Invertebrate models for coenzyme q10 deficiency.

ABSTRACT: The human syndrome of coenzyme Q (CoQ) deficiency is a heterogeneous mitochondrial disease characterized by a diminution of CoQ content in cells and tissues that affects all the electron transport processes CoQ is responsible for, like the electron transference in mitochondria for respiration and ATP production and the antioxidant capacity that it exerts in membranes and lipoproteins. Supplementation with external CoQ is the main attempt to address these pathologies, but quite variable results have been obtained ranging from little response to a dramatic recovery. Here, we present the importance of modeling human CoQ deficiencies in animal models to understand the genetics and the pathology of this disease, although the election of an organism is crucial and can sometimes be controversial.

SUBMITTER: Fernandez-Ayala DJ 

PROVIDER: S-EPMC4112529 | biostudies-literature | 2014 Jul

REPOSITORIES: biostudies-literature