Pediatrics international : official journal of the Japan Pediatric Society · 2004
Long-term expressed human alpha-galactosidase A in tissues of HalphaG transgenic mice.
Сравнительное исследованиеСтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Hoshikawa Masahiro, Kase Ryoichi, Tadokoro Mamoru, Sakuraba Hitoshi, Sakiyama Takeshi
Аннотация
BACKGROUND: Human alpha-galactosidase A (halphaG) 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 halphaG, Fabry disease. In recent years, halphaG transgenic mice (TGM) have been established, and the expression of halphaG in their general organs has been reported. However, detailed distribution of the cells expressing halphaG have not yet been defined.
METHODS: The distribution of halphaG in organs of the halphaG-TGM was studied by means of immunohistochemistry and enzyme assay.
RESULTS: Immunohistochemical analysis revealed a systematic halphaG 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 halphaG in the TGM during 14-20 months after birth.
CONCLUSION: A long-term expression of halphaG in organs may indicate halphaG-TGM as a useful tool in the research of gene and replacement therapies for Fabry disease.
METHODS: The distribution of halphaG in organs of the halphaG-TGM was studied by means of immunohistochemistry and enzyme assay.
RESULTS: Immunohistochemical analysis revealed a systematic halphaG 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 halphaG in the TGM during 14-20 months after birth.
CONCLUSION: A long-term expression of halphaG in organs may indicate halphaG-TGM as a useful tool in the research of gene and replacement therapies for Fabry disease.