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Copper and bezafibrate cooperate to rescue cytochrome c oxidase deficiency in cells of patients with sco2 mutations

BACKGROUND: Mutations in SCO2 cause cytochrome c oxidase deficiency (COX) and a fatal infantile cardioencephalomyopathy. SCO2 encodes a protein involved in COX copper metabolism; supplementation with copper salts rescues the defect in patients’ cells. Bezafibrate (BZF), an approved hypolipidemic age...

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Hauptverfasser: Casarin, Alberto, Giorgi, Gianpietro, Pertegato, Vanessa, Siviero, Roberta, Cerqua, Cristina, Doimo, Mara, Basso, Giuseppe, Sacconi, Sabrina, Cassina, Matteo, Rizzuto, Rosario, Brosel, Sonja, M Davidson, Mercy, DiMauro, Salvatore, Schon, Eric A, Clementi, Maurizio, Trevisson, Eva, Salviati, Leonardo
Format: Artigo
Sprache:Inglês
Veröffentlicht: BioMed Central 2012
Schlagworte:
Research
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3445839/
https://ncbi.nlm.nih.gov/pubmed/22515166
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1750-1172-7-21
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