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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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Bibliografske podrobnosti
Main Authors: Atorino, Luigia, Silvestri, Laura, Koppen, Mirko, Cassina, Laura, Ballabio, Andrea, Marconi, Roberto, Langer, Thomas, Casari, Giorgio
Format: Artigo
Jezik:Inglês
Izdano: The Rockefeller University Press 2003
Teme:
Online dostop: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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