Roscoe O. Brady

Overview

Best Publications

• d-Serine is an endogenous ligand for the glycine site of the N-methyl-d-aspartate receptor

  Jean Pierre Mothet;Angèle T. Parent;Angèle T. Parent;Herman Wolosker;Roscoe O. Brady

  1411
  Citations

• Enzymatic defect in Fabry's disease. Ceramidetrihexosidase deficiency.

  Roscoe O. Brady;Andrew E. Gal;Roy M. Bradley;Erik Martensson

  1310
  Citations

• METABOLISM OF GLUCOCEREBROSIDES. II. EVIDENCE OF AN ENZYMATIC DEFICIENCY IN GAUCHER'S DISEASE.

  Roscoe O. Brady;Julian N. Kanfer;David Shapiro

  1098
  Citations

• Biosynthesis and function of gangliosides.

  Peter H. Fishman;Roscoe O. Brady

  1061
  Citations

• Targeted disruption of the cyclin-dependent kinase 5 gene results in abnormal corticogenesis, neuronal pathology and perinatal death.

  Toshio Ohshima;Jerrold M. Ward;Chang Goo Huh;Glenn Longenecker

  1028
  Citations

• Fabry disease, an under-recognized multisystemic disorder: expert recommendations for diagnosis, management, and enzyme replacement therapy.

  Robert J. Desnick;Roscoe Brady;John Barranger;Allan J. Collins

  959
  Citations

• A Chemosensory Gene Family Encoding Candidate Gustatory and Olfactory Receptors in Drosophila

  Kristin Scott;Roscoe Brady;Anibal Cravchik;Pavel Morozov

  824
  Citations

• Elevated globotriaosylsphingosine is a hallmark of Fabry disease

  Johannes M. Aerts;Johanna E. Groener;Sijmen Kuiper;Wilma E. Donker-Koopman

  799
  Citations

• Purification of serine racemase: Biosynthesis of the neuromodulator d-serine

  Herman Wolosker;Kevin N. Sheth;Masaaki Takahashi;Jean Pierre Mothet

  640
  Citations

• Enzyme Therapy in Type 1 Gaucher Disease: Comparative Efficacy of Mannose-Terminated Glucocerebrosidase from Natural and Recombinant Sources

  Gregory A. Grabowski;Norman W. Barton;Gregory Pastores;James M. Dambrosia

  597
  Citations

• Natural history of Fabry renal disease: influence of alpha-galactosidase A activity and genetic mutations on clinical course.

  Mary H Branton;Raphael Schiffmann;Sharda G Sabnis;Gary J Murray

  571
  Citations

• A defect in cholesterol esterification in Niemann-Pick disease (type C) patients

  Peter G. Pentchev;Marcella E. Comly;Howard S. Kruth;Marie T. Vanier

  551
  Citations

• The metabolism of sphingomyelin. II. Evidence of an enzymatic deficiency in Niemann-Pick diseae.

  Roscoe O. Brady;Julian N. Kanfer;Michael B. Mock;Donald S. Fredrickson

  545
  Citations

• d-Serine as a Neuromodulator: Regional and Developmental Localizations in Rat Brain Glia Resemble NMDA Receptors

  Michael J. Schell;Roscoe O. Brady;Mark E. Molliver;Solomon H. Snyder

  529
  Citations

• Transduction of nondividing cells using pseudotyped defective high-titer HIV type 1 particles

  Jakob Reiser;George Harmison;Stephanie Kluepfel-Stahl;Roscoe O. Brady

  482
  Citations

• Infusion of α-galactosidase A reduces tissue globotriaosylceramide storage in patients with Fabry disease

  R. Schiffmann;G. J. Murray;D. Treco;P. Daniel

  458
  Citations

• α-Galactosidase A deficient mice: A model of Fabry disease

  Toshio Ohshima;Gary J. Murray;William D. Swaim;Glenn Longenecker

  457
  Citations

• The Niemann-Pick C1 Protein Resides in a Vesicular Compartment Linked to Retrograde Transport of Multiple Lysosomal Cargo

  Edward B. Neufeld;Meryl Wastney;Shutish Patel;Sundar Suresh

  449
  Citations

• High-Titer Human Immunodeficiency Virus Type 1-Based Vector Systems for Gene Delivery into Nondividing Cells

  Hideki Mochizuki;Joan P. Schwartz;Koichi Tanaka;Roscoe O. Brady

  416
  Citations

• Replacement therapy for inherited enzyme deficiency. Use of purified glucocerebrosidase in Gaucher's disease.

  Roscoe O. Brady;Peter G. Pentchev;Andrew E. Gal;Sue R. Hibbert

  393
  Citations