| Year |
Citation |
Score |
| 2016 |
Long Y, Xu M, Li R, Dai S, Beers J, Chen G, Soheilian F, Baxa U, Wang M, Marugan JJ, Muro S, Li Z, Brady R, Zheng W. Induced Pluripotent Stem Cells for Disease Modeling and Evaluation of Therapeutics for Niemann-Pick Disease Type A. Stem Cells Translational Medicine. PMID 27484861 DOI: 10.5966/Sctm.2015-0373 |
0.333 |
|
| 2016 |
Kaneski CR, Brady RO, Hanover JA, Schueler UH. Development of a model system for neuronal dysfunction in Fabry disease. Molecular Genetics and Metabolism. PMID 27471012 DOI: 10.1016/J.Ymgme.2016.07.010 |
0.315 |
|
| 2015 |
Yang C, Wang H, Zhu D, Hong CS, Dmitriev P, Zhang C, Li Y, Ikejiri B, Brady RO, Zhuang Z. Mutant glucocerebrosidase in Gaucher disease recruits Hsp27 to the Hsp90 chaperone complex for proteasomal degradation. Proceedings of the National Academy of Sciences of the United States of America. 112: 1137-42. PMID 25583479 DOI: 10.1073/Pnas.1424288112 |
0.312 |
|
| 2015 |
Schiffmann R, Swift C, Wang X, Brady RO, Ries M. A prospective 10year study of individualized, intensified enzyme replacement therapy in advanced Fabry disease Molecular Genetics and Metabolism. 114: S103. DOI: 10.1016/J.Ymgme.2014.12.232 |
0.337 |
|
| 2014 |
El-Amouri SS, Dai M, Han JF, Brady RO, Pan D. Normalization and improvement of CNS deficits in mice with hurler syndrome after long-term peripheral delivery of BBB-targeted iduronidase. Molecular Therapy : the Journal of the American Society of Gene Therapy. 22: 2028-37. PMID 25088464 DOI: 10.1038/Mt.2014.152 |
0.308 |
|
| 2014 |
Mistry PK, Liu J, Sun L, Chuang WL, Yuen T, Yang R, Lu P, Zhang K, Li J, Keutzer J, Stachnik A, Mennone A, Boyer JL, Jain D, Brady RO, et al. Glucocerebrosidase 2 gene deletion rescues type 1 Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 111: 4934-9. PMID 24639522 DOI: 10.1073/Pnas.1400768111 |
0.347 |
|
| 2014 |
Dai M, Han J, El-Amouri SS, Brady RO, Pan D. Platelets are efficient and protective depots for storage, distribution, and delivery of lysosomal enzyme in mice with Hurler syndrome. Proceedings of the National Academy of Sciences of the United States of America. 111: 2680-5. PMID 24550296 DOI: 10.1073/Pnas.1323155111 |
0.304 |
|
| 2014 |
Barranger JA, Brady RO, Grabowski GA, Mankin H, Mistry PK, Weinreb NJ. Position statement: National Gaucher Foundation Medical Advisory Board, January 7, 2014. American Journal of Hematology. 89: 457-8. PMID 24488939 DOI: 10.1002/Ajh.23687 |
0.372 |
|
| 2014 |
Meng X, Shen J, Kong M, Brady R, Li RA, Schiffmann R. Abnormal intracellular calcium handling: a key pathogenic and therapeutic target of the cardiac manifestations in Fabry disease Molecular Genetics and Metabolism. 111: S77. DOI: 10.1016/J.Ymgme.2013.12.177 |
0.335 |
|
| 2013 |
Erba PA, Minichilli F, Giona F, Linari S, Dambrosia J, Pierini A, Filocamo M, Di Rocco M, Buffoni F, Brady RO, Mariani G. 99mTc-sestamibi scintigraphy to monitor the long-term efficacy of enzyme replacement therapy on bone marrow infiltration in patients with Gaucher disease. Journal of Nuclear Medicine : Official Publication, Society of Nuclear Medicine. 54: 1717-24. PMID 23990684 DOI: 10.2967/Jnumed.113.121871 |
0.367 |
|
| 2013 |
Wang D, El-Amouri SS, Dai M, Kuan CY, Hui DY, Brady RO, Pan D. Engineering a lysosomal enzyme with a derivative of receptor-binding domain of apoE enables delivery across the blood-brain barrier. Proceedings of the National Academy of Sciences of the United States of America. 110: 2999-3004. PMID 23382178 DOI: 10.1073/Pnas.1222742110 |
0.311 |
|
| 2013 |
Yang C, Rahimpour S, Lu J, Pacak K, Ikejiri B, Brady RO, Zhuang Z. Histone deacetylase inhibitors increase glucocerebrosidase activity in Gaucher disease by modulation of molecular chaperones. Proceedings of the National Academy of Sciences of the United States of America. 110: 966-71. PMID 23277556 DOI: 10.1073/Pnas.1221046110 |
0.306 |
|
| 2013 |
Brady RO, Yang C, Zhuang Z. An innovative approach to the treatment of Gaucher disease and possibly other metabolic disorders of the brain. Journal of Inherited Metabolic Disease. 36: 451-4. PMID 22814681 DOI: 10.1007/S10545-012-9515-9 |
0.356 |
|
| 2010 |
Lu J, Chiang J, Iyer RR, Thompson E, Kaneski CR, Xu DS, Yang C, Chen M, Hodes RJ, Lonser RR, Brady RO, Zhuang Z. Decreased glucocerebrosidase activity in Gaucher disease parallels quantitative enzyme loss due to abnormal interaction with TCP1 and c-Cbl. Proceedings of the National Academy of Sciences of the United States of America. 107: 21665-70. PMID 21098288 DOI: 10.1073/Pnas.1014376107 |
0.369 |
|
| 2010 |
Kaneski CR, Schiffmann R, Brady RO, Murray GJ. Use of lissamine rhodamine ceramide trihexoside as a functional assay for alpha-galactosidase A in intact cells. Journal of Lipid Research. 51: 2808-17. PMID 20526001 DOI: 10.1194/Jlr.D007294 |
0.336 |
|
| 2010 |
Meng XL, Shen JS, Kawagoe S, Ohashi T, Brady RO, Eto Y. Induced pluripotent stem cells derived from mouse models of lysosomal storage disorders. Proceedings of the National Academy of Sciences of the United States of America. 107: 7886-91. PMID 20385825 DOI: 10.1073/Pnas.1002758107 |
0.351 |
|
| 2010 |
Giannini EH, Mehta AB, Hilz MJ, Beck M, Bichet DG, Brady RO, West M, Germain DP, Wanner C, Waldek S, Clarke JT, Mengel E, Strotmann JM, Warnock DG, Linhart A. A validated disease severity scoring system for Fabry disease. Molecular Genetics and Metabolism. 99: 283-90. PMID 19951842 DOI: 10.1016/J.Ymgme.2009.10.178 |
0.305 |
|
| 2009 |
Enquist IB, Nilsson E, Bianco CL, Månsson JE, Ehinger M, Brady RO, Richter J, Karlsson S. Development of novel therapies in murine models for Gaucher disease Clinical Therapeutics. 31: S198-S199. DOI: 10.1016/S0149-2918(09)80022-2 |
0.307 |
|
| 2008 |
Di Rocco M, Giona F, Carubbi F, Linari S, Minichilli F, Brady RO, Mariani G, Cappellini MD. A new severity score index for phenotypic classification and evaluation of responses to treatment in type I Gaucher disease. Haematologica. 93: 1211-8. PMID 18603565 DOI: 10.3324/Haematol.12379 |
0.337 |
|
| 2008 |
Aerts JM, Groener JE, Kuiper S, Donker-Koopman WE, Strijland A, Ottenhoff R, van Roomen C, Mirzaian M, Wijburg FA, Linthorst GE, Vedder AC, Rombach SM, Cox-Brinkman J, Somerharju P, Boot RG, ... ... Brady RO, et al. Elevated globotriaosylsphingosine is a hallmark of Fabry disease. Proceedings of the National Academy of Sciences of the United States of America. 105: 2812-7. PMID 18287059 DOI: 10.1073/Pnas.0712309105 |
0.382 |
|
| 2008 |
Moore DF, Brady RO. Systems-biology approach to sphingolipid-storage disorders Future Lipidology. 3: 105-111. DOI: 10.2217/17460875.3.1.105 |
0.368 |
|
| 2007 |
