Acquired coenzyme Q10 deficiency in children with recurrent food intolerance and allergies

Importance of muscle light microscopic mitochondrial subsarcolemmal aggregates in the diagnosis of respiratory chain deficiency

“…However, mitochondrial proliferation with increased SSMA in myofibers has been described elsewhere in children with and without RCD [1,12,23,29,30]. The current results indicate that ETC complex deficiencies in skeletal muscle are more commonly associated with SSMA 4% or less than with SSMA 10% or more (Fig.…”

Oxidized proportion of muscle coenzyme Q10 increases with age in healthy children

“…Reports comparing plasma and tissue CoQ10 concentrations are rare. Duncan et al (30) found no correlation between plasma and muscle CoQ10, while Miles et al (35) found correlations between basal plasma CoQ10 levels and activities of mitochondrial respiratory chain complexes II and I+III in children with recurrent food intolerance and allergies. In the current study, we found no significant correlations between CoQ10 concentrations or redox statuses in plasma versus muscle tissue in children.…”

Survival transcriptome in the coenzyme Q10deficiency syndrome is acquired by epigenetic modifications: a modelling study for human coenzyme Q10deficiencies

“…[8][9][10][11][12][13] Although each form presents a specific clinical phenotype, all these conditions display a substantial reduction of cellular CoQ 10 content and deficit in the mitochondrial enzymatic activities of respiratory chain (table 1). Accordingly to these results, we have shown here that fibroblasts from patients with CoQ 10 deficiency have reorganised their genetic resources to cope with this mitochondrial dysfunction.…”

“…7 Secondary CoQ 10 deficiency has also been associated with diverse mitochondrial diseases. [8][9][10][11][12][13] In all of these conditions, CoQ 10 supplementation partially improves symptoms 14 15 and usually induces a return to normal growth and respiration in CoQ 10 -deficient fibroblasts. 8 16 17 Adaptation of somatic cells to CoQ 10 deficiency may affect both onset and course of the disease.…”