Muscle coenzyme Q10 in mitochondrial encephalomyopathies.
Coenzyme Q10 (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.
Matsuoka
T
T
Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan.
Maeda
H
H
Goto
Y
Y
Nonaka
I
I
D000293:Adolescent; D000328:Adult; D002648:Child; D003067:Coenzymes; D004272:DNA, Mitochondrial / Q000235:genetics*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000737:chemistry*; D009135:Muscular Diseases / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D009154:Mutation; D009468:Neuromuscular Diseases / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000032:analysis
D003067:Coenzymes; D004272:DNA, Mitochondrial; D014451:Ubiquinone; C024989:coenzyme Q10