Enquist IB, Lo Bianco C, Ooka A, Nilsson E, MÃ¥nsson JE, Ehinger M, Richter J, Brady RO, Kirik D, Karlsson S. Murine models of acute neuronopathic Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 104: 17483-8. PMID 17954912 DOI: 10.1073/Pnas.0708086104 |
0.371 |
|
| 2007 |
Shen JS, Meng XL, Schiffmann R, Brady RO, Kaneski CR. Establishment and characterization of Fabry disease endothelial cells with an extended lifespan. Molecular Genetics and Metabolism. 92: 137-44. PMID 17644384 DOI: 10.1016/J.Ymgme.2007.06.003 |
0.311 |
|
| 2007 |
Shin SH, Murray GJ, Kluepfel-Stahl S, Cooney AM, Quirk JM, Schiffmann R, Brady RO, Kaneski CR. Screening for pharmacological chaperones in Fabry disease. Biochemical and Biophysical Research Communications. 359: 168-73. PMID 17532296 DOI: 10.1016/J.Bbrc.2007.05.082 |
0.318 |
|
| 2007 |
Moore DF, Gelderman MP, Ferreira PA, Fuhrmann SR, Yi H, Elkahloun A, Lix LM, Brady RO, Schiffmann R, Goldin E. Genomic abnormalities of the murine model of Fabry disease after disease-related perturbation, a systems biology approach. Proceedings of the National Academy of Sciences of the United States of America. 104: 8065-70. PMID 17470787 DOI: 10.1073/Pnas.0701991104 |
0.344 |
|
| 2007 |
Schiffmann R, Askari H, Timmons M, Robinson C, Benko W, Brady RO, Ries M. Weekly enzyme replacement therapy may slow decline of renal function in patients with Fabry disease who are on long-term biweekly dosing. Journal of the American Society of Nephrology : Jasn. 18: 1576-83. PMID 17409308 DOI: 10.1681/Asn.2006111263 |
0.302 |
|
| 2007 |
Moore DF, Krokhin OV, Beavis RC, Ries M, Robinson C, Goldin E, Brady RO, Wilkins JA, Schiffmann R. Proteomics of specific treatment-related alterations in Fabry disease: a strategy to identify biological abnormalities. Proceedings of the National Academy of Sciences of the United States of America. 104: 2873-8. PMID 17301227 DOI: 10.1073/Pnas.0611315104 |
0.34 |
|
| 2007 |
Ries M, Kim HJ, Zalewski CK, Mastroianni MA, Moore DF, Brady RO, Dambrosia JM, Schiffmann R, Brewer CC. Neuropathic and cerebrovascular correlates of hearing loss in Fabry disease. Brain : a Journal of Neurology. 130: 143-50. PMID 17105746 DOI: 10.1093/Brain/Awl310 |
0.313 |
|
| 2007 |
Lonser RR, Schiffman R, Robison RA, Butman JA, Quezado Z, Walker ML, Morrison PF, Walbridge S, Murray GJ, Park DM, Brady RO, Oldfield EH. Image-guided, direct convective delivery of glucocerebrosidase for neuronopathic Gaucher disease. Neurology. 68: 254-61. PMID 17065591 DOI: 10.1212/01.Wnl.0000247744.10990.E6 |
0.372 |
|
| 2007 |
Yoshimitsu M, Higuchi K, Ramsubir S, Nonaka T, Rasaiah VI, Siatskas C, Liang SB, Murray GJ, Brady RO, Medin JA. Efficient correction of Fabry mice and patient cells mediated by lentiviral transduction of hematopoietic stem/progenitor cells. Gene Therapy. 14: 256-65. PMID 16929352 DOI: 10.1038/Sj.Gt.3302839 |
0.31 |
|
| 2006 |
Enquist IB, Nilsson E, Ooka A, MÃ¥nsson JE, Olsson K, Ehinger M, Brady RO, Richter J, Karlsson S. Effective cell and gene therapy in a murine model of Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 103: 13819-24. PMID 16954197 DOI: 10.1073/Pnas.0606016103 |
0.346 |
|
| 2006 |
Ries M, Clarke JT, Whybra C, Timmons M, Robinson C, Schlaggar BL, Pastores G, Lien YH, Kampmann C, Brady RO, Beck M, Schiffmann R. Enzyme-replacement therapy with agalsidase alfa in children with Fabry disease. Pediatrics. 118: 924-32. PMID 16950982 DOI: 10.1542/Peds.2005-2895 |
0.369 |
|
| 2006 |
Moore DF, Gelderman MP, Fuhrmann SR, Schiffmann R, Brady RO, Goldin E. Fabry disease and vascular risk factors: future strategies for patient-based studies and the knockout murine model. Acta Paediatrica (Oslo, Norway : 1992). Supplement. 95: 69-71. PMID 16720469 DOI: 10.1080/08035320600618932 |
0.354 |
|
| 2006 |
Brady RO. Emerging strategies for the treatment of hereditary metabolic storage disorders. Rejuvenation Research. 9: 237-44. PMID 16706651 DOI: 10.1089/Rej.2006.9.237 |
0.394 |
|
| 2006 |
Schiffmann R, Hauer P, Freeman B, Ries M, Scott LJ, Polydefkis M, Brady RO, McArthur JC, Wagner K. Enzyme replacement therapy and intraepidermal innervation density in Fabry disease. Muscle & Nerve. 34: 53-6. PMID 16583374 DOI: 10.1002/Mus.20550 |
0.326 |
|
| 2006 |
Ries M, Moore DF, Robinson CJ, Tifft CJ, Rosenbaum KN, Brady RO, Schiffmann R, Krasnewich D. Quantitative dysmorphology assessment in Fabry disease. Genetics in Medicine : Official Journal of the American College of Medical Genetics. 8: 96-101. PMID 16481892 DOI: 10.1097/01.Gim.0000200950.25118.Dd |
0.345 |
|
| 2006 |
Brady RO. Enzyme replacement for lysosomal diseases. Annual Review of Medicine. 57: 283-96. PMID 16409150 DOI: 10.1146/Annurev.Med.57.110104.115650 |
0.355 |
|
| 2006 |
Schiffmann R, Ries M, Timmons M, Flaherty JT, Brady RO. Long-term therapy with agalsidase alfa for Fabry disease: safety and effects on renal function in a home infusion setting. Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association. 21: 345-54. PMID 16204287 DOI: 10.1093/Ndt/Gfi152 |
0.349 |
|
| 2005 |
Lee KO, Luu N, Kaneski CR, Schiffmann R, Brady RO, Murray GJ. Improved intracellular delivery of glucocerebrosidase mediated by the HIV-1 TAT protein transduction domain. Biochemical and Biophysical Research Communications. 337: 701-7. PMID 16223608 DOI: 10.1016/J.Bbrc.2005.05.207 |
0.32 |
|
| 2005 |
de Grey AD, Alvarez PJ, Brady RO, Cuervo AM, Jerome WG, McCarty PL, Nixon RA, Rittmann BE, Sparrow JR. Medical bioremediation: prospects for the application of microbial catabolic diversity to aging and several major age-related diseases. Ageing Research Reviews. 4: 315-38. PMID 16040282 DOI: 10.1016/J.Arr.2005.03.008 |
0.31 |
|
| 2005 |
Lonser RR, Walbridge S, Murray GJ, Aizenberg MR, Vortmeyer AO, Aerts JM, Brady RO, Oldfield EH. Convection perfusion of glucocerebrosidase for neuronopathic Gaucher's disease. Annals of Neurology. 57: 542-8. PMID 15786474 DOI: 10.1002/Ana.20444 |
0.361 |
|
| 2005 |
Ries M, Gupta S, Moore DF, Sachdev V, Quirk JM, Murray GJ, Rosing DR, Robinson C, Schaefer E, Gal A, Dambrosia JM, Garman SC, Brady RO, Schiffmann R. Pediatric Fabry disease. Pediatrics. 115: e344-55. PMID 15713906 DOI: 10.1542/Peds.2004-1678 |
0.357 |
|
| 2005 |
Yoshimitsu M, Siatskas C, Liang S, Rasaiah VI, Higuchi K, Murray GJ, Takenaka T, Tei C, Brady RO, Medin JA. Long-Term and Sustained Correction of the α-Galactosidase A Deficiency in Fabry Mice and Patient Cells Receiving Lentivirally Transduced Hematopoietic Stem/Progenitor Cells. Blood. 106: 1285-1285. DOI: 10.1182/Blood.V106.11.1285.1285 |
0.332 |
|
| 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 |
0.354 |
|
| 2004 |
Brady RO, Schiffmann R. Enzyme-replacement therapy for metabolic storage disorders. The Lancet. Neurology. 3: 752-6. PMID 15556808 DOI: 10.1016/S1474-4422(04)00938-X |
0.378 |
|
| 2004 |
