Yanjanin NM, Porter FD, Schaffer JE, Ory DS. Identification of Niemann-Pick C1 disease biomarkers through sphingolipid profiling. Journal of Lipid Research. 54: 2800-14. PMID 23881911 DOI: 10.1194/Jlr.M040618 |
0.397 |
|
| 2013 |
Bauer P, Balding DJ, Klünemann HH, Linden DE, Ory DS, Pineda M, Priller J, Sedel F, Muller A, Chadha-Boreham H, Welford RW, Strasser DS, Patterson MC. Genetic screening for Niemann-Pick disease type C in adults with neurological and psychiatric symptoms: findings from the ZOOM study. Human Molecular Genetics. 22: 4349-56. PMID 23773996 DOI: 10.1093/Hmg/Ddt284 |
0.317 |
|
| 2013 |
Fu R, Wassif CA, Yanjanin NM, Watkins-Chow DE, Baxter LL, Incao A, Liscum L, Sidhu R, Firnkes S, Graham M, Ory DS, Porter FD, Pavan WJ. Efficacy of N-acetylcysteine in phenotypic suppression of mouse models of Niemann-Pick disease, type C1. Human Molecular Genetics. 22: 3508-23. PMID 23666527 DOI: 10.1093/Hmg/Ddt206 |
0.386 |
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| 2013 |
Sene A, Khan AA, Cox D, Nakamura RE, Santeford A, Kim BM, Sidhu R, Onken MD, Harbour JW, Hagbi-Levi S, Chowers I, Edwards PA, Baldan A, Parks JS, Ory DS, et al. Impaired cholesterol efflux in senescent macrophages promotes age-related macular degeneration. Cell Metabolism. 17: 549-61. PMID 23562078 DOI: 10.1016/J.Cmet.2013.03.009 |
0.392 |
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| 2013 |
Stampfer M, Theiss S, Amraoui Y, Jiang X, Keller S, Ory DS, Mengel E, Fischer C, Runz H. Niemann-Pick disease type C clinical database: cognitive and coordination deficits are early disease indicators. Orphanet Journal of Rare Diseases. 8: 35. PMID 23433426 DOI: 10.1186/1750-1172-8-35 |
0.303 |
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| 2013 |
Schilling JD, Machkovech HM, He L, Sidhu R, Fujiwara H, Weber K, Ory DS, Schaffer JE. Palmitate and lipopolysaccharide trigger synergistic ceramide production in primary macrophages. The Journal of Biological Chemistry. 288: 2923-32. PMID 23250746 DOI: 10.1074/Jbc.M112.419978 |
0.312 |
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| 2013 |
Xu M, Liu K, Swaroop M, Porter FD, Sidhu R, Firnkes S, Ory DS, Marugan JJ, Xiao J, Southall N, Pavan WJ, Davidson C, Walkley SU, Remaley AT, Baxa U, et al. δ-Tocopherol reduces lipid accumulation in Niemann-Pick type C1 and Wolman cholesterol storage disorders (Journal of Biological Chemistry (2012) 287, (39349-39360)) Journal of Biological Chemistry. 288: 296. DOI: 10.1074/Jbc.A112.357707 |
0.453 |
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| 2013 |
Olsen BN, Bielska A, Daily M, Schlesinger P, Baker N, Ory D. The Structural Basis of Cholesterol Activation in Membranes Biophysical Journal. 104: 662a. DOI: 10.1016/J.Bpj.2012.11.3657 |
0.81 |
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| 2013 |
Daily MD, Olsen BN, Schlesinger PH, Ory DS, Baker NA. Improved Coarse-Grained Modeling of Cholesterol Activation in Lipid Bilayers Biophysical Journal. 104: 590a-591a. DOI: 10.1016/J.Bpj.2012.11.3281 |
0.43 |
|
| 2012 |
Xu M, Liu K, Swaroop M, Porter FD, Sidhu R, Firnkes S, Finkes S, Ory DS, Marugan JJ, Xiao J, Southall N, Pavan WJ, Davidson C, Walkley SU, Remaley AT, et al. δ-Tocopherol reduces lipid accumulation in Niemann-Pick type C1 and Wolman cholesterol storage disorders. The Journal of Biological Chemistry. 287: 39349-60. PMID 23035117 DOI: 10.1074/Jbc.M112.357707 |
0.52 |
|
| 2012 |
Scruggs BS, Michel CI, Ory DS, Schaffer JE. SmD3 regulates intronic noncoding RNA biogenesis. Molecular and Cellular Biology. 32: 4092-103. PMID 22869524 DOI: 10.1128/Mcb.00022-12 |
