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Muscle coenzyme Q: a potential test for mitochondrial activity and redox status.
Pediatr Neurol. 2005 May; 32(5):318-24.PN

Abstract

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.

Authors+Show Affiliations

Division of Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

15866432

Citation

Miles, Lili, et al. "Muscle Coenzyme Q: a Potential Test for Mitochondrial Activity and Redox Status." Pediatric Neurology, vol. 32, no. 5, 2005, pp. 318-24.
Miles L, Miles MV, Tang PH, et al. Muscle coenzyme Q: a potential test for mitochondrial activity and redox status. Pediatr Neurol. 2005;32(5):318-24.
Miles, L., Miles, M. V., Tang, P. H., Horn, P. S., Wong, B. L., DeGrauw, T. J., Morehart, P. J., & Bove, K. E. (2005). Muscle coenzyme Q: a potential test for mitochondrial activity and redox status. Pediatric Neurology, 32(5), 318-24.
Miles L, et al. Muscle Coenzyme Q: a Potential Test for Mitochondrial Activity and Redox Status. Pediatr Neurol. 2005;32(5):318-24. PubMed PMID: 15866432.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Muscle coenzyme Q: a potential test for mitochondrial activity and redox status. AU - Miles,Lili, AU - Miles,Michael V, AU - Tang,Peter H, AU - Horn,Paul S, AU - Wong,Brenda L, AU - DeGrauw,Ton J, AU - Morehart,Paula J, AU - Bove,Kevin E, PY - 2004/09/20/received PY - 2004/12/16/revised PY - 2005/01/17/accepted PY - 2005/5/4/pubmed PY - 2005/7/9/medline PY - 2005/5/4/entrez SP - 318 EP - 24 JF - Pediatric neurology JO - Pediatr Neurol VL - 32 IS - 5 N2 - 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. SN - 0887-8994 UR - https://www.unboundmedicine.com/medline/citation/15866432/Muscle_coenzyme_Q:_a_potential_test_for_mitochondrial_activity_and_redox_status_ DB - PRIME DP - Unbound Medicine ER -