Ontology highlight
ABSTRACT:
SUBMITTER: Perier C
PROVIDER: S-EPMC1323177 | biostudies-literature | 2005 Dec
REPOSITORIES: biostudies-literature
Perier Celine C Tieu Kim K Guégan Christelle C Caspersen Casper C Jackson-Lewis Vernice V Carelli Valerio V Martinuzzi Andrea A Hirano Michio M Przedborski Serge S Vila Miquel M
Proceedings of the National Academy of Sciences of the United States of America 20051219 52
Dysfunction of mitochondrial complex I is a feature of human neurodegenerative diseases such as Leber hereditary optic neuropathy and Parkinson's disease. This mitochondrial defect is associated with a recruitment of the mitochondrial-dependent apoptotic pathway in vivo. However, in isolated brain mitochondria, complex I dysfunction caused by either pharmacological or genetic means fails to directly activate this cell death pathway. Instead, deficits of complex I stimulate intramitochondrial oxi ...[more]