0.363 |
|
| 2012 |
Cluzeau CV, Watkins-Chow DE, Fu R, Borate B, Yanjanin N, Dail MK, Davidson CD, Walkley SU, Ory DS, Wassif CA, Pavan WJ, Porter FD. Microarray expression analysis and identification of serum biomarkers for Niemann-Pick disease, type C1. Human Molecular Genetics. 21: 3632-46. PMID 22619379 DOI: 10.1093/Hmg/Dds193 |
0.417 |
|
| 2012 |
Xie Y, Kennedy S, Sidhu R, Luo J, Ory DS, Davidson NO. Liver X receptor agonist modulation of cholesterol efflux in mice with intestine-specific deletion of microsomal triglyceride transfer protein. Arteriosclerosis, Thrombosis, and Vascular Biology. 32: 1624-31. PMID 22580900 DOI: 10.1161/Atvbaha.112.246066 |
0.382 |
|
| 2012 |
Bielska AA, Schlesinger P, Covey DF, Ory DS. Oxysterols as non-genomic regulators of cholesterol homeostasis. Trends in Endocrinology and Metabolism: Tem. 23: 99-106. PMID 22244444 DOI: 10.1016/J.Tem.2011.12.002 |
0.823 |
|
| 2012 |
Olsen BN, Schlesinger PH, Ory DS, Baker NA. Side-chain oxysterols: from cells to membranes to molecules. Biochimica Et Biophysica Acta. 1818: 330-6. PMID 21745458 DOI: 10.1016/J.Bbamem.2011.06.014 |
0.53 |
|
| 2011 |
Bowden KL, Bilbey NJ, Bilawchuk LM, Boadu E, Sidhu R, Ory DS, Du H, Chan T, Francis GA. Lysosomal acid lipase deficiency impairs regulation of ABCA1 gene and formation of high density lipoproteins in cholesteryl ester storage disease. The Journal of Biological Chemistry. 286: 30624-35. PMID 21757691 DOI: 10.1074/Jbc.M111.274381 |
0.513 |
|
| 2011 |
Jiang X, Sidhu R, Porter FD, Yanjanin NM, Speak AO, te Vruchte DT, Platt FM, Fujiwara H, Scherrer DE, Zhang J, Dietzen DJ, Schaffer JE, Ory DS. A sensitive and specific LC-MS/MS method for rapid diagnosis of Niemann-Pick C1 disease from human plasma. Journal of Lipid Research. 52: 1435-45. PMID 21518695 DOI: 10.1194/Jlr.D015735 |
0.322 |
|
| 2011 |
Bielska AA, Ory DS, Covey DF. Synthesis of the enantiomer of the oxysterol-antagonist LY295427. Steroids. 76: 986-90. PMID 21470559 DOI: 10.1016/J.Steroids.2011.03.008 |
0.816 |
|
| 2011 |
Olsen BN, Schlesinger PH, Ory DS, Baker NA. 25-Hydroxycholesterol increases the availability of cholesterol in phospholipid membranes. Biophysical Journal. 100: 948-56. PMID 21320439 DOI: 10.1016/J.Bpj.2010.12.3728 |
0.529 |
|
| 2011 |
Vite C, Mauldin E, Ward S, Stein V, Prociuk M, Haskins ME, Strattan R, Kao M, Ory D, Walkley SU, Vanier MT. Intrathecal cyclodextrin therapy of feline Niemann-Pick Type C disease Molecular Genetics and Metabolism. 102: S44. DOI: 10.1016/J.Ymgme.2010.11.148 |
0.305 |
|
| 2010 |
Porter FD, Scherrer DE, Lanier MH, Langmade SJ, Molugu V, Gale SE, Olzeski D, Sidhu R, Dietzen DJ, Fu R, Wassif CA, Yanjanin NM, Marso SP, House J, Vite C, ... ... Ory DS, et al. Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 disease. Science Translational Medicine. 2: 56ra81. PMID 21048217 DOI: 10.1126/Scitranslmed.3001417 |
0.426 |
|
| 2010 |
Marquart TJ, Allen RM, Ory DS, Baldán A. miR-33 links SREBP-2 induction to repression of sterol transporters. Proceedings of the National Academy of Sciences of the United States of America. 107: 12228-32. PMID 20566875 DOI: 10.1073/Pnas.1005191107 |
0.355 |
|
| 2010 |
Ory D, Porter F, Scherrer D, Lanier M, Langmade S, Molugu V, Gale S, Olzeski D, Sidhu R, Wassif C, Yanjanin N, Schaffer J. 99. Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann–Pick C1 disease Molecular Genetics and Metabolism. 99. DOI: 10.1016/J.Ymgme.2009.10.116 |
0.363 |
|
| 2009 |
