The enzymatic complexes of the mitochondrial respiratory chain have been extensively
investigated in their structural and functional properties. A clear distinction is possible today …

Coenzyme Q 10 (CoQ 10) deficiency has been associated with 5 major clinical phenotypes:
encephalomyopathy, severe infantile multisystemic disease, nephropathy, cerebellar ataxia …

Coenzyme Q 10 (CoQ 10) is a vital lipophilic molecule that transfers electrons from
mitochondrial respiratory chain complexes I and II to complex III. Deficiency of CoQ 10 has …

Objectives Bioavailability of supplements with coenzyme Q10 (CoQ 10) in humans seems to
depend on the excipients of formulations and on physiological characteristics of the …

For a number of years, coenzyme Q10 (CoQ10) was known for its key role in mitochondrial
bioenergetics; later studies demonstrated its presence in other subcellular fractions and in …

Abstract Treatment of motor symptoms of degenerative cerebellar ataxia remains difficult.
Yet there are recent developments that are likely to lead to significant improvements in the …

Abstract Primary Coenzyme Q (CoQ) deficiencies are clinically heterogeneous conditions
and lack clear genotype-phenotype correlations, complicating diagnosis and prognostic …

Primary Coenzyme Q deficiencies represent a group of rare conditions caused by mutations
in one of the genes required in its biosynthetic pathway at the enzymatic or regulatory level …

Abstract Coenzyme Q10 (CoQ10) is an essential electron carrier in the mitochondrial
respiratory chain and an important antioxidant. Deficiency of CoQ10 is a clinically and …

Autosomal recessive cerebellar ataxias (ARCA) are a heterogeneous group of rare
neurological disorders involving both central and peripheral nervous system, and in some …