β‐RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9 (R239X) mice

Coenzyme Q (CoQ) deficiency has been associated with primary defects in the CoQ biosynthetic pathway or to secondary events. In some cases, the exogenous CoQ supplementation has limited efficacy. In the Coq9 (R239X) mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we have t...

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Detalles Bibliográficos
Autores principales: Hidalgo‐Gutiérrez, Agustín, Barriocanal‐Casado, Eliana, Bakkali, Mohammed, Díaz‐Casado, M Elena, Sánchez‐Maldonado, Laura, Romero, Miguel, Sayed, Ramy K, Prehn, Cornelia, Escames, Germaine, Duarte, Juan, Acuña‐Castroviejo, Darío, López, Luis C
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2018
Materias:
Research Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6328940/
https://www.ncbi.nlm.nih.gov/pubmed/30482867
http://dx.doi.org/10.15252/emmm.201809466

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6328940/
https://www.ncbi.nlm.nih.gov/pubmed/30482867
http://dx.doi.org/10.15252/emmm.201809466

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