Muscle coenzyme Q: a potential test for mitochondrial activity and redox status.
The aim of this study is to determine whether coenzyme Q (CoQ) muscle concentrations and redox state are associated with pathologic changes in muscle biopsy specimens. Skeletal muscle biopsies were collected (January 2002-February 2004) and underwent pathologic evaluation. Quadriceps specimens (n = 47) were stratified accordingly: Group 1, controls without evidence of pathologic abnormalities; Group 2, type I myofiber predominance; Group 3, type II myofiber atrophy; Group 4, lower motor unit disease; and Group 5, muscular dystrophy. Ubiquinol-10, ubiquinone-10, total coenzyme Q10 (CoQ10), coenzyme Q9 (CoQ9), total CoQ (CoQ9+CoQ10) concentrations were analyzed in biopsy muscle by high-performance liquid chromatography. Ubiquinone-10, total CoQ10, and total CoQ concentrations were significantly decreased in Group 5. Significant positive correlations (r congruent with 0.40) were found between muscle ubiquinone-10, total CoQ10, and total CoQ concentrations vs the percentage of myofibers having subsarcolemmal mitochondrial aggregates. CoQ redox ratio and the fraction CoQ9/total CoQ were negatively correlated with subsarcolemmal mitochondrial aggregates. A significant correlation (r = 0.328) also occurred between ubiquinol-10 concentration and citrate synthase activity. This study suggests that total CoQ concentration provides a new method for estimating mitochondrial activity in biopsy muscle; and that the muscle CoQ test is feasible and potentially useful for diagnosing CoQ deficiency states.
Miles
Lili
L
Division of Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229, USA.
Miles
Michael V
MV
Tang
Peter H
PH
Horn
Paul S
PS
Wong
Brenda L
BL
DeGrauw
Ton J
TJ
Morehart
Paula J
PJ
Bove
Kevin E
KE
D000293:Adolescent; D015415:Biomarkers; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008928:Mitochondria / Q000201:enzymology*; D018485:Muscle Fibers, Skeletal / Q000201:enzymology / Q000473:pathology; D018908:Muscle Weakness / Q000378:metabolism / Q000473:pathology; D018482:Muscle, Skeletal / Q000201:enzymology*; D009468:Neuromuscular Diseases / Q000378:metabolism* / Q000473:pathology*; D010084:Oxidation-Reduction; D014451:Ubiquinone / Q000172:deficiency* / Q000378:metabolism
D015415:Biomarkers; D014451:Ubiquinone