| Autor: |
Hoshikawa, Masahiro, Kase, Ryoichi, Tadokoro, Mamoru, Sakuraba, Hitoshi, Sakiyama, Takeshi |
| Předmět: |
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| Zdroj: |
Pediatrics International; Dec2004, Vol. 46 Issue 6, p673-677, 5p, 2 Color Photographs, 1 Chart |
| Abstrakt: |
Background: Human alpha-galactosidase A (hαG) is an essential lysosomal enzyme in catalyzing the hydrolysis of ceramide trihexoside in humans. Effects have been directed to develop effective gene and replacement therapies for the deficiency of hαG, Fabry disease. In recent years, hαG transgenic mice (TGM) have been established, and the expression of hαG in their general organs has been reported. However, detailed distribution of the cells expressing hαG have not yet been defined.Methods: The distribution of hαG in organs of the hαG-TGM was studied by means of immunohistochemistry and enzyme assay.Results: Immunohistochemical analysis revealed a systematic hαG expression in the TGM, including endothelial cells of the bone marrow, liver, spleen, pancreas, lungs, uriniferous tubules in the kidneys, and choroids plexus in the brain. Enzyme assay demonstrated a persistent expression of hαG in the TGM during 14–20 months after birth.Conclusion: A long-term expression of hαG in organs may indicate hαG-TGM as a useful tool in the research of gene and replacement therapies for Fabry disease. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
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