Yoshimitsu M, Sato T, Tao K, Walia JS, Rasaiah VI, Sleep GT, Murray GJ, Poeppl AG, Underwood J, West L, Brady RO, Medin JA. Bioluminescent imaging of a marking transgene and correction of Fabry mice by neonatal injection of recombinant lentiviral vectors. Proceedings of the National Academy of Sciences of the United States of America. 101: 16909-14. PMID 15550536 DOI: 10.1073/Pnas.0407572101 |
0.315 |
|
| 2004 |
Moore DF, Ye F, Brennan ML, Gupta S, Barshop BA, Steiner RD, Rhead WJ, Brady RO, Hazen SL, Schiffmann R. Ascorbate decreases Fabry cerebral hyperperfusion suggesting a reactive oxygen species abnormality: an arterial spin tagging study. Journal of Magnetic Resonance Imaging : Jmri. 20: 674-83. PMID 15390234 DOI: 10.1002/Jmri.20162 |
0.335 |
|
| 2004 |
Ries M, Bettis KE, Choyke P, Kopp JB, Austin HA, Brady RO, Schiffmann R. Parapelvic kidney cysts: a distinguishing feature with high prevalence in Fabry disease. Kidney International. 66: 978-82. PMID 15327390 DOI: 10.1111/J.1523-1755.2004.00846.X |
0.345 |
|
| 2004 |
Desnick RJ, Brady RO. Fabry disease in childhood. The Journal of Pediatrics. 144: S20-6. PMID 15126980 DOI: 10.1016/J.Jpeds.2004.01.051 |
0.375 |
|
| 2004 |
Kim EY, Hong YB, Lai Z, Kim HJ, Cho YH, Brady RO, Jung SC. Expression and secretion of human glucocerebrosidase mediated by recombinant lentivirus vectors in vitro and in vivo: implications for gene therapy of Gaucher disease. Biochemical and Biophysical Research Communications. 318: 381-90. PMID 15120612 DOI: 10.1016/J.Bbrc.2004.04.040 |
0.329 |
|
| 2004 |
Kim EY, Hong YB, Lai Z, Brady RO, Jung S. 863. Expression and Secretion of Human Glucocerebrosidase Mediated by Recombinant Lentivirus Vectors In Vitro and In Vivo Molecular Therapy. 9. DOI: 10.1016/J.Ymthe.2004.06.770 |
0.332 |
|
| 2003 |
Brady RO. Gaucher and Fabry diseases: from understanding pathophysiology to rational therapies. Acta Paediatrica (Oslo, Norway : 1992). Supplement. 92: 19-24. PMID 14989461 DOI: 10.1111/J.1651-2227.2003.Tb00215.X |
0.356 |
|
| 2003 |
Matsuda J, Suzuki O, Oshima A, Yamamoto Y, Noguchi A, Takimoto K, Itoh M, Matsuzaki Y, Yasuda Y, Ogawa S, Sakata Y, Nanba E, Higaki K, Ogawa Y, Tominaga L, ... ... Brady RO, et al. Chemical chaperone therapy for brain pathology in G(M1)-gangliosidosis. Proceedings of the National Academy of Sciences of the United States of America. 100: 15912-7. PMID 14676316 DOI: 10.1073/Pnas.2536657100 |
0.359 |
|
| 2003 |
Schiffmann R, Floeter MK, Dambrosia JM, Gupta S, Moore DF, Sharabi Y, Khurana RK, Brady RO. Enzyme replacement therapy improves peripheral nerve and sweat function in Fabry disease. Muscle & Nerve. 28: 703-10. PMID 14639584 DOI: 10.1002/Mus.10497 |
0.346 |
|
| 2003 |
Brady RO. Enzyme replacement therapy: conception, chaos and culmination. Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 358: 915-9. PMID 12803925 DOI: 10.1098/Rstb.2003.1269 |
0.391 |
|
| 2003 |
Park J, Murray GJ, Limaye A, Quirk JM, Gelderman MP, Brady RO, Qasba P. Long-term correction of globotriaosylceramide storage in Fabry mice by recombinant adeno-associated virus-mediated gene transfer. Proceedings of the National Academy of Sciences of the United States of America. 100: 3450-4. PMID 12624185 DOI: 10.1073/Pnas.0537900100 |
0.308 |
|
| 2003 |
Desnick RJ, Brady R, Barranger J, Collins AJ, Germain DP, Goldman M, Grabowski G, Packman S, Wilcox WR. Fabry disease, an under-recognized multisystemic disorder: Expert recommendations for diagnosis, management, and enzyme replacement therapy Annals of Internal Medicine. 138: 338-346. PMID 12585833 DOI: 10.7326/0003-4819-138-4-200302180-00014 |
0.365 |
|
| 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 |
0.373 |
|
| 2002 |
Schiffmann R, Brady RO. New prospects for the treatment of lysosomal storage diseases. Drugs. 62: 733-42. PMID 11929328 DOI: 10.2165/00003495-200262050-00002 |
0.378 |
|
| 2002 |
Moore DF, Altarescu G, Ling GS, Jeffries N, Frei KP, Weibel T, Charria-Ortiz G, Ferri R, Arai AE, Brady RO, Schiffmann R. Elevated cerebral blood flow velocities in Fabry disease with reversal after enzyme replacement. Stroke; a Journal of Cerebral Circulation. 33: 525-31. PMID 11823664 DOI: 10.1161/Hs0202.102601 |
0.335 |
|
| 2001 |
Brady RO, Murray GJ, Moore DF, Schiffmann R. Enzyme replacement therapy in Fabry disease. Journal of Inherited Metabolic Disease. 24: 18-24; discussion 11. PMID 11758675 DOI: 10.1023/A:1012451320105 |
0.362 |
|
| 2001 |
Germain DP, Kaneski CR, Brady RO. Mutation analysis of the acid beta-glucosidase gene in a patient with type 3 Gaucher disease and neutralizing antibody to alglucerase. Mutation Research. 483: 89-94. PMID 11600137 DOI: 10.1016/S0027-5107(01)00232-9 |
0.372 |
|
| 2001 |
Brady RO. Tay-Sachs disease: the search for the enzymatic defect. Advances in Genetics. 44: 51-60. PMID 11596998 DOI: 10.1016/S0065-2660(01)44070-3 |
0.35 |
|
| 2001 |
Moore DF, Scott LT, Gladwin MT, Altarescu G, Kaneski C, Suzuki K, Pease-Fye M, Ferri R, Brady RO, Herscovitch P, Schiffmann R. Regional cerebral hyperperfusion and nitric oxide pathway dysregulation in Fabry disease: reversal by enzyme replacement therapy. Circulation. 104: 1506-12. PMID 11571244 DOI: 10.1161/Hc3801.096352 |
0.321 |
|
| 2001 |
Schiffmann R, Kopp JB, Austin HA, Sabnis S, Moore DF, Weibel T, Balow JE, Brady RO. Enzyme replacement therapy in Fabry disease: a randomized controlled trial. Jama. 285: 2743-9. PMID 11386930 DOI: 10.1001/Jama.285.21.2743 |
0.307 |
|
| 2001 |
Altarescu G, Hill S, Wiggs E, Jeffries N, Kreps C, Parker CC, Brady RO, Barton NW, Schiffmann R. The efficacy of enzyme replacement therapy in patients with chronic neuronopathic Gaucher's disease. The Journal of Pediatrics. 138: 539-47. PMID 11295718 DOI: 10.1067/Mpd.2001.112171 |
0.789 |
|
| 2001 |
Qin G, Takenaka T, Telsch K, Kelley L, Howard T, Levade T, Deans R, Howard BH, Malech HL, Brady RO, Medin JA. Preselective gene therapy for Fabry disease. Proceedings of the National Academy of Sciences of the United States of America. 98: 3428-33. PMID 11248095 DOI: 10.1073/Pnas.061020598 |
0.351 |
|
| 2001 |
Jung SC, Han IP, Limaye A, Xu R, Gelderman MP, Zerfas P, Tirumalai K, Murray GJ, During MJ, Brady RO, Qasba P. Adeno-associated viral vector-mediated gene transfer results in long-term enzymatic and functional correction in multiple organs of Fabry mice. Proceedings of the National Academy of Sciences of the United States of America. 98: 2676-81. PMID 11226298 DOI: 10.1073/Pnas.051634498 |
0.346 |
|
| 2000 |
Brady RO, Schiffmann R. Clinical features of and recent advances in therapy for Fabry disease. Jama. 284: 2771-5. PMID 11105184 DOI: 10.1001/Jama.284.21.2771 |
0.369 |
|
| 2000 |
