Loss of hepatic LRPPRC alters mitochondrial bioenergetics, regulation of permeability transition and trans-membrane ROS diffusion

The French-Canadian variant of Leigh Syndrome (LSFC) is an autosomal recessive oxidative phosphorylation (OXPHOS) disorder caused by a mutation in LRPPRC, coding for a protein involved in the stability of mitochondrially-encoded mRNAs. Low levels of LRPPRC are present in all patient tissues, but res...

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Detalhes bibliográficos
Publicado no:Hum Mol Genet
Main Authors: Cuillerier, Alexanne, Honarmand, Shamisa, Cadete, Virgilio J.J., Ruiz, Matthieu, Forest, Anik, Deschênes, Sonia, Beauchamp, Claudine, Charron, Guy, Rioux, John D., Des Rosiers, Christine, Shoubridge, Eric A., Burelle, Yan
Formato: Artigo
Idioma:Inglês
Publicado em: Oxford University Press 2017
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Articles
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5886084/
https://ncbi.nlm.nih.gov/pubmed/28575497
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddx202
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