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Analysis of coenzyme Q10 in muscle and fibroblasts for the diagnosis of CoQ10 deficiency syndromes.
Clin Biochem. 2008 Jun; 41(9):697-700.CB

Abstract

OBJECTIVES

To study CoQ(10) concentrations in muscle and fibroblast from 6 patients with a CoQ(10) deficiency syndrome.

DESIGN AND METHODS

CoQ(10) was quantified by HPLC with electrochemical detection.

RESULTS

Four out of the 6 cases showed muscle CoQ(10) deficiency plus a reduction of mitochondrial respiratory chain enzyme activities. All cases showed decreased CoQ(10) values in fibroblasts when compared with controls.

CONCLUSIONS

Biochemical study of CoQ(10) in both muscle and fibroblasts seems advisable to demonstrate the deficiency in all patients.

Links

Publisher Full Text (DOI)

Authors+Show Affiliations

Montero R
Department of Clinical Biochemistry, Hospital Sant Joan de Déu and Centre for Biomedical Research on Rare Diseases (CIBERER), Instituto de Salud Carlos III. Barcelona, Spain.
Sánchez-Alcázar JA
No affiliation info available
Briones P
No affiliation info available
Hernández AR
No affiliation info available
Cordero MD
No affiliation info available
Trevisson E
No affiliation info available
Salviati L
No affiliation info available
Pineda M
No affiliation info available
García-Cazorla A
No affiliation info available
Navas P
No affiliation info available
Artuch R
No affiliation info available

MeSH

AdolescentAdultBiomarkersChildChild, PreschoolElectron TransportFibroblastsHumansInfantInfant, NewbornMiddle AgedMitochondria, MuscleMuscle, SkeletalSyndromeUbiquinone

Pub Type(s)

Comparative Study
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

18387363

Citation

Montero, Raquel, et al. "Analysis of Coenzyme Q10 in Muscle and Fibroblasts for the Diagnosis of CoQ10 Deficiency Syndromes." Clinical Biochemistry, vol. 41, no. 9, 2008, pp. 697-700.
Montero R, Sánchez-Alcázar JA, Briones P, et al. Analysis of coenzyme Q10 in muscle and fibroblasts for the diagnosis of CoQ10 deficiency syndromes. Clin Biochem. 2008;41(9):697-700.
Montero, R., Sánchez-Alcázar, J. A., Briones, P., Hernández, A. R., Cordero, M. D., Trevisson, E., Salviati, L., Pineda, M., García-Cazorla, A., Navas, P., & Artuch, R. (2008). Analysis of coenzyme Q10 in muscle and fibroblasts for the diagnosis of CoQ10 deficiency syndromes. Clinical Biochemistry, 41(9), 697-700. https://doi.org/10.1016/j.clinbiochem.2008.03.007
Montero R, et al. Analysis of Coenzyme Q10 in Muscle and Fibroblasts for the Diagnosis of CoQ10 Deficiency Syndromes. Clin Biochem. 2008;41(9):697-700. PubMed PMID: 18387363.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Analysis of coenzyme Q10 in muscle and fibroblasts for the diagnosis of CoQ10 deficiency syndromes. AU - Montero,Raquel, AU - Sánchez-Alcázar,José Antonio, AU - Briones,Paz, AU - Hernández,Angeles Rodríguez, AU - Cordero,Mario D, AU - Trevisson,Eva, AU - Salviati,Leonardo, AU - Pineda,Mercé, AU - García-Cazorla,Angels, AU - Navas,Plácido, AU - Artuch,Rafael, Y1 - 2008/03/20/ PY - 2007/10/25/received PY - 2008/02/12/revised PY - 2008/03/08/accepted PY - 2008/4/5/pubmed PY - 2008/10/11/medline PY - 2008/4/5/entrez SP - 697 EP - 700 JF - Clinical biochemistry JO - Clin Biochem VL - 41 IS - 9 N2 - OBJECTIVES: To study CoQ(10) concentrations in muscle and fibroblast from 6 patients with a CoQ(10) deficiency syndrome. DESIGN AND METHODS: CoQ(10) was quantified by HPLC with electrochemical detection. RESULTS: Four out of the 6 cases showed muscle CoQ(10) deficiency plus a reduction of mitochondrial respiratory chain enzyme activities. All cases showed decreased CoQ(10) values in fibroblasts when compared with controls. CONCLUSIONS: Biochemical study of CoQ(10) in both muscle and fibroblasts seems advisable to demonstrate the deficiency in all patients. SN - 1873-2933 UR - https://www.unboundmedicine.com/medline/citation/18387363/Analysis_of_coenzyme_Q10_in_muscle_and_fibroblasts_for_the_diagnosis_of_CoQ10_deficiency_syndromes_ DB - PRIME DP - Unbound Medicine ER -