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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Detalles Bibliográficos
Autores principales: Atorino, Luigia, Silvestri, Laura, Koppen, Mirko, Cassina, Laura, Ballabio, Andrea, Marconi, Roberto, Langer, Thomas, Casari, Giorgio
Formato: Texto
Lenguaje:English
Publicado: The Rockefeller University Press 2003
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2173682/
https://www.ncbi.nlm.nih.gov/pubmed/14623864
http://dx.doi.org/10.1083/jcb.200304112

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2173682/
https://www.ncbi.nlm.nih.gov/pubmed/14623864
http://dx.doi.org/10.1083/jcb.200304112

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