Muscle coenzyme Q10 in mitochondrial encephalomyopathies

T Matsuoka, H Maeda, Y Goto, I Nonaka - Neuromuscular disorders, 1991 - Elsevier
Coenzyme Q 10 (CoQ) content was measured in isolated muscle mitochondria from 25
patients with mitochondrial encephalomyopathies (MEM), most of whom had mitochondrial
DNA mutations. The CoQ level was significantly lower in MEM patients than in controls. CoQ
levels varied widely from patient to patient, especially in those with chronic progressive
external ophthalmoplegia including Kearns-Sayre syndrome, which may explain, at least in
part, the variable response of patients to CoQ administration.