Altarescu G, Schiffmann R, Parker CC, Moore DF, Kreps C, Brady RO, Barton NW. Comparative efficacy of dose regimens in enzyme replacement therapy of type I Gaucher disease. Blood Cells, Molecules & Diseases. 26: 285-90. PMID 11042029 DOI: 10.1006/Bcmd.2000.0310 |
0.765 |
|
| 2000 |
Takenaka T, Murray GJ, Qin G, Quirk JM, Ohshima T, Qasba P, Clark K, Kulkarni AB, Brady RO, Medin JA. Long-term enzyme correction and lipid reduction in multiple organs of primary and secondary transplanted Fabry mice receiving transduced bone marrow cells. Proceedings of the National Academy of Sciences of the United States of America. 97: 7515-20. PMID 10840053 DOI: 10.1073/Pnas.120177997 |
0.353 |
|
| 2000 |
Brady RO. Neurogene therapy for the 21st century. Archives of Neurology. 57: 54. PMID 10634437 DOI: 10.1001/Archneur.57.1.54 |
0.423 |
|
| 2000 |
Schiffmann R, Murray GJ, Treco D, Daniel P, Sellos-Moura M, Myers M, Quirk JM, Zirzow GC, Borowski M, Loveday K, Anderson T, Gillespie F, Oliver KL, Jeffries NO, Doo E, ... ... Brady RO, et al. Infusion of alpha-galactosidase A reduces tissue globotriaosylceramide storage in patients with Fabry disease. Proceedings of the National Academy of Sciences of the United States of America. 97: 365-70. PMID 10618424 DOI: 10.1073/Pnas.97.1.365 |
0.351 |
|
| 1999 |
Goldin E, Cooney A, Kaneski CR, Brady RO, Schiffmann R. Mucolipidosis IV consists of one complementation group. Proceedings of the National Academy of Sciences of the United States of America. 96: 8562-6. PMID 10411915 DOI: 10.1073/Pnas.96.15.8562 |
0.325 |
|
| 1999 |
Takenaka T, Hendrickson CS, Tworek DM, Tudor M, Schiffmann R, Brady RO, Medin JA. Enzymatic and functional correction along with long-term enzyme secretion from transduced bone marrow hematopoietic stem/progenitor and stromal cells derived from patients with Fabry disease. Experimental Hematology. 27: 1149-59. PMID 10390190 DOI: 10.1016/S0301-472X(99)00050-8 |
0.338 |
|
| 1999 |
Ohshima T, Schiffmann R, Murray GJ, Kopp J, Quirk JM, Stahl S, Chan CC, Zerfas P, Tao-Cheng JH, Ward JM, Brady RO, Kulkarni AB. Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice. Proceedings of the National Academy of Sciences of the United States of America. 96: 6423-7. PMID 10339603 DOI: 10.1073/Pnas.96.11.6423 |
0.333 |
|
| 1999 |
Zirzow GC, Sanchez OA, Murray GJ, Brady RO, Oldfield EH. Delivery, distribution, and neuronal uptake of exogenous mannose-terminal glucocerebrosidase in the intact rat brain. Neurochemical Research. 24: 301-5. PMID 9972879 DOI: 10.1023/A:1022578424693 |
0.387 |
|
| 1998 |
YOKOYAMA M, TRAMS EG, BRADY RO. Sphingolipid antibodies in sera of animals and patients with central nervous system lesions. Proceedings of the Society For Experimental Biology and Medicine. Society For Experimental Biology and Medicine (New York, N.Y.). 111: 350-2. PMID 14002620 DOI: 10.3181/00379727-111-27789 |
0.313 |
|
| 1998 |
TRAMS EG, BRADY RO. Cerebroside synthesis in Gaucher's disease. The Journal of Clinical Investigation. 39: 1546-50. PMID 13777865 DOI: 10.1172/Jci104175 |
0.309 |
|
| 1998 |
Argoff CE, Barton NW, Brady RO, Ziessman HA. Gastrointestinal symptoms and delayed gastric emptying in Fabry's disease: response to metoclopramide. Nuclear Medicine Communications. 19: 887-91. PMID 10581595 DOI: 10.1097/00006231-199809000-00009 |
0.75 |
|
| 1998 |
Dunbar CE, Kohn DB, Schiffmann R, Barton NW, Nolta JA, Esplin JA, Pensiero M, Long Z, Lockey C, Emmons RV, Csik S, Leitman S, Krebs CB, Carter C, Brady RO, et al. Retroviral transfer of the glucocerebrosidase gene into CD34+ cells from patients with Gaucher disease: in vivo detection of transduced cells without myeloablation. Human Gene Therapy. 9: 2629-40. PMID 9853529 DOI: 10.1089/Hum.1998.9.17-2629 |
0.759 |
|
| 1998 |
Brady RO. Therapy for the sphingolipidoses. Archives of Neurology. 55: 1055-6. PMID 9708954 DOI: 10.1001/Archneur.55.8.1055 |
0.367 |
|
| 1997 |
Brady RO. Gaucher's disease: past, present and future. Bailliã¨Re's Clinical Haematology. 10: 621-34. PMID 9497855 DOI: 10.1016/S0950-3536(97)80031-5 |
0.388 |
|
| 1997 |
Brady RO, Murray GJ, Oliver KL, Leitman SF, Sneller MC, Fleisher TA, Barton NW. Management of neutralizing antibody to Ceredase in a patient with type 3 Gaucher disease. Pediatrics. 100: E11. PMID 9382912 DOI: 10.1542/Peds.100.6.E11 |
0.78 |
|
| 1997 |
Schiffmann R, Heyes MP, Aerts JM, Dambrosia JM, Patterson MC, DeGraba T, Parker CC, Zirzow GC, Oliver K, Tedeschi G, Brady RO, Barton NW. Prospective study of neurological responses to treatment with macrophage-targeted glucocerebrosidase in patients with type 3 Gaucher's disease. Annals of Neurology. 42: 613-21. PMID 9382473 DOI: 10.1002/Ana.410420412 |
0.785 |
|
| 1997 |
Ohshima T, Murray GJ, Swaim WD, Longenecker G, Quirk JM, Cardarelli CO, Sugimoto Y, Pastan I, Gottesman MM, Brady RO, Kulkarni AB. alpha-Galactosidase A deficient mice: a model of Fabry disease. Proceedings of the National Academy of Sciences of the United States of America. 94: 2540-4. PMID 9122231 DOI: 10.1073/Pnas.94.6.2540 |
0.31 |
|
| 1996 |
BRADY RO, KANFER JN, SHAPIRO D. METABOLISM OF GLUCOCEREBROSIDES. II. EVIDENCE OF AN ENZYMATIC DEFICIENCY IN GAUCHER'S DISEASE. Biochemical and Biophysical Research Communications. 18: 221-5. PMID 14282020 DOI: 10.1016/0006-291X(65)90743-6 |
0.303 |
|
| 1996 |
SOMMERS JE, KANFER JN, BRADY RO. SPHINGOLIPID ANTIBODIES. II. SPECIFICITY OF ANTIBODY IN PATIENTS WITH DEMYELINATING DISEASES. Proceedings of the Society For Experimental Biology and Medicine. Society For Experimental Biology and Medicine (New York, N.Y.). 114: 350-2. PMID 14101186 DOI: 10.3181/00379727-114-28673 |
0.315 |
|
| 1996 |
Hill SC, Damaska BM, Tsokos M, Kreps C, Brady RO, Barton NW. Radiographic findings in type 3b Gaucher disease. Pediatric Radiology. 26: 852-60. PMID 8929296 DOI: 10.1007/Bf03178036 |
0.758 |
|
| 1996 |
Dunbar PIC, Kohn D, Karlsson SCS, Barton IN, Brady R, Cottler-Fox M, Emmons R, Esplin J, Leitman S, Lenarsky C, Nolta J, Parkman R, Pensiero M, Schifmann R, Tolstoshev P, et al. Retroviral Mediated Transfer of the cDNA for Human Glucocerebrosidase into Hematopoietic Stem Cells of Patients with Gaucher Disease. A Phase I Study. National Institutes of Health, Bethesda, Maryland Human Gene Therapy. 7: 231-253. PMID 8788174 DOI: 10.1089/Hum.1996.7.2-231 |
0.354 |
|
| 1996 |
Medin JA, Tudor M, Simovitch R, Quirk JM, Jacobson S, Murray GJ, Brady RO. Correction in trans for Fabry disease: expression, secretion and uptake of alpha-galactosidase A in patient-derived cells driven by a high-titer recombinant retroviral vector. Proceedings of the National Academy of Sciences of the United States of America. 93: 7917-22. PMID 8755577 DOI: 10.1073/Pnas.93.15.7917 |
0.363 |
|
| 1996 |
Brady RO, Barton NW. Enzyme replacement and gene therapy for Gaucher's disease. Lipids. 31: S137-9. PMID 8729108 DOI: 10.1007/Bf02637065 |
