ubiquinone and Lewy-Body-Disease

Alpha-synuclein (alphaS) is the major component of the filamentous inclusions that constitute defining characteristics of Lewy body diseases (LBD) and multiple system atrophy (MSA). Clinically, antioxidant vitamins, such as vitamin E and the vitamin-like substance coenzyme Q10, have been used in the treatment of LBD with some efficacy. Using fluorescence spectroscopy with thioflavin S, electron microscopy and atomic force microscopy, here we examined the effects of ten antioxidant vitamins and vitamin-like substances, vitamin A (retinol, retinal and retinoic acid), beta-carotene, vitamins B2, B6, C, E, coenzyme Q10 and alpha-lipoic acid, on the formation of alphaS fibrils (falphaS) and on preformed falphaS. Among them, vitamin A, beta-carotene and coenzyme Q10 dose-dependently inhibited the formation of falphaS. Moreover