Total and reduced/oxidized forms of coenzyme Q₁₀ in fibroblasts of patients with mitochondrial disease.

ABSTRACT: Coenzyme Q₁₀ (CoQ₁₀) is involved in ATP production through electron transfer in the mitochondrial respiratory chain complex. CoQ₁₀ receives electrons from respiratory chain complex I and II to become the reduced form, and then transfers electrons at complex III to become the oxidized form. The redox state of CoQ₁₀ has been reported to be a marker of the mitochondrial metabolic state, but to our knowledge, no reports have focused on the individual quantification of reduced and oxidized CoQ₁₀ or the ratio of reduced to total CoQ₁₀ (reduced/total CoQ₁₀) in patients with mitochondrial diseases. We measured reduced and oxidized CoQ₁₀ in skin fibroblasts from 24 mitochondrial disease patients, including 5 pri

SUBMITTER: Watanabe C 

PROVIDER: S-EPMC9826971 | biostudies-literature | 2023 Mar

REPOSITORIES: biostudies-literature