Mitochondrial Vulnerability and Increased Susceptibility to Nutrient-Induced Cytotoxicity in Fibroblasts from Leigh Syndrome French Canadian Patients

Mutations in LRPPRC are responsible for the French Canadian variant of Leigh Syndrome (LSFC), a severe disorder characterized biochemically by a tissue-specific deficiency of cytochrome c oxidase (COX) and clinically by the occurrence of severe and deadly acidotic crises. Factors that precipitate th...

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Publicat a:PLoS One
Autors principals: Burelle, Yan, Bemeur, Chantal, Rivard, Marie-Eve, Thompson Legault, Julie, Boucher, Gabrielle, Morin, Charles, Coderre, Lise, Des Rosiers, Christine
Format: Artigo
Idioma:Inglês
Publicat: Public Library of Science 2015
Matèries:
Research Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4383560/
https://ncbi.nlm.nih.gov/pubmed/25835550
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0120767
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