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ProteomicsIdentification of disease biomarkers and potential endocrine factors in CoQ deficiency
4-Hydroxybenzoic acid (4-HB) is the natural precursor for CoQ biosynthesis. Hydroxybenzoic acids derivatives (HBAs) have been recently used for the experimental treatment of primary CoQ deficiency. However, the mechanisms of these therapeutic effects remain obscure. In this study, we performed an an...
ORGANISM(S): Mus musculus (Mouse) 
2022-07-15 | PXD030303 | Pride
4-dihydroxybenzoic acid  plasma  mitochondria  c57bl/6 Vanillic acid  -resorcylic acid  2 
Genomicsβ-RA reduces DMQ/CoQ ratio and recues the encephalopathic phenotype in Coq9R239X mice
β-RA reduces DMQ/CoQ ratio and recues the encephalopathic phenotype in Coq9R239X mice
PRJNA492403 | ENA
Transcriptomicsβ-RA reduces DMQ/CoQ ratio and recues the encephalopathic phenotype in Coq9R239X mice
Coenzyme Q (CoQ) deficiency has been associated to 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 of fatal mitochondrial encephalopathy due to CoQ deficiency, we have tested t...
ORGANISM(S): Mus musculus 
2018-09-22 | GSE120287 | GEO
TranscriptomicsProbucol ameliorates renal and metabolic sequelae of primary CoQ deficiency in Pdss2 mutant mice
To discern whether the same transcriptional signature of mitochondrial dysfunction previously reported in B6.Alb/cre,Pdss2loxP/loxP liver-conditional knockout mice (Peng, Falk et al. PLoS Genetics, 2008 [PMID 18437205]) was present regardless of Pdss2 mutation type, as well as to assess whether phar...
ORGANISM(S): Mus musculus 
2011-06-07 | E-GEOD-27954 | biostudies-arrayexpress
UnknownManganese-driven CoQ deficiency.
Not available
S-EPMC9563070 | biostudies-literature
GenomicCoenzyme Q10 (CoQ) in Huntington's Disease (HD) (2CARE)

This is a multi-center, randomized, double-blind, placebo-controlled trial of CoQ involving 609 ambulatory subjects with HD who are not requiring skilled care or institutionalization. Eligible subjects were randomized to receive either CoQ 2400 mg/day or matching placebo. Subjects were followed p...

phs001065 | dbGaP
UnknownUbiB proteins regulate cellular CoQ distribution in Saccharomyces cerevisiae.
Not available
S-EPMC8346625 | biostudies-literature
UnknownCoQ imbalance drives reverse electron transport to disrupt liver metabolism.
Not available
S-EPMC12758653 | biostudies-literature
UnknownElevated cellular accumulation of endogenous and exogenous CoQ by altered intracellular trafficking.
Not available
S-EPMC12702331 | biostudies-literature
UnknownCoQ Regulates Brown Adipose Tissue Respiration and Uncoupling Protein 1 Expression.
Not available
S-EPMC9854525 | biostudies-literature
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