title: Pediatric reference intervals for muscle coenzyme Q(10).
abstract: Coenzyme Q(10) (CoQ(10)) is present in humans in both the reduced (ubiquinol, CoQ(10)H(2)) and oxidized (ubiquinone, CoQ(10)) forms. CoQ(10) is an essential cofactor in mitochondrial oxidative phosphorylation, and is necessary for ATP production. Total, reduced and oxidized CoQ(10) levels in skeletal muscle of 148 children were determined by HPLC coupled with electrochemical detection, and we established three level thresholds for total CoQ(10) in muscle. We defined as "severe deficiency", CoQ(10) levels falling in the range between 0.82 and 4.88 μmol/g tissue; as "intermediate deficiency", those ranging between 5.40 and 9.80 μmol/g tissue, and as "mild deficiency", the amount of CoQ(10) included between 10.21 and 19.10 μmol/g tissue. Early identification of CoQ(10) deficiency has important implications in children, not only for those with primary CoQ(10) defect, but also for patients with neurodegenerative disorders, in order to encourage earlier supplementation with this agent also in mild and intermediate deficiency.
authors.affiliation: Laboratory of Metabolomics and Proteomics, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy. anna.pastore@opbg.net
authors.forename: Anna
authors.initials: A
authors.lastname: Pastore
authors.forename: Gianna
authors.initials: G
authors.lastname: Di Giovamberardino
authors.forename: Sara
authors.initials: S
authors.lastname: Petrillo
authors.forename: Sara
authors.initials: S
authors.lastname: Boenzi
authors.forename: Enrico
authors.initials: E
authors.lastname: Bertini
authors.forename: Carlo
authors.initials: C
authors.lastname: Dionisi-Vici
authors.forename: Fiorella
authors.initials: F
authors.lastname: Piemonte
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D002851:Chromatography, High Pressure Liquid; D055664:Electrochemical Techniques; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D018482:Muscle, Skeletal / Q000378:metabolism*; D019636:Neurodegenerative Diseases / Q000175:diagnosis / Q000378:metabolism; D010084:Oxidation-Reduction; D012016:Reference Values; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000172:deficiency / Q000378:metabolism
chemical_list: D015415:Biomarkers; D014451:Ubiquinone; C024989:coenzyme Q10