Respiratory chain dysfunction and oxidative stress correlate with severity of primary CoQ(10) deficiency

Coenzyme Q(10) (CoQ(10)) is essential for electron transport in the mitochondrial respiratory chain and antioxidant defense. Last year, we reported the first mutations in CoQ(10) biosynthetic genes, COQ2, which encodes 4-parahydroxybenzoate: polyprenyl transferase; and PDSS2, which encodes subunit 2...

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Autores principales: Quinzii, Catarina M., López, Luis C., Von-Moltke, Jakob, Naini, Ali, Krishna, Sindu, Schuelke, Markus, Salviati, Leonardo, Navas, Plácido, DiMauro, Salvatore, Hirano, Michio
Formato: Artigo
Lenguaje:Inglês
Publicado: 2008
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Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC2731482/
https://ncbi.nlm.nih.gov/pubmed/18230681
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1096/fj.07-100149
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