TY  - JOUR
AU  - Hidalgo-Gutierrez, Agustín
AU  - Barriocanal-Casado, Eliana
AU  - Bakkali, Mohammed
AU  - Diaz-Casado, M Elena
AU  - Sanchez-Maldonado, Laura
AU  - Romero, Miguel
AU  - Sayed, Ramy K
AU  - Prehn, Cornelia
AU  - Escames, Germaine
AU  - Duarte, Juan
AU  - Acuña-Castroviejo, Darío
AU  - Lopez, Luis C
PY  - 2019
DO  - 10.15252/emmm.201809466
UR  - http://hdl.handle.net/10668/13248
T2  - EMBO molecular medicine
AB  - 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...
LA  - en
KW  - 2,4‐dihydroxybenzoic acid
KW  - Q synthome
KW  - astrogliosis
KW  - mitochondrial encephalopathy
KW  - spongiosis
KW  - Animals
KW  - Brain
KW  - Disease Models, Animal
KW  - Energy Metabolism
KW  - Histocytochemistry
KW  - Hydroxybenzoates
KW  - Mice
KW  - Mitochondrial Encephalomyopathies
KW  - Neuroprotective Agents
KW  - Salicylic Acid
KW  - Survival Analysis
KW  - Treatment Outcome
KW  - Ubiquinone
TI  - β-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9R239X mice.
TY  - research article
VL  - 11
ER  -