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Systematic evaluation of muscle coenzyme Q10 content in children with mitochondrial respiratory chain enzyme deficiencies.
Mitochondrion. 2008 Mar; 8(2):170-80.M

Abstract

Coenzyme Q10 content, pathology evaluation, and electron transport chain (ETC) enzyme analysis were determined in muscle biopsy specimens of 82 children with suspected mitochondrial myopathy. Data were stratified into three groups: "probable" ETC defects, "possible" ETC defects, and disease controls. Muscle total, oxidized, and reduced coenzyme Q10 concentrations were significantly decreased in the probable defect group. Stepwise logistic regression indicated that only total coenzyme Q10 was significantly associated with probable ETC defect. Receiver operator characteristic (ROC) analysis suggested that total muscle coenzyme Q10 was the best predictor of an ETC complex abnormality. Determination of muscle coenzyme Q10 deficiency in children with suspected mitochondrial disease may facilitate diagnosis and encourage earlier supplementation of this agent.

Authors+Show Affiliations

Department of Pediatrics, University of Cincinnati, College of Medicine, 3333 Burnet Avenue, Cincinnati, OH 45229-3039, USA. michael.miles@cchmc.org <michael.miles@cchmc.org>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

18313367

Citation

Miles, Michael V., et al. "Systematic Evaluation of Muscle Coenzyme Q10 Content in Children With Mitochondrial Respiratory Chain Enzyme Deficiencies." Mitochondrion, vol. 8, no. 2, 2008, pp. 170-80.
Miles MV, Miles L, Tang PH, et al. Systematic evaluation of muscle coenzyme Q10 content in children with mitochondrial respiratory chain enzyme deficiencies. Mitochondrion. 2008;8(2):170-80.
Miles, M. V., Miles, L., Tang, P. H., Horn, P. S., Steele, P. E., DeGrauw, A. J., Wong, B. L., & Bove, K. E. (2008). Systematic evaluation of muscle coenzyme Q10 content in children with mitochondrial respiratory chain enzyme deficiencies. Mitochondrion, 8(2), 170-80. https://doi.org/10.1016/j.mito.2008.01.003
Miles MV, et al. Systematic Evaluation of Muscle Coenzyme Q10 Content in Children With Mitochondrial Respiratory Chain Enzyme Deficiencies. Mitochondrion. 2008;8(2):170-80. PubMed PMID: 18313367.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Systematic evaluation of muscle coenzyme Q10 content in children with mitochondrial respiratory chain enzyme deficiencies. AU - Miles,Michael V, AU - Miles,Lili, AU - Tang,Peter H, AU - Horn,Paul S, AU - Steele,Paul E, AU - DeGrauw,Antonius J, AU - Wong,Brenda L, AU - Bove,Kevin E, Y1 - 2008/02/02/ PY - 2007/08/30/received PY - 2007/12/19/revised PY - 2008/01/18/accepted PY - 2008/3/4/pubmed PY - 2008/7/9/medline PY - 2008/3/4/entrez SP - 170 EP - 80 JF - Mitochondrion JO - Mitochondrion VL - 8 IS - 2 N2 - Coenzyme Q10 content, pathology evaluation, and electron transport chain (ETC) enzyme analysis were determined in muscle biopsy specimens of 82 children with suspected mitochondrial myopathy. Data were stratified into three groups: "probable" ETC defects, "possible" ETC defects, and disease controls. Muscle total, oxidized, and reduced coenzyme Q10 concentrations were significantly decreased in the probable defect group. Stepwise logistic regression indicated that only total coenzyme Q10 was significantly associated with probable ETC defect. Receiver operator characteristic (ROC) analysis suggested that total muscle coenzyme Q10 was the best predictor of an ETC complex abnormality. Determination of muscle coenzyme Q10 deficiency in children with suspected mitochondrial disease may facilitate diagnosis and encourage earlier supplementation of this agent. SN - 1567-7249 UR - https://www.unboundmedicine.com/medline/citation/18313367/Systematic_evaluation_of_muscle_coenzyme_Q10_content_in_children_with_mitochondrial_respiratory_chain_enzyme_deficiencies_ DB - PRIME DP - Unbound Medicine ER -