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

  • Published:2015-04-02 Issue:4 Volume:10 Page:e0120767
  • ISSN:1932-6203
  • Container-title:PLOS ONE
  • language:en
  • Short-container-title:PLoS ONE

Author:

Burelle Yan,Bemeur Chantal,Rivard Marie-Eve,Thompson Legault Julie,Boucher Gabrielle,Morin Charles,Coderre Lise,Des Rosiers Christine,

Publisher

Public Library of Science (PLoS)

Subject

Multidisciplinary

Reference69 articles.

1. A biochemically distinct form of cytochrome oxidase (COX) deficiency in the Saguenay-Lac-Saint-Jean region of Quebec;F Merante;Am J Hum Genet,1993

2. Clinical, metabolic, and genetic aspects of cytochrome C oxidase deficiency in Saguenay-Lac-Saint-Jean;C Morin;Am J Hum Genet,1993

3. LRPPRC mutations cause a phenotypically distinct form of Leigh syndrome with cytochrome c oxidase deficiency;F-G Debray;J Med Genet,2011

4. LRPPRC and SLIRP Interact in a Ribonucleoprotein Complex That Regulates Posttranscriptional Gene Expression in Mitochondria;F Sasarman;Mol Biol Cell,2010

5. The role of the LRPPRC (leucine-rich pentatricopeptide repeat cassette) gene in cytochrome oxidase assembly: mutation causes lowered levels of COX (cytochrome c oxidase) I and COX III mRNA;F Xu;Biochem J,2004
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