Pediatric reference intervals for muscle coenzyme Q10

Anna Pastore, Gianna Di Giovamberardino, Sara Petrillo, Sara Boenzi, Enrico Bertini, Carlo Dionisi-Vici, Fiorella Piemonte

Research output: Contribution to journalArticlepeer-review


Coenzyme Q10 (CoQ10) is present in humans in both the reduced (ubiquinol, CoQ10H2) and oxidized (ubiquinone, CoQ10) forms. CoQ10 is an essential cofactor in mitochondrial oxidative phosphorylation, and is necessary for ATP production. Total, reduced and oxidized CoQ10 levels in skeletal muscle of 148 children were determined by HPLC coupled with electrochemical detection, and we established three level thresholds for total CoQ10 in muscle. We defined as "severe deficiency", CoQ10 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 CoQ10 included between 10.21 and 19.10 μmol/g tissue. Early identification of CoQ10 deficiency has important implications in children, not only for those with primary CoQ10 defect, but also for patients with neurodegenerative disorders, in order to encourage earlier supplementation with this agent also in mild and intermediate deficiency.

Original languageEnglish
Pages (from-to)764-766
Number of pages3
Issue number8
Publication statusPublished - Dec 2012


  • Cut-off values
  • Neurological disease

ASJC Scopus subject areas

  • Biochemistry
  • Clinical Biochemistry
  • Health, Toxicology and Mutagenesis


Dive into the research topics of 'Pediatric reference intervals for muscle coenzyme Q10'. Together they form a unique fingerprint.

Cite this