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...

ver descrição completa

Na minha lista:
Detalhes bibliográficos
Main Authors: 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
Formato: Artigo
Idioma:Inglês
Publicado em: BioMed Central 2012
Assuntos:
Research
Acesso em linha: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
Tags: Adicionar Tag
Sem tags, seja o primeiro a adicionar uma tag!

Registos relacionados