Data presented in this paper show that the size of the endogenous coenzyme Q (CoQ) pool is not a limiting factor in the activation of mitochondrial glycerophosphate-dependent respiration by exogenous CoQ(3), since successive additions of succinate and NADH to brown adipose tissue mitochondria further increase the rate of oxygen uptake. Because the inhibition of glycerophosphate-dependent respir...

This article discusses the integration of the authoring of a mathematical document with the formalisation of the mathematics contained in that document. To achieve this we have started the development of a Coq plugin for the TEXmacs scientific editor, called tmEgg. TEXmacs allows the wysiwyg editing of mathematical documents, much in the style of LATEX. Our plugin allows to integrate into a TEX...

The presence of Coenzyme Q (CoQ) in food, its role in cellular bioenergetics and antioxidant protection and the key role played by dietary fatty acids on membrane structure support the interest for a wide research concerning the relationship between dietary fats, CoQ content and biochemical behaviour. Several models of peroxidative stress 'in vivo' have been extensively investigated in our labo...

Primary coenzyme Q10 (CoQ10) deficiency is due to mutations in genes involved in CoQ biosynthesis. The disease has been associated with five major phenotypes, but a genotype-phenotype correlation is unclear. Here, we compare two mouse models with a genetic modification in Coq9 gene (Coq9(Q95X) and Coq9(R239X)), and their responses to 2,4-dihydroxybenzoic acid (2,4-diHB). Coq9(R239X) mice manife...

Deduction-based software verification tools have reached a maturity allowing them to be used in industrial context where a very high level of assurance is required. This raises the question of the level of confidence we can grant to the tools themselves. We present a certified implementation of a verification condition generator. An originality is its genericity with respect to the logical cont...

We describe an implementation and a proof of correctness of binary decision diagrams (BDDs), completely formalized in Coq. This allows us to run BDD-based algorithms inside Coq and paves the way for a smooth integration of symbolic model checking in the Coq proof assistant by using reeection. It also gives us, by Coq's extraction mechanism, certiied BDD algorithms implemented in Caml. We also i...

We analyzed plasma 8OHdG concentrations in 20 individuals enrolled in the Pre-2CARE study before and after treatment with CoQ. Treatment resulted in a mean reduction in 8OHdG of 2.9 ± 2.9 pg/ml for the cohort (p = 0.0003) and 3.0 ± 2.6 pg/ml, for the HD group (p = 0.002). Baseline 8OHdG levels were not different between individuals with HD and controls (19.3 ± 3.2 pg/ml vs. 19.5 ± 4.7 pg/ml, p ...

UNLABELLED Our aim was to report a new case with cerebellar ataxia associated with coenzyme Q10 (CoQ) deficiency, the biochemical findings caused by this deficiency and the response to CoQ supplementation. PATIENT A 12-year-old girl presenting ataxia and cerebellar atrophy. BIOCHEMICAL STUDIES: Coenzyme Q10 in muscle was analysed by HPLC with electrochemical detection and mitochondrial respir...

Coenzyme Q (CoQ) is an electron acceptor for sulfide-quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by phar...

Coq3 O-methyltransferase carries out both O-methylation steps in coenzyme Q (ubiquinone) biosynthesis. The degree to which Coq3 O-methyltransferase activity and expression are dependent on the other seven COQ gene products has been investigated. A panel of yeast mutant strains harboring null mutations in each of the genes required for coenzyme Q biosynthesis (COQ1-COQ8) have been prepared. Mito...