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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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Detaylı Bibliyografya
Asıl Yazarlar: Yan Burelle, Chantal Bemeur, Marie-Eve Rivard, Julie Thompson Legault, Gabrielle Boucher, LSFC Consortium, Charles Morin, Lise Coderre, Christine Des Rosiers
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: Public Library of Science (PLoS) 2015-01-01
Seri Bilgileri:PLoS ONE
Online Erişim:https://doi.org/10.1371/journal.pone.0120767
Etiketler: Etiketle
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