[Ubiquinone: metabolism and functions. Ubiquinone deficiency and its implication in mitochondrial encephalopathies. Treatment with ubiquinone].
OBJECTIVE
Review of ubiquinone-10 metabolism and functions in humans, focusing its implication in the pathogenesis and physiopathology of mitochondrial encephalomyopathies.


METHODS
Ubiquinone-10 is an endogenously synthesized lipid with a wide distribution in tissues. Tyrosine and acetil-CoA are involved in ubiquinone biosynthesis. This molecule has several biological functions in cells: it is a movil electron carrier in the mitochondrial respiratory chain and also acts as antioxidant. Owing to its implication in these functions, ubiquinone deficiency may cause important deletereous effects in tissues. Several authors reported ubiquinone deficient status in some physiological and pathological conditions. Mitochondrial encephalomyopathies may be related to a primary or secondary ubiquinone deficient status, or even to an altered function of ubiquinone in the respiratory chain. Moreover, some relevant aspects about ubiquinone therapy in mitochondrial disorders are reported.


CONCLUSIONS
According to recent reports about ubiquinone implication in several diseases, its determination in different biological samples seems very useful to elucidate the physiopathological mechanisms involved and even the to start a therapy in cases with ubiquinone deficiency.
Artuch
R
R
Servicio de Bioquímica, Unitat Integrada Hospital Sant Joan de Déu-Hospital Clínic, Universitat de Barcelona, España. vilaseca@hsjdbcn.org
Colomé
C
C
Vilaseca
M A
MA
Pineda
M
M
Campistol
J
J
D000105:Acetyl Coenzyme A / Q000378:metabolism; D000975:Antioxidants / Q000378:metabolism; D002784:Cholesterol / Q000096:biosynthesis; D003067:Coenzymes; D004579:Electron Transport; D004734:Energy Metabolism; D006056:Golgi Apparatus / Q000378:metabolism; D006801:Humans; D008798:Mevalonic Acid / Q000378:metabolism; D008928:Mitochondria / Q000378:metabolism; D017237:Mitochondrial Encephalomyopathies / Q000188:drug therapy / Q000378:metabolism*; D018384:Oxidative Stress; D014443:Tyrosine / Q000378:metabolism; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000172:deficiency / Q000502:physiology / Q000627:therapeutic use
D000975:Antioxidants; D003067:Coenzymes; D014451:Ubiquinone; D014443:Tyrosine; D000105:Acetyl Coenzyme A; D002784:Cholesterol; C024989:coenzyme Q10; D008798:Mevalonic Acid