Roscoe Brady
Roscoe Owen Brady ( * 1923 in Philadelphia, Pennsylvania) is an American biochemist and geneticist at the National Institute of Neurological Disorders and Stroke in Bethesda, Maryland.
Life
After attending the Pennsylvania State University Brady acquired in 1947 at Harvard Medical School, an MD as graduation from medical school. As a postdoctoral fellow, he worked in the Department of Biochemistry at the University of Pennsylvania School of Medicine, where he was also a doctor in internal medicine. After two and a half years of military service for the U.S. Naval Medical Corps Brady went in 1952 to the National Institutes of Health ( NIH) of the United States. There he was head of the Department of Developmental Neurology and neurological metabolism ( Developmental and Metabolic Neurology ) at the National Institute of Neurological Disorders and Stroke, a facility of the NIH, based in Bethesda, Maryland. In 2006 he became Professor Emeritus.
Work
Brady could contribute significantly to the understanding of the molecular mechanisms of Sphingolipidoses ( sphingolipid storage diseases ). He discovered various enzyme defects of lipid metabolism as a specific cause, for example, of the Gaucher's disease, Niemann -Pick disease, Fabry disease or Tay- Sachs disease. Brady developed procedures of genetic counseling and was able to establish successful enzyme replacement therapies for hitherto incurable diseases. Brady suggested that the successful establishment of numerous biotechnology companies that produce the appropriate therapeutic substances.
More recent work focuses on the genetic causes of neurodegenerative diseases such as mucolipidosis IV, leukodystrophies of unknown etiology and new forms of peripheral neuropathy. The previously unrecognized diseases Childhood Ataxia with Central Nervous System Hypomyelination ( CACH ) and Ovarioleukodystrophie were identified. New methods to determine the efficacy of enzyme therapy, and new methods of gene therapy of inherited neurological metabolic disorders have been developed.