Davidson CD, Ali NF, Micsenyi MC, Stephney G, Renault S, Dobrenis K, Ory DS, Vanier MT, Walkley SU. Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression. Plos One. 4: e6951. PMID 19750228 DOI: 10.1371/Journal.Pone.0006951 |
0.388 |
|
| 2009 |
Ory DS. Getting a "Hold" on NPC2. Cell Metabolism. 10: 161-2. PMID 19723490 DOI: 10.1016/J.Cmet.2009.08.006 |
0.443 |
|
| 2009 |
Lange Y, Steck TL, Ye J, Lanier MH, Molugu V, Ory D. Regulation of fibroblast mitochondrial 27-hydroxycholesterol production by active plasma membrane cholesterol. Journal of Lipid Research. 50: 1881-8. PMID 19401598 DOI: 10.1194/Jlr.M900116-Jlr200 |
0.513 |
|
| 2009 |
Brookheart RT, Michel CI, Listenberger LL, Ory DS, Schaffer JE. The non-coding RNA gadd7 is a regulator of lipid-induced oxidative and endoplasmic reticulum stress. The Journal of Biological Chemistry. 284: 7446-54. PMID 19150982 DOI: 10.1074/Jbc.M806209200 |
0.383 |
|
| 2009 |
Gale SE, Westover EJ, Dudley N, Krishnan K, Merlin S, Scherrer DE, Han X, Zhai X, Brockman HL, Brown RE, Covey DF, Schaffer JE, Schlesinger P, Ory DS. Side chain oxygenated cholesterol regulates cellular cholesterol homeostasis through direct sterol-membrane interactions. The Journal of Biological Chemistry. 284: 1755-64. PMID 18996837 DOI: 10.1074/Jbc.M807210200 |
0.5 |
|
| 2008 |
Zhang JR, Coleman T, Langmade SJ, Scherrer DE, Lane L, Lanier MH, Feng C, Sands MS, Schaffer JE, Semenkovich CF, Ory DS. Niemann-Pick C1 protects against atherosclerosis in mice via regulation of macrophage intracellular cholesterol trafficking. The Journal of Clinical Investigation. 118: 2281-90. PMID 18483620 DOI: 10.1172/Jci32561 |
0.481 |
|
| 2008 |
Gelsthorpe ME, Baumann N, Millard E, Gale SE, Langmade SJ, Schaffer JE, Ory DS. Niemann-Pick type C1 I1061T mutant encodes a functional protein that is selected for endoplasmic reticulum-associated degradation due to protein misfolding. The Journal of Biological Chemistry. 283: 8229-36. PMID 18216017 DOI: 10.1074/Jbc.M708735200 |
0.368 |
|
| 2008 |
Lange Y, Ory DS, Ye J, Lanier MH, Hsu FF, Steck TL. Effectors of rapid homeostatic responses of endoplasmic reticulum cholesterol and 3-hydroxy-3-methylglutaryl-CoA reductase. The Journal of Biological Chemistry. 283: 1445-55. PMID 18024962 DOI: 10.1074/Jbc.M706967200 |
0.535 |
|
| 2007 |
Ory DS. Chylomicrons and lipoprotein lipase at the endothelial surface: bound and GAG-ged? Cell Metabolism. 5: 229-31. PMID 17403366 DOI: 10.1016/J.Cmet.2007.03.003 |
0.353 |
|
| 2006 |
Borradaile NM, Han X, Harp JD, Gale SE, Ory DS, Schaffer JE. Disruption of endoplasmic reticulum structure and integrity in lipotoxic cell death. Journal of Lipid Research. 47: 2726-37. PMID 16960261 DOI: 10.1194/Jlr.M600299-Jlr200 |
0.418 |
|
| 2006 |
Langmade SJ, Gale SE, Frolov A, Mohri I, Suzuki K, Mellon SH, Walkley SU, Covey DF, Schaffer JE, Ory DS. Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C disease. Proceedings of the National Academy of Sciences of the United States of America. 103: 13807-12. PMID 16940355 DOI: 10.1073/Pnas.0606218103 |
0.345 |
|
| 2006 |
Gale SE, Frolov A, Han X, Bickel PE, Cao L, Bowcock A, Schaffer JE, Ory DS. A regulatory role for 1-acylglycerol-3-phosphate-O-acyltransferase 2 in adipocyte differentiation. The Journal of Biological Chemistry. 281: 11082-9. PMID 16495223 DOI: 10.1074/Jbc.M509612200 |
0.324 |
|
| 2006 |
Borradaile NM, Buhman KK, Listenberger LL, Magee CJ, Morimoto ET, Ory DS, Schaffer JE. A critical role for eukaryotic elongation factor 1A-1 in lipotoxic cell death. Molecular Biology of the Cell. 17: 770-8. PMID 16319173 DOI: 10.1091/Mbc.E05-08-0742 |
0.374 |
|
| 2005 |
