Normal platelet mitochondrial complex I activity in Huntington's disease

NEUROBIOLOGY OF DISEASE

卷:27

Article

Powers, William J. ; Haas, Richard H. ; Le, Thuy ; Videen, Tom O. ; Hershey, Tamara ; McGee-Minnich, Lori ; Perlmutter, Joel S.

关键词: Huntington's disease;    mitochondria;    electron transport system;    platelets;    human;   

DOI  :  10.1016/j.nbd.2007.04.008

来源: Elsevier

【 摘 要 】

Two small case series of platelet mitochondrial complex I activity assays in Huntington's Disease (HD) report discrepant results. We measured platelet complex I and complex I/III activity in 21 subjects with early gene-positive HD and 14 age-matched controls. The 21 participants with HD that we studied are greater than the total of 16 in the two previously published of platelet ETS activity in HD. Reductions > 10% were excluded with 80% confidence. A systemic defect in complex I activity is not present in early HD when striatal neuronal degeneration is already present. (c) 2007 Elsevier Inc. All rights reserved.

【 授权许可】

Free