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Metabolism and function of coenzyme Q.
This review summarizes the findings available to day concerning CoQ distribution, biosynthesis, regulatory modifications and its participation in cellular metabolism. Expand
Biochemical, physiological and medical aspects of ubiquinone function.
This presentation is a brief review of current knowledge concerning some biochemical, physiological and medical aspects of the function of ubiquinone (coenzyme Q) in mammalian organisms. In additionExpand
Coenzyme Q--biosynthesis and functions.
One approach for this purpose is administration of epoxidated all-trans polyisoprenoids, which enhance both CoQ biosynthesis and levels in experimental systems. Expand
Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders.
2 novel inborn errors of CoQ10 biosynthesis in 2 distinct families are identified, indicating that these mutations are indeed the cause of OXPHOS deficiency. Expand
Fatty acid composition of brain phospholipids in aging and in Alzheimer’s disease
The findings do not support the hypothesis that AD reflects an accelerated aging process, and changes in saturated/polyunsaturated FA ratio are likely to influence cellular function, which in turn may cause certain neural deficiencies. Expand
Decrease and structural modifications of phosphatidylethanolamine plasmalogen in the brain with Alzheimer disease.
Both the vinyl ether bond of phosphatidylethanolamine plasmalogen and polyunsaturated fatty acids are major targets in oxidative stress; thus, these specific lipid modifications strongly support the involvement of free radicals in the pathogenesis of AD. Expand
The antioxidant role of coenzyme Q.
CoQ biosynthesis is influenced by nuclear receptors which may give the possibility, in the future, by using agonists or antagonists, of reestablishing the normal level in deficiencies caused by genetic mutations, aging or cardiomyopathy. Expand
Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency
Undetectable coenzyme Q10 and results of radiolabelling experiments in cultured fibroblasts supported the diagnosis of widespread coen enzyme Q10 deficiency, and particular attention should be paid to multiple quinone-responsive respiratory-chain enzyme deficiency, which can be successfully treated by oral ubidecarenone. Expand
Distribution and redox state of ubiquinones in rat and human tissues.
It was found in model experiments that in postmortem tissue neither oxidation nor reduction of ubiquinone occurs, and high levels of reduction could be observed in human tissues, with the exception of brain and lung. Expand