論文 - 詳細
| RRC ID | 38515 |
|---|---|
| 著者 | Kodama T, Togawa T, Tsukimura T, Kawashima I, Matsuoka K, Kitakaze K, Tsuji D, Itoh K, Ishida Y, Suzuki M, Suzuki T, Sakuraba H. |
| タイトル | Lyso-GM2 ganglioside: a possible biomarker of Tay-Sachs disease and Sandhoff disease. |
| ジャーナル | PLoS One |
| Abstract |
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. |
| 巻・号 | 6(12) |
| ページ | e29074 |
| 公開日 | 2011-1-1 |
| DOI | 10.1371/journal.pone.0029074 |
| PII | PONE-D-11-20535 |
| PMID | 22205997 |
| PMC | PMC3243693 |
| MeSH | Adult Animals Biomarkers / blood Biomarkers / metabolism Brain / metabolism G(M2) Activator Protein / deficiency G(M2) Ganglioside / analogs & derivatives* G(M2) Ganglioside / blood G(M2) Ganglioside / metabolism Hexosaminidases / blood Humans Infant Mice Sandhoff Disease / blood Sandhoff Disease / enzymology Sandhoff Disease / metabolism* Tay-Sachs Disease / blood Tay-Sachs Disease / enzymology Tay-Sachs Disease / metabolism* |
| IF | 2.74 |
| 引用数 | 15 |
| WOS 分野 | BIOCHEMISTRY & MOLECULAR BIOLOGY |
| リソース情報 | |
| ヒト・動物細胞 | CHO |