PloS one · 2011
Lyso-GM2 ganglioside: a possible biomarker of Tay-Sachs disease and Sandhoff disease.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Kodama Takashi, Togawa Tadayasu, Tsukimura Takahiro, Kawashima Ikuo, Matsuoka Kazuhiko, Kitakaze Keisuke, Tsuji Daisuke, Itoh Kohji, Ishida Yo-Ichi, Suzuki Minoru, Suzuki Toshihiro, Sakuraba Hitoshi
Аннотация
To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2) levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified hexosaminidase (Hex) B exhibiting Hex A-like activity was examined. Then, the lyso-GM2 concentrations in human plasma samples were determined. The lyso-GM2 levels in the brain and plasma in Sandhoff mice were apparently increased compared with those in wild-type mice, and they decreased on intracerebroventricular administration of the modified Hex B. The lyso-GM2 levels in plasma of patients with Tay-Sachs disease and Sandhoff disease were increased, and the increase in lyso-GM2 was associated with a decrease in Hex A activity. Lyso-GM2 is expected to be a potential biomarker of Tay-Sachs disease and Sandhoff disease.