Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia

Mmutations in paraplegin, a putative mitochondrial metallopeptidase of the AAA family, cause an autosomal recessive form of hereditary spastic paraplegia (HSP). Here, we analyze the function of paraplegin at the cellular level and characterize the phenotypic defects of HSP patients' cells lacki...

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Main Authors: Atorino, Luigia, Silvestri, Laura, Koppen, Mirko, Cassina, Laura, Ballabio, Andrea, Marconi, Roberto, Langer, Thomas, Casari, Giorgio
Formato: Artigo
Idioma:Inglês
Publicado: The Rockefeller University Press 2003
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Article
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC2173682/
https://ncbi.nlm.nih.gov/pubmed/14623864
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1083/jcb.200304112
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