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Dr. Kirby Smith is a research scientist at the Kennedy Krieger Institute. He is also a Professor in Pediatrics and Biology at the Johns Hopkins University School of Medicine.
Biographical Sketch:
Dr. Smith obtained his Ph.D. in Biology at Johns Hopkins. He currently directs Molecular Genetics at Kennedy Krieger Institute.
Research Summary:
Dr. Smith’s research centers on the genetic and pharmacologic suppression of genetically determined disorders of neurodegeneration and hemoglobinopathies. Animal models are used to investigate pathophysiology and evaluate potential therapeutic interventions.
Recent Publications/Presentations:
Kemp S, Wei H-M, Lu J-F, Braiterman LT, McGuinness MC, Moser AB, Watkins PA, Smith KD, 1998. Gene redundancy and pharmacological gene therapy: Potential for X-linked adrenoleukodystrophy. Nature Med 4:1261-1268.
Smith KD, Kemp S, Braiterman LT, Lu J-F, Wei H-M, Geraghty MT, Stetten G, Bergin JS, Pevsner J, Watkins PA, 1999. X-linked adrenoleukodystrophy: Genes, mutations and phenotypes. Neurochem Res 24:511-525.
Braiterman LT, Watkins PA, Moser AB and Smith KD, 1999. Peroxisomal Very Long Chain Fatty Acid ß-Oxidation Activity is Determined by the Level of Adrenoleukodystrophy Protein (ALDP) Expression. Molecular Genetics and Metabolism 66: 91-99.
Wei H-M, Kemp S, McGuinness MM, Moser AB, and Smith KD, 2000. Pharmacological Induction of Peroxisomes in Peroxisome Biogenesis Disorders. Annals of. Neurology 47:286-296
Lachtermacher MBR, Seuanez HN, Moser AB, Moser HW and Smith KD, 2000. Determination of 30 X-linked Adrenoleukodystrophy Mutations, including 15 Not Previously Described . Human Mutation 15: 348-353.
McGuinness MM, Wei H-M, and Smith KD, 2000. Therapeutic developments in peroxisome biogenesis disorders. Expert Opinion on Investigative Drugs 9: | 