Article
Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome.
Proceedings of the National Academy of Sciences of the United States of America - 15 Jun 2010
Quintana Albert, Kruse Shane E, Kapur Raj P, Sanz Elisenda, Palmiter Richard D
Abstract excerpt
To explore the lethal, ataxic phenotype of complex I deficiency in Ndufs4 knockout (KO) mice, we inactivated Ndufs4 selectively in neurons and glia (NesKO mice). NesKO mice manifested the same symptoms as KO mice including retarded growth, loss of motor ability, breathing abnormalities, and death by approximately 7 wk. Progressive neuronal deterioration and gliosis in specific brain areas corresponded to...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.