Muscle Coenzyme Q: A Potential Test for Mitochondrial Activity and Redox Status

Miles, Lili; Miles, Michael V.; Tang, Peter H.; Horn, Paul S.; Wong, Brenda L.; DeGrauw, Ton J.; Morehart, Paula J.; Bove, Kevin E.

doi:10.1016/j.pediatrneurol.2005.01.009

« Previous Next »Pediatric Neurology
Volume 32, Issue 5 , Pages 318-324, May 2005

Muscle Coenzyme Q: A Potential Test for Mitochondrial Activity and Redox Status

Lili Miles, MD
- Division of Pathology and Laboratory Medicine, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
- Communications should be addressed to: Dr. Miles; Division of Pathology and Laboratory Medicine; Cincinnati Children’s Hospital Medical Center; 3333 Burnet Ave.; Cincinnati, OH 45229.
Michael V. Miles, PharmD
- Division of Pathology and Laboratory Medicine, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
- Division of Child Neurology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
Peter H. Tang, PhD
- Division of Pathology and Laboratory Medicine, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
- Division of Child Neurology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
Paul S. Horn, PhD
- Department of Mathematical Sciences, University of Cincinnati, Psychiatry Service, Veteran’s Affairs Medical Center, Cincinnati, Ohio.
Brenda L. Wong, MD
- Division of Child Neurology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
Ton J. DeGrauw, MD
- Division of Child Neurology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
Paula J. Morehart, RN
- Division of Child Neurology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
Kevin E. Bove, MD
- Division of Pathology and Laboratory Medicine, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio

Received 20 September 2004; accepted 17 January 2005.

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 ≅ 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.