Coenzyme Q (CoQ, ubiquinone) is a redox-active lipid produced across all domains of life
that functions in electron transport and oxidative phosphorylation and whose deficiency …

Hereditary defects in the repair of DNA damage are implicated in a variety of diseases, many
of which are typified by neurological dysfunction and/or increased genetic instability and …

Identification of single-gene causes of steroid-resistant nephrotic syndrome (SRNS) has
furthered the understanding of the pathogenesis of this disease. Here, using a combination …

Introduction In the last 10 years the field of mitochondrial genetics has widened, shifting the
focus from rare sporadic, metabolic disease to the effects of mitochondrial DNA (mtDNA) …

Mitochondrial diseases (encephalomyopathies) have traditionally been ascribed to defects
of the respiratory chain, which has helped researchers explain their genetic and clinical …

Coenzyme Q 10 is a remarkable lipid involved in many cellular processes such as energy
production through the mitochondrial respiratory chain (RC), beta-oxidation of fatty acids …

Although two main hypotheses of mitochondrial origin have been proposed, ie, the
autogenous and the endosymbiotic, only the second is being seriously considered currently …

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 …

The aging process includes impairment in mitochondrial function, a reduction in anti-oxidant
activity, and an increase in oxidative stress, marked by an increase in reactive oxygen …

Abstract Primary coenzyme Q 10 (CoQ 10) deficiency includes a group of rare autosomal
recessive disorders primarily characterized by neurological and muscular symptoms. Rarely …