0.779 |
|
| 1996 |
Miller SP, Zirzow GC, Doppelt SH, Brady RO, Barton NW. Analysis of the lipids of normal and Gaucher bone marrow. The Journal of Laboratory and Clinical Medicine. 127: 353-8. PMID 8656038 DOI: 10.1016/S0022-2143(96)90183-3 |
0.746 |
|
| 1996 |
Medin JA, Migita M, Pawliuk R, Jacobson S, Amiri M, Kluepfel-Stahl S, Brady RO, Humphries RK, Karlsson S. A bicistronic therapeutic retroviral vector enables sorting of transduced CD34+ cells and corrects the enzyme deficiency in cells from Gaucher patients. Blood. 87: 1754-62. PMID 8634421 DOI: 10.1182/Blood.V87.5.1754.Bloodjournal8751754 |
0.321 |
|
| 1995 |
Ohshima T, Murray GJ, Nagle JW, Quirk JM, Kraus MH, Barton NW, Brady RO, Kulkarni AB. Structural organization and expression of the mouse gene encoding alpha-galactosidase A. Gene. 166: 277-80. PMID 8543175 DOI: 10.1016/0378-1119(95)00592-7 |
0.734 |
|
| 1995 |
Grabowski GA, Barton NW, Pastores G, Dambrosia JM, Banerjee TK, McKee MA, Parker C, Schiffmann R, Hill SC, Brady RO. Enzyme therapy in type 1 Gaucher disease: comparative efficacy of mannose-terminated glucocerebrosidase from natural and recombinant sources. Annals of Internal Medicine. 122: 33-9. PMID 7985893 DOI: 10.7326/0003-4819-122-1-199501010-00005 |
0.768 |
|
| 1995 |
Murray GJ, Oliver KL, Jin FS, Brady RO. Studies on the turnover of exogenous mannose-terminal glucocerebrosidase in rat liver lysosomes. Journal of Cellular Biochemistry. 57: 208-17. PMID 7759558 DOI: 10.1002/Jcb.240570205 |
0.338 |
|
| 1995 |
Zimran A, Elstein D, Schiffmann R, Abrahamov A, Goldberg M, Bar-Maor JA, Brady RO, Guzzetta PC, Barton NW. Outcome of partial splenectomy for type I Gaucher disease. The Journal of Pediatrics. 126: 596-7. PMID 7699540 DOI: 10.1016/S0022-3476(95)70358-6 |
0.777 |
|
| 1995 |
Tedeschi G, Schiffmann R, Barton NW, Shih HH, Gospe SM, Brady RO, Alger JR, Di Chiro G. Proton magnetic resonance spectroscopic imaging in childhood ataxia with diffuse central nervous system hypomyelination. Neurology. 45: 1526-32. PMID 7644053 DOI: 10.1212/Wnl.45.8.1526 |
0.746 |
|
| 1994 |
Brady RO, Murray GJ, Barton NW. Modifying exogenous glucocerebrosidase for effective replacement therapy in Gaucher disease. Journal of Inherited Metabolic Disease. 17: 510-9. PMID 7967500 DOI: 10.1007/Bf00711365 |
0.389 |
|
| 1994 |
Higgins JJ, Kaneski CR, Bernardini I, Brady RO, Barton NW. Pyridoxine-responsive hyper-beta-alaninemia associated with Cohen's syndrome. Neurology. 44: 1728-32. PMID 7936305 DOI: 10.1212/Wnl.44.9.1728 |
0.76 |
|
| 1994 |
Goldin E, Imai Y, Kaneski CR, Pentchev PG, Brady RO, Hascall VC. Mucolipidosis IV fibroblasts synthesize normal amounts of hyaluronic acid. Journal of Inherited Metabolic Disease. 17: 545-53. PMID 7837760 DOI: 10.1007/Bf00711589 |
0.315 |
|
| 1993 |
Smanik EJ, Tavill AS, Jacobs GH, Schafer IA, Farquhar L, Weber FL, Mayes JT, Schulak JA, Petrelli M, Zirzow GC, Oliver KL, Miller SPF, Brady RO. Orthotopic liver transplantation in two adults with Niemann-Pick and Gaucher's diseases: Implications for the treatment of inherited metabolic disease Hepatology. 17: 42-49. PMID 8423040 DOI: 10.1002/Hep.1840170109 |
0.351 |
|
| 1993 |
Hill SC, Parker CC, Brady RO, Barton NW. MRI of multiple platyspondyly in Gaucher disease: response to enzyme replacement therapy. Journal of Computer Assisted Tomography. 17: 806-9. PMID 8370839 DOI: 10.1097/00004728-199309000-00026 |
0.764 |
|
| 1993 |
Banerjee TK, Grubb W, Otero C, McKee M, Brady RO, Barton NW. Musculocutaneous mononeuropathy complicating Capnocytophaga canimorsus infection. Neurology. 43: 2411-2. PMID 8232970 DOI: 10.1212/Wnl.43.11.2411 |
0.711 |
|
| 1993 |
Brady RO, Barton NW, Grabowski GA. The role of neurogenetics in Gaucher disease. Archives of Neurology. 50: 1212-24. PMID 8215980 DOI: 10.1001/Archneur.1993.00540110088009 |
0.784 |
|
| 1992 |
Barton NW, Brady RO, Dambrosia JM, Doppelt SH, Hill SC, Holder CA, Mankin HJ, Murray GJ, Zirzow GC, Parker RI. Dose-dependent responses to macrophage-targeted glucocerebrosidase in a child with Gaucher disease. The Journal of Pediatrics. 120: 277-80. PMID 1735829 DOI: 10.1016/S0022-3476(05)80444-5 |
0.739 |
|
| 1992 |
Higgins JJ, Patterson MC, Papadopoulos NM, Brady RO, Pentchev PG, Barton NW. Hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP syndrome). Neurology. 42: 194-8. PMID 1734303 DOI: 10.1212/Wnl.42.1.194 |
0.766 |
|
| 1992 |
Johnson LA, Hoppel BE, Gerard EL, Miller SP, Doppelt SH, Zirzow GC, Rosenthal DI, Dambrosia JM, Hill SC, Brady RO. Quantitative chemical shift imaging of vertebral bone marrow in patients with Gaucher disease. Radiology. 182: 451-5. PMID 1732964 DOI: 10.1148/Radiology.182.2.1732964 |
0.309 |
|
| 1992 |
Hill SC, Damaska BM, Ling A, Patterson K, Di Bisceglie AM, Brady RO, Barton NW. Gaucher disease: abdominal MR imaging findings in 46 patients. Radiology. 184: 561-6. PMID 1620865 DOI: 10.1148/Radiology.184.2.1620865 |
0.757 |
|
| 1992 |
Filling-Katz MR, Miller SPF, Merrick HF, Travis WD, Gregg RE, Tsokos M, Comly M, Kaneski CR, Mackie S, Lebovics RS, Blanchette-Mackie EJ, Brady RO, Pentchev PG. Clinical, pathologic, and biochemical features of a cholesterol lipidosis accompanied by hyperlipidemia and xanthomas Neurology. 42: 1768-1774. PMID 1513468 DOI: 10.1212/Wnl.42.9.1768 |
0.33 |
|
| 1992 |
Higgins JJ, Patterson MC, Dambrosia JM, Pikus AT, Pentchev PG, Sato S, Brady RO, Barton NW. A clinical staging classification for type C Niemann-Pick disease. Neurology. 42: 2286-90. PMID 1461380 DOI: 10.1212/Wnl.42.12.2286 |
0.761 |
|
| 1992 |
Rosenthal DI, Barton NW, McKusick KA, Rosen BR, Hill SC, Castronovo FP, Brady RO, Doppelt SH, Mankin HJ. Quantitative imaging of Gaucher disease. Radiology. 185: 841-5. PMID 1438773 DOI: 10.1148/Radiology.185.3.1438773 |
0.754 |
|
| 1992 |
Goldin E, Roff CF, Miller SP, Rodriguez-Lafrasse C, Vanier MT, Brady RO, Pentchev PG. Type C Niemann-Pick disease: a murine model of the lysosomal cholesterol lipidosis accumulates sphingosine and sphinganine in liver. Biochimica Et Biophysica Acta. 1127: 303-11. PMID 1324734 DOI: 10.1016/0005-2760(92)90236-O |
0.324 |
|
| 1991 |
Barton NW, Brady RO, Dambrosia JM, Di Bisceglie AM, Doppelt SH, Hill SC, Mankin HJ, Murray GJ, Parker RI, Argoff CE. Replacement therapy for inherited enzyme deficiency--macrophage-targeted glucocerebrosidase for Gaucher's disease. The New England Journal of Medicine. 324: 1464-70. PMID 2023606 DOI: 10.1056/Nejm199105233242104 |
0.783 |
|
| 1991 |
Murray GJ, Howard KD, Richards SM, Barton NW, Brady RO. Gaucher's disease: lack of antibody response in 12 patients following repeated intravenous infusions of mannose terminal glucocerebrosidase. Journal of Immunological Methods. 137: 113-20. PMID 2010615 DOI: 10.1016/0022-1759(91)90400-A |