Millard EE, Gale SE, Dudley N, Zhang J, Schaffer JE, Ory DS. The sterol-sensing domain of the Niemann-Pick C1 (NPC1) protein regulates trafficking of low density lipoprotein cholesterol. The Journal of Biological Chemistry. 280: 28581-90. PMID 15908696 DOI: 10.1074/Jbc.M414024200 |
0.485 |
|
| 2005 |
Chiu HC, Kovacs A, Blanton RM, Han X, Courtois M, Weinheimer CJ, Yamada KA, Brunet S, Xu H, Nerbonne JM, Welch MJ, Fettig NM, Sharp TL, Sambandam N, Olson KM, ... Ory DS, et al. Transgenic expression of fatty acid transport protein 1 in the heart causes lipotoxic cardiomyopathy. Circulation Research. 96: 225-33. PMID 15618539 DOI: 10.1161/01.Res.0000154079.20681.B9 |
0.324 |
|
| 2004 |
Ory DS. Nuclear receptor signaling in the control of cholesterol homeostasis: have the orphans found a home? Circulation Research. 95: 660-70. PMID 15459087 DOI: 10.1161/01.Res.0000143422.83209.Be |
0.435 |
|
| 2004 |
Ory DS. The niemann-pick disease genes; regulators of cellular cholesterol homeostasis. Trends in Cardiovascular Medicine. 14: 66-72. PMID 15030792 DOI: 10.1016/J.Tcm.2003.12.003 |
0.549 |
|
| 2004 |
Zhang J, Dudley-Rucker N, Crowley JR, Lopez-Perez E, Issandou M, Schaffer JE, Ory DS. The steroidal analog GW707 activates the SREBP pathway through disruption of intracellular cholesterol trafficking. Journal of Lipid Research. 45: 223-31. PMID 14617742 DOI: 10.1194/Jlr.M300409-Jlr200 |
0.511 |
|
| 2003 |
Frolov A, Zielinski SE, Crowley JR, Dudley-Rucker N, Schaffer JE, Ory DS. NPC1 and NPC2 regulate cellular cholesterol homeostasis through generation of low density lipoprotein cholesterol-derived oxysterols. The Journal of Biological Chemistry. 278: 25517-25. PMID 12719428 DOI: 10.1074/Jbc.M302588200 |
0.555 |
|
| 2003 |
Listenberger LL, Han X, Lewis SE, Cases S, Farese RV, Ory DS, Schaffer JE. Triglyceride accumulation protects against fatty acid-induced lipotoxicity. Proceedings of the National Academy of Sciences of the United States of America. 100: 3077-82. PMID 12629214 DOI: 10.1073/Pnas.0630588100 |
0.373 |
|
| 2003 |
Richards MR, Listenberger LL, Kelly AA, Lewis SE, Ory DS, Schaffer JE. Oligomerization of the murine fatty acid transport protein 1. The Journal of Biological Chemistry. 278: 10477-83. PMID 12533547 DOI: 10.1074/Jbc.M212469200 |
0.336 |
|
| 2001 |
Frolov A, Srivastava K, Daphna-Iken D, Traub LM, Schaffer JE, Ory DS. Cholesterol Overload Promotes Morphogenesis of a Niemann-Pick C (NPC)-like Compartment Independent of Inhibition of NPC1 or HE1/NPC2 Function Journal of Biological Chemistry. 276: 46414-46421. PMID 11571306 DOI: 10.1074/Jbc.M108099200 |
0.523 |
|
| 2001 |
Lewis SE, Listenberger LL, Ory DS, Schaffer JE. Membrane topology of the murine fatty acid transport protein 1. The Journal of Biological Chemistry. 276: 37042-50. PMID 11470793 DOI: 10.1074/Jbc.M105556200 |
0.344 |
|
| 2001 |
Listenberger LL, Ory DS, Schaffer JE. Palmitate-induced apoptosis can occur through a ceramide-independent pathway. The Journal of Biological Chemistry. 276: 14890-5. PMID 11278654 DOI: 10.1074/Jbc.M010286200 |
0.335 |
|
| 2000 |
Ory DS. Niemann-Pick type C: A disorder of cellular cholesterol trafficking Biochimica Et Biophysica Acta - Molecular and Cell Biology of Lipids. 1529: 331-339. PMID 11111100 DOI: 10.1016/S1388-1981(00)00158-X |
0.42 |
|
| 2000 |
Millard EE, Srivastava K, Traub LM, Schaffer JE, Ory DS. Niemann-pick type C1 (NPC1) overexpression alters cellular cholesterol homeostasis. The Journal of Biological Chemistry. 275: 38445-51. PMID 10964915 DOI: 10.1074/Jbc.M003180200 |
0.559 |
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