ubiquinone and Xeroderma-Pigmentosum

Serum concentrations of CoQ were measured on 22 xeroderma pigmentosum (XP) patients. 10 severely handicapped patients and 32 healthy children and adults. CoQ levels in XP patients (0.5 +/- 0.16 microgram/ml) and handicapped patients (0.57 +/- 0.21 microgram/ml) were lower than those in healthy children and adults (0.73 +/- 0.2 microgram/ml). CoQ levels in XP patients tended to decrease with age and were extremely low in two bed-ridden patients (0.29 and 0.21 microgram/ml), suggesting that CoQ levels decreased with disease progression. We studied the clinical effects of CoQ therapy in 19 XP patients with low CoQ levels. Eight patients showed increased activity in daily life. It suggests that XP patients have the disturbance of energy metabolism due to mitochondrial dysfunction. We consider it is worthwhile to research the mitocondrial function and the trial of CoQ therapy on XP patients.

Topics: Adolescent; Adult; Age Factors; Biomarkers; Child; Child, Preschool; Electron Transport; Energy Metabolism; Female; Humans; Male; Mitochondria; Ubiquinone; Xeroderma Pigmentosum