0.77 |
|
| 1991 |
Parker RI, Barton NW, Read EJ, Brady RO. Hematologic improvement in a patient with Gaucher disease on long-term enzyme replacement therapy: evidence for decreased splenic sequestration and improved red blood cell survival. American Journal of Hematology. 38: 130-7. PMID 1951303 DOI: 10.1002/Ajh.2830380211 |
0.775 |
|
| 1991 |
Grewal RP, Doppelt SH, Thompson MA, Katz D, Brady RO, Barton NW. Neurologic complications of nonneuronopathic Gaucher's disease. Archives of Neurology. 48: 1271-2. PMID 1845033 DOI: 10.1001/Archneur.1991.00530240075025 |
0.771 |
|
| 1990 |
Argoff CE, Kaneski CR, Blanchette-Mackie EJ, Comly M, Dwyer NK, Brown A, Brady RO, Pentchev PG. Type C Niemann-Pick disease: documentation of abnormal LDL processing in lymphocytes. Biochemical and Biophysical Research Communications. 171: 38-45. PMID 2393397 DOI: 10.1016/0006-291X(90)91353-T |
0.328 |
|
| 1990 |
Fink JK, Correll PH, Perry LK, Brady RO, Karlsson S. Correction of glucocerebrosidase deficiency after retroviral-mediated gene transfer into hematopoietic progenitor cells from patients with Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 87: 2334-8. PMID 2315324 DOI: 10.1073/Pnas.87.6.2334 |
0.315 |
|
| 1990 |
Barton NW, Furbish FS, Murray GJ, Garfield M, Brady RO. Therapeutic response to intravenous infusions of glucocerebrosidase in a patient with Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 87: 1913-6. PMID 2308952 DOI: 10.1073/Pnas.87.5.1913 |
0.782 |
|
| 1989 |
Brady RO, Gal AE, Pentchev PG. Evolution of enzyme replacement therapy for lipid storage diseases. Life Sciences. 15: 1235-48. PMID 4220261 DOI: 10.1016/0024-3205(74)90305-1 |
0.345 |
|
| 1989 |
Fink JK, Filling-Katz MR, Sokol J, Cogan DG, Pikus A, Sonies B, Soong B, Pentchev PG, Comly ME, Brady RO. Clinical spectrum of Niemann-Pick disease type C. Neurology. 39: 1040-9. PMID 2761697 DOI: 10.1212/Wnl.39.8.1040 |
0.338 |
|
| 1989 |
Brady RO, Filling-Katz MR, Barton NW, Pentchev PG. Niemann-Pick disease types C and D. Neurologic Clinics. 7: 75-88. PMID 2646522 DOI: 10.1016/S0733-8619(18)30829-6 |
0.763 |
|
| 1989 |
Fink JK, Ravin P, Argoff CE, Levine RA, Brady RO, Hallett M, Barton NW. Tetrahydrobiopterin administration in biopterin-deficient progressive dystonia with diurnal variation. Neurology. 39: 1393-5. PMID 2477772 DOI: 10.1212/Wnl.39.10.1393 |
0.744 |
|
| 1988 |
Vanier MT, Wenger DA, Comly ME, Rousson R, Brady RO, Pentchev PG. Niemann-Pick disease group C: clinical variability and diagnosis based on defective cholesterol esterification. A collaborative study on 70 patients. Clinical Genetics. 33: 331-48. PMID 3378364 DOI: 10.1111/J.1399-0004.1988.Tb03460.X |
0.346 |
|
| 1988 |
Brady RO, Quarles RH. Developmental and pathophysiological aspects of the myelin-associated glycoprotein. Cellular and Molecular Neurobiology. 8: 139-48. PMID 2457442 DOI: 10.1007/Bf00711240 |
0.309 |
|
| 1986 |
Cowan MJ, Brady RO, Widder KJ. Elevated erythrocyte adenosine deaminase activity in patients with acquired immunodeficiency syndrome. Proceedings of the National Academy of Sciences of the United States of America. 83: 1089-91. PMID 3006027 DOI: 10.1073/Pnas.83.4.1089 |
0.312 |
|
| 1985 |
Murray GJ, Doebber TW, Shen TY, Wu MS, Ponpipom MM, Bugianesi RL, Brady RO, Barranger JA. Targeting of synthetically glycosylated human placental glucocerebrosidase. Biochemical Medicine. 34: 241-6. PMID 4084247 DOI: 10.1016/0006-2944(85)90117-6 |
0.309 |
|
| 1985 |
Weintraub H, Abramovici A, Sandbank U, Pentchev PG, Brady RO, Sekine M, Suzuki A, Sela B. Neurological mutation characterized by dysmyelination in NCTR-Balb/C mouse with lysosomal lipid storage disease. Journal of Neurochemistry. 45: 665-72. PMID 4031853 DOI: 10.1111/J.1471-4159.1985.Tb04044.X |
0.309 |
|
| 1985 |
Pentchev PG, Comly ME, Kruth HS, Vanier MT, Wenger DA, Patel S, Brady RO. A defect in cholesterol esterification in Niemann-Pick disease (type C) patients. Proceedings of the National Academy of Sciences of the United States of America. 82: 8247-51. PMID 3865225 DOI: 10.1073/Pnas.82.23.8247 |
0.304 |
|
| 1985 |
Ilyas AA, Quarles RH, Dalakas MC, Fishman PH, Brady RO. Monoclonal IgM in a patient with paraproteinemic polyneuropathy binds to gangliosides containing disialosyl groups. Annals of Neurology. 18: 655-9. PMID 2417543 DOI: 10.1002/Ana.410180605 |
0.31 |
|
| 1985 |
Yanagisawa K, Quarles RH, Johnson D, Brady RO, Whitaker JN. A derivative of myelin-associated glycoprotein in cerebrospinal fluid of normal subjects and patients with neurological disease. Annals of Neurology. 18: 464-9. PMID 2416268 DOI: 10.1002/Ana.410180409 |
0.302 |
|
| 1984 |
Pirruccello S, Barranger JA, Barton NW, Brady RO, Ginns EI. Molecular weight characterization of beta-D-glucocerebrosidase in mononuclear white blood cells in Gaucher's disease. Biochemical Medicine. 31: 73-9. PMID 6743300 DOI: 10.1016/0006-2944(84)90061-9 |
0.763 |
|
| 1983 |
Ginns EI, Tegelaers FP, Barneveld R, Galjaard H, Reuser AJ, Brady RO, Tager JM, Barranger JA. Determination of Gaucher's disease phenotypes with monoclonal antibody. Clinica Chimica Acta; International Journal of Clinical Chemistry. 131: 283-7. PMID 6883722 DOI: 10.1016/0009-8981(83)90097-9 |
0.324 |
|
| 1982 |
Ginns EI, Brady RO, Pirruccello S, Moore C, Sorrell S, Furbish FS, Murray GJ, Tager J, Barranger JA. Mutations of glucocerebrosidase: discrimination of neurologic and non-neurologic phenotypes of Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 79: 5607-10. PMID 6957882 DOI: 10.1073/Pnas.79.18.5607 |
0.327 |
|
| 1982 |
Brady RO. Lysosomal storage diseases. Pharmacology & Therapeutics. 19: 327-36. PMID 6821325 DOI: 10.1016/0163-7258(82)90072-9 |
0.326 |
|
| 1981 |
Pentchev PG, Gal AE, Wong R, Morrone S, Neumeyer B, Massey J, Kanter R, Sawitsky A, Brady RO. Biliary excretion of glycolipid in induced or inherited glucosylceramide lipidosis. Biochimica Et Biophysica Acta. 665: 615-8. PMID 7295755 DOI: 10.1016/0005-2760(81)90279-4 |
0.312 |
|
| 1981 |
Brady RO, Barranger JA. Therapeutic strategies for lipid storage diseases Trends in Neurosciences. 4: 265-267. DOI: 10.1016/0166-2236(81)90083-7 |
0.353 |
|
| 1980 |
Ginns EI, Brady RO, Stowens DW, Furbish FS, Barranger JA. A new group of glucocerebrosidase isozymes found in human white blood cells. Biochemical and Biophysical Research Communications. 97: 1103-7. PMID 7470138 DOI: 10.1016/0006-291X(80)91489-8 |
0.313 |
|
| 1980 |
Gal AE, Brady RO, Barranger JA, Pentchev PG. The diagnosis of type A and type B Niemann Pick disease and detection of carriers using leukocytes and a chromogenic analogue of sphingomyelin. Clinica Chimica Acta; International Journal of Clinical Chemistry. 104: 129-32. PMID 7389124 DOI: 10.1016/0009-8981(80)90143-6 |
0.33 |
|
| 1980 |
Pentchev PG, Gal AE, Booth AD, Omodeo-Sale F, Fouks J, Neumeyer BA, Quirk JM, Dawson G, Brady RO. A lysosomal storage disorder in mice characterized by a dual deficiency of sphingomyelinase and glucocerebrosidase. Biochimica Et Biophysica Acta. 619: 669-79. PMID 6257302 DOI: 10.1016/0005-2760(80)90116-2 |
0.321 |
|
| 1980 |
Rosenberg DM, Ferrans VJ, Fulmer JD, Line BR, Barranger JA, Brady RO, Crystal RG. Chronic airflow obstruction in Fabry's disease. The American Journal of Medicine. 68: 898-905. PMID 6247911 DOI: 10.1016/0002-9343(80)90224-7 |
0.314 |
|
| 1978 |
Britton DE, Leinikki PO, Barranger JA, Brady RO. Gaucher's disease: lack of antibody response to intravenous glucocerebrosidase. Life Sciences. 23: 2517-9. PMID 732537 DOI: 10.1016/0024-3205(78)90177-7 |
0.322 |
|
| 1978 |
Furbish FS, Steer CJ, Barranger JA, Jones EA, Brady RO. The uptake of native and desialylated glucocerebrosidase by rat hepatocytes and Kupffer cells. Biochemical and Biophysical Research Communications. 81: 1047-53. PMID 666784 DOI: 10.1016/0006-291X(78)91456-0 |
0.319 |
|
| 1978 |
Pentchev PG, Kusiak JW, Barranger JA, Furbish FS, Rapoport SI, Massey JM, Brady RO. Factors that influence the uptake and turnover of glucocerebrosidase and alpha-galactosidase in mammalian liver Advances in Experimental Medicine and Biology. 101: 745-752. PMID 208371 DOI: 10.1007/978-1-4615-9071-2_69 |
0.355 |
|
| 1978 |
Brady RO. Ophthalmologic aspects of lipid storage diseases. Ophthalmology. 85: 1007-13. PMID 105334 DOI: 10.1016/S0161-6420(78)35592-5 |
0.364 |
|
| 1978 |
Pentchev PG, Brady RO, Blair HE, Britton DE, Sorrell SH. Gaucher disease: isolation and comparison of normal and mutant glucocerebrosidase from human spleen tissue. Proceedings of the National Academy of Sciences of the United States of America. 75: 3970-3. PMID 29293 DOI: 10.1073/Pnas.75.8.3970 |
0.365 |
|
| 1977 |
Duffard RO, Fishman PH, Bradley RM, Lauter CJ, Brady RO, Trams EG. Ganglioside composition and biosynthesis in cultred cells derived from CNS. Journal of Neurochemistry. 28: 1161-6. PMID 406360 DOI: 10.1111/J.1471-4159.1977.Tb12304.X |
0.305 |
|
| 1977 |
Furbish FS, Blair HE, Shiloach J, Pentchev PG, Brady RO. Enzyme replacement therapy in Gaucher's disease: large-scale purification of glucocerebrosidase suitable for human administration. Proceedings of the National Academy of Sciences of the United States of America. 74: 3560-3. PMID 269414 DOI: 10.1073/Pnas.74.8.3560 |
0.351 |
|
| 1977 |
Sakuragawa N, Sakuragaw M, Kuwabara T, Pentchev PG, Barranger JA, Brady RO. Niemann-Pick disease experimental model: sphingomyelinase reduction induced by AY-9944. Science (New York, N.Y.). 196: 317-9. PMID 66749 DOI: 10.1126/Science.66749 |
0.304 |
|
| 1977 |
Gal AE, Brady RO, Pentchev PG, Furbish FS, Suzuki K, Tanaka H, Schneider EL. A practical chromogenic procedure for the diagnosis of Krabbe's disease. Clinica Chimica Acta; International Journal of Clinical Chemistry. 77: 53-9. PMID 17489 DOI: 10.1016/0009-8981(77)90401-6 |
0.365 |
|
| 1977 |
Brady RO. Heritable catabolic and anabolic disorders of lipid metabolism. Metabolism: Clinical and Experimental. 26: 329-45. PMID 13262 DOI: 10.1016/0026-0495(77)90080-4 |
0.369 |
|
| 1976 |
Brady RO, Pentchev PG, Gal AE, Hibbert SR, Quirk JM, Mook GE, Kusiak JW, Tallman JF, Dekaban AS. Enzyme replacement therapy for the sphingolipidoses. Advances in Experimental Medicine and Biology. 68: 523-32. PMID 820173 DOI: 10.1007/978-1-4684-7735-1_34 |
0.63 |
|
| 1976 |
Brady RO. Biochemical genetics in neurology. Archives of Neurology. 33: 145-51. PMID 814883 DOI: 10.1001/Archneur.1976.00500030001001 |
0.356 |
|
| 1975 |
Tallman JF, Kolodny EH, Brady RO. The preparation of Tay-Sachs ganglioside specifically labeled in either the N-acetylneuraminosyl or N-acetylgalactosaminyl portion of the molecule. Methods in Enzymology. 35: 541-8. PMID 1121293 DOI: 10.1016/0076-6879(75)35182-3 |
0.55 |
|
| 1975 |
Fishman PH, Max SR, Tallman JF, Brady RO, Maclaren NK, Cornblath M. Deficient Ganglioside Biosynthesis: a novel human sphingolipidosis. Science (New York, N.Y.). 187: 68-70. PMID 803227 DOI: 10.1126/Science.803227 |
0.589 |
|
| 1975 |
Gal AE, Brady RO, Hibbert SR, Pentchev PG. A practical chromogenic procedure for the detection of homozygotes and heterozygous carriers of Niemann-Pick disease. The New England Journal of Medicine. 293: 632-6. PMID 239343 DOI: 10.1056/Nejm197509252931304 |
0.369 |
|
| 1975 |
Brady RO. The lipid storage diseases: new concepts and control. Annals of Internal Medicine. 82: 257-61. PMID 163609 DOI: 10.7326/0003-4819-82-2-257 |
0.375 |
|
| 1974 |
Tallman JF, Brady RO, Navon R, Padeh B. Ganglioside catabolism in hexosaminidase A-deficient adults. Nature. 252: 254-5. PMID 4421837 DOI: 10.1038/252254A0 |
0.566 |
|
| 1974 |
Brady RO, Pentchev PG, Gal AE, Hibbert SR, Dekaban AS. Replacement therapy for inherited enzyme deficiency. Use of purified glucocerebrosidase in Gaucher's disease. The New England Journal of Medicine. 291: 989-93. PMID 4415565 DOI: 10.1056/Nejm197411072911901 |
0.355 |
|
| 1974 |
Tallman JF, Brady RO, Quirk JM, Villalba M, Gal AE. Isolation and relationship of human hexosaminidases. The Journal of Biological Chemistry. 249: 3489-99. PMID 4364658 |
0.466 |
|
| 1974 |
Tallman JF, Pentchev PG, Brady RO. An enzymological approach to the lipidoses. Enzyme. 18: 136-49. PMID 4211795 |
0.448 |
|
| 1973 |
Brady RO, Johnson WG, Uhlendorf BW. Identification of heterozygous carriers of lipid storage diseases. Current status and clinical applications. The American Journal of Medicine. 51: 423-31. PMID 5155766 DOI: 10.1016/0002-9343(71)90249-X |
0.366 |
|
| 1973 |
Tallman JF, Brady RO. The purification and properties of a mammalian neuraminidase (sialidase). Biochimica Et Biophysica Acta. 293: 434-43. PMID 4711815 DOI: 10.1016/0005-2744(73)90350-1 |
0.461 |
|
| 1973 |
Schneider EL, Ellis WG, Brady RO, McCulloch JR, Epstein CJ. Infantile (type II) Gaucher's disease: in utero diagnosis and fetal pathology. The Journal of Pediatrics. 81: 1134-9. PMID 4674572 DOI: 10.1016/S0022-3476(72)80245-2 |
0.327 |
|
| 1973 |
Brady RO, Tallman JF, Johnson WG, Gal AE, Leahy WR, Quirk JM, Dekaban AS. Replacement therapy for inherited enzyme deficiency. Use of purified ceramidetrihexosidase in Fabry's disease. The New England Journal of Medicine. 289: 9-14. PMID 4196713 DOI: 10.1056/Nejm197307052890103 |
0.635 |
|
| 1972 |
Cumar FA, Tallman JF, Brady RO. The biosynthesis of a disialylganglioside by galactosyltransferase from rat brain tissue. The Journal of Biological Chemistry. 247: 2322-7. PMID 5019950 |
0.479 |
|
| 1972 |
Tallman JF, Johnson WG, Brady RO. The metabolism of Tay-Sachs ganglioside: catabolic studies with lysosomal enzymes from normal and Tay-Sachs brain tissue. The Journal of Clinical Investigation. 51: 2339-45. PMID 4639018 DOI: 10.1172/Jci107045 |
0.598 |
|
| 1972 |
Tallman JF, Brady RO. The catabolism of Tay-Sachs ganglioside in rat brain lysosomes. The Journal of Biological Chemistry. 247: 7570-5. PMID 4636324 |
0.477 |
|
| 1972 |
Tallman JF, Brady RO. [109] Neuraminidase (sialidase) from rat heart Methods in Enzymology. 28: 825-829. DOI: 10.1016/0076-6879(72)28115-0 |
0.513 |
|
| 1972 |
Quirk JM, Tallman JF, Brady RO. The preparation of trihexosyl - and tetrahexosyl gangliosides specifically labeled in the n-acetylgalactosaminyl moiety Journal of Labelled Compounds. 8: 483-494. DOI: 10.1002/JLCR.2590080314 |
0.477 |
|
| 1971 |
Brady RO, Uhlendorf BW, Jacobson CB. Fabry's disease: antenatal detection. Science (New York, N.Y.). 172: 174-5. PMID 5547732 DOI: 10.1126/Science.172.3979.174 |
0.344 |
|
| 1971 |
Tallman JF, Brady RO, Suzuki K. Enzymic activities associated with membranous cytoplasmic bodies and isolated brain lysosomes. Journal of Neurochemistry. 18: 1775-7. PMID 4328346 |
0.507 |
|
| 1970 |
Austin J, Suzuki K, Armstrong D, Brady R, Bachhawat BK, Schlenker J, Stumpf D. Studies in Globoid (Krabbe) Leukodystrophy (GLD): V. Controlled Enzymic Studies in Ten Human Cases Jama Neurology. 23: 502-512. PMID 5478272 DOI: 10.1001/Archneur.1970.00480300024003 |
0.312 |
|
| 1970 |
Brady RO. Prenatal Diagnosis of Lipid Storage Diseases Clinical Chemistry. 16: 811-815. DOI: 10.1093/Clinchem/16.10.811 |
0.343 |
|
| 1969 |
Sloan HR, Uhlendorf BW, Kanfer JN, Brady RO, Fredrickson DS. Deficiency of sphingomyelin-cleaving enzyme activity in tissue cultures derived from patients with Niemann-Pick disease. Biochemical and Biophysical Research Communications. 34: 582-8. PMID 5777777 DOI: 10.1016/0006-291X(69)90777-3 |
0.373 |
|
| 1969 |
Kolodny EH, Brady RO, Volk BW. Demonstration of an alteration of ganglioside metabolism in Tay-Sachs disease. Biochemical and Biophysical Research Communications. 37: 526-31. PMID 5388728 DOI: 10.1016/0006-291X(69)90947-4 |
0.348 |
|
| 1969 |
Brady RO. Tay-sachs disease. The New England Journal of Medicine. 281: 1243-4. PMID 5347842 DOI: 10.1056/Nejm196911272812213 |
0.366 |
|
| 1969 |
Snyder RA, Brady RO. The use of white cells as a source of diagnostic material for lipid storage diseases. Clinica Chimica Acta; International Journal of Clinical Chemistry. 25: 331-8. PMID 4895581 DOI: 10.1016/0009-8981(69)90275-7 |
0.34 |
|
| 1969 |
Brady RO. Enzymatic defects in the sphingolipidoses. Advances in Clinical Chemistry. 11: 1-19. PMID 4885754 DOI: 10.1016/S0065-2423(08)60055-4 |
0.34 |
|
| 1969 |
KATTLOVE HE, WILLIAMS JC, GAYNOR E, SPIVACK M, BRADLEY RM, BRADY RO. Gaucher Cells in Chronic Myelocytic Leukemia: An Acquired Abnormality Blood. 33: 379-390. DOI: 10.1182/Blood.V33.2.379.379 |
0.319 |
|
| 1967 |
Brady RO, Gal AE, Bradley RM, Martensson E, Warshaw AL, Laster L. Enzymatic defect in Fabry's disease. Ceramidetrihexosidase deficiency. The New England Journal of Medicine. 276: 1163-7. PMID 6023233 DOI: 10.1056/Nejm196705252762101 |
0.326 |
|
| 1966 |
Brady RO, Kanfer JN, Bradley RM, Shapiro D. Demonstration of a deficiency of glucocerebroside-cleaving enzyme in Gaucher's disease. The Journal of Clinical Investigation. 45: 1112-5. PMID 5338605 DOI: 10.1172/Jci105417 |
0.354 |
|
| 1958 |
AGRANOFF BW, BRADLEY RM, BRADY RO. The enzymatic synthesis of inositol phosphatide. The Journal of Biological Chemistry. 233: 1077-83. PMID 13598735 |
0.425 |
|
| 1958 |
AGRANOFF BW, BRADY RO. Studies with tritium-labeled inositol. Neurology. 8: 79-80. PMID 13541616 DOI: 10.1212/Wnl.8.Suppl_1.79 |
0.478 |
|
| 1957 |
AGRANOFF BW, BRADLEY RM, BRADY RO. The role of cytidine nucleotides in the formation of inositol-containing lipid. Biochimica Et Biophysica Acta. 25: 445-6. PMID 13471604 DOI: 10.1016/0006-3002(57)90508-5 |
0.497 |
|
| 1957 |
AXELROD J, BRADY RO, WITKOP B, EVARTS EV. The distribution and metabolism of lysergic acid diethylamide. Annals of the New York Academy of Sciences. 66: 435-44. PMID 13425233 DOI: 10.1111/J.1749-6632.1957.Tb40739.X |
0.415 |
|
| 1956 |
AGRANOFF BW, BRADY RO. Purification and properties of calf liver ribokinase. The Journal of Biological Chemistry. 219: 221-9. PMID 13295274 |
0.424 |
|
| 1954 |
AGRANOFF BW, BRADY RO, COLODZIN M. Differential conversion of specifically labeled glucose to C14O2. The Journal of Biological Chemistry. 211: 773-9. PMID 13221582 |
0.41 |
|
| 1953 |
BRADY RO, WALSER M, AGRANOFF BW. Elaboration of steroid hormones by surviving adrenal tissue slices obtained from thermally stressed dogs. Proceedings of the Society For Experimental Biology and Medicine. Society For Experimental Biology and Medicine (New York, N.Y.). 84: 634-6. PMID 13134240 |
0.417 |
|
| 1952 |
BRADY RO, GURIN S. The biosynthesis of fatty acids in cell-free systems. Brookhaven Symposia in Biology. 5: 162-73; discussion, . PMID 13126604 |
0.547 |
|
| 1952 |
BRADY RO, GURIN S. Biosynthesis of fatty acids by cell-free or water-soluble enzyme systems. The Journal of Biological Chemistry. 199: 421-31. PMID 12999856 |
0.562 |
|
| 1951 |
BRADY RO, LUKENS FD, GURIN S. Synthesis of radioactive fatty acids in vitro and its hormonal control. The Journal of Biological Chemistry. 193: 459-64. PMID 14907734 |
0.541 |
|
| 1951 |
BRADY RO, RABINOWITZ J, VAN BAALEN J, GURIN S. The synthesis of radioactive cholesterol and fatty acids in vitro. II. A further study of precursors. The Journal of Biological Chemistry. 193: 137-43. PMID 14907698 |
0.546 |
|
| 1951 |
BRADY RO, GURIN S. The biosynthesis of radioactive long-chain fatty acids by homogenized pigeon liver tissue. Archives of Biochemistry and Biophysics. 34: 221-2. PMID 14904055 |
0.544 |
|
| 1951 |
BRADY RO, GURIN S. The synthesis of radioactive cholesterol and fatty acids in vitro. The Journal of Biological Chemistry. 189: 371-7. PMID 14832250 |
0.545 |
|
| 1951 |
BRADY RO, LUKENS FD, GURIN S. Hormonal influence upon the in vitro synthesis of radioactive fatty acids. Science (New York, N.Y.). 113: 413-5. PMID 14817331 DOI: 10.1126/Science.113.2937.413 |
0.581 |
|
| 1950 |
BRADY RO, GURIN S. Biosynthesis of labeled fatty acids and cholesterol in experimental diabetes. The Journal of Biological Chemistry. 187: 589-96. PMID 14803440 |
0.535 |
|
| 1950 |
BRADY RO, GURIN S. The biosynthesis of radioactive fatty acids and cholesterol. The Journal of Biological Chemistry. 186: 461-9. PMID 14794641 |
0.544 |
|
| 1949 |
CRANDALL DI, BRADY RO, GURIN S. Studies of acetoacetate formation with labeled carbon; the conversion of zeta-(C7)-labeled octanoate to acetoacetate. The Journal of Biological Chemistry. 181: 845-52. PMID 15393802 |
0.52 |
|
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