The Value of Coenzyme Q10Determination in Mitochondrial Patients.
Coenzyme Q10(CoQ) is a lipid that is ubiquitously synthesized in tissues and has a key role in mitochondrial oxidative phosphorylation. Its biochemical determination provides insight into the CoQ status of tissues and may detect CoQ deficiency that can result from either an inherited primary deficiency of CoQ metabolism or may be secondary to different genetic and environmental conditions. Rapid identification of CoQ deficiency can also allow potentially beneficial treatment to be initiated as early as possible. CoQ may be measured in different specimens, including plasma, blood mononuclear cells, platelets, urine, muscle, and cultured skin fibroblasts. Blood and urinary CoQ also have good utility for CoQ treatment monitoring.
Clinical Biochemistry and Molecular Medicine Department, Institut de Recerca Sant Joan de Déu and CIBERER-ISCIII, Passeig Sant Joan de Déu, 2, 08950 Esplugues, Barcelona, Spain. dyubero@hsjdbcn.org.
Delia
D
Yubero
Department of Blood Sciences, Royal Devon and Exeter NHS Foundation Trust, Exeter EX2 5DW, UK. george.allen@nhs.net.
George
G
Allen
Clinical Biochemistry and Molecular Medicine Department, Institut de Recerca Sant Joan de Déu and CIBERER-ISCIII, Passeig Sant Joan de Déu, 2, 08950 Esplugues, Barcelona, Spain. rartuch@hsjdbcn.org.
Rafael
R
Artuch
Clinical Biochemistry and Molecular Medicine Department, Institut de Recerca Sant Joan de Déu and CIBERER-ISCIII, Passeig Sant Joan de Déu, 2, 08950 Esplugues, Barcelona, Spain. rmontero@hsjdbcn.org.
Raquel
R
Montero
coenzyme Q10 deficiency; mitochondrial diseases; treatment monitoring