mTORC1 is required for expression of LRPPRC and cytochrome-c oxidase but not HIF-1α in Leigh syndrome French Canadian type patient fibroblasts

Leigh syndrome French Canadian type (LSFC) is a mitochondrial disease caused by mutations in the leucine-rich pentatricopeptide repeat-containing (LRPPRC) gene leading to a reduction of cytochrome-c oxidase (COX) expression reaching 50% in skin fibroblasts. We have shown that under basal conditions,...

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Detalhes bibliográficos
Publicado no:Am J Physiol Cell Physiol
Main Authors: Mukaneza, Yvette, Cohen, Aaron, Rivard, Marie-Ève, Tardif, Jessica, Deschênes, Sonia, Ruiz, Matthieu, Laprise, Catherine, Des Rosiers, Christine, Coderre, Lise
Formato: Artigo
Idioma:Inglês
Publicado em: American Physiological Society 2019
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6689754/
https://ncbi.nlm.nih.gov/pubmed/30995105
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/ajpcell.00160.2017
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