β‐RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9R239X mice

β‐RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9R239X mice

Abstract 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 Coq9R239X mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we...

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Bibliographic Details
Main Authors: Agustín Hidalgo‐Gutiérrez, Eliana Barriocanal‐Casado, Mohammed Bakkali, M Elena Díaz‐Casado, Laura Sánchez‐Maldonado, Miguel Romero, Ramy K Sayed, Cornelia Prehn, Germaine Escames, Juan Duarte, Darío Acuña‐Castroviejo, Luis C López
Format: Article
Language:English
Published: Springer Nature 2019-01-01
Series:EMBO Molecular Medicine
Subjects:
2,4‐dihydroxybenzoic acid
astrogliosis
mitochondrial encephalopathy
Q synthome
spongiosis
Online Access:https://doi.org/10.15252/emmm.201809466

Internet

https://doi.org/10.15252/emmm.201809466

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