Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Project (R01)
Project #
7R01NS024289-01
Application #
3408729
Study Section
Neurological Sciences Subcommittee 1 (NLS)
Project Start
1986-03-01
Project End
1992-08-31
Budget Start
1986-03-01
Budget End
1986-08-31
Support Year
1
Fiscal Year
1985
Total Cost
Indirect Cost
Name
University of North Carolina Chapel Hill
Department
Type
Schools of Medicine
DUNS #
078861598
City
Chapel Hill
State
NC
Country
United States
Zip Code
27599
Suzuki, Kunihiko (2003) Globoid cell leukodystrophy (Krabbe's disease): update. J Child Neurol 18:595-603
Suzuki, K; Ezoe, T; Tohyama, J et al. (2003) Are animal models useful for understanding the pathophysiology of lysosomal storage disease? Acta Paediatr Suppl 92:54-62; discussion 45
Matsuda, J; Vanier, M T; Saito, Y et al. (2001) A mutation in the saposin A domain of the sphingolipid activator protein (prosaposin) gene results in a late-onset, chronic form of globoid cell leukodystrophy in the mouse. Hum Mol Genet 10:1191-9
Wu, Y P; McMahon, E J; Matsuda, J et al. (2001) Expression of immune-related molecules is downregulated in twitcher mice following bone marrow transplantation. J Neuropathol Exp Neurol 60:1062-74
Matsuda, J; Vanier, M T; Saito, Y et al. (2001) Dramatic phenotypic improvement during pregnancy in a genetic leukodystrophy: estrogen appears to be a critical factor. Hum Mol Genet 10:2709-15
Tohyama, J; Matsuda, J; Suzuki, K (2001) Psychosine is as potent an inducer of cell death as C6-ceramide in cultured fibroblasts and in MOCH-1 cells. Neurochem Res 26:667-71
Morales, C R; Zhao, Q; El-Alfy, M et al. (2000) Targeted disruption of the mouse prosaposin gene affects the development of the prostate gland and other male reproductive organs. J Androl 21:765-75
Tohyama, J; Vanier, M T; Suzuki, K et al. (2000) Paradoxical influence of acid beta-galactosidase gene dosage on phenotype of the twitcher mouse (genetic galactosylceramidase deficiency). Hum Mol Genet 9:1699-707
Ezoe, T; Vanier, M T; Oya, Y et al. (2000) Biochemistry and neuropathology of mice doubly deficient in synthesis and degradation of galactosylceramide. J Neurosci Res 59:170-8
Fujimoto, H; Tadano-Aritomi, K; Tokumasu, A et al. (2000) Requirement of seminolipid in spermatogenesis revealed by UDP-galactose: Ceramide galactosyltransferase-deficient mice. J Biol Chem 275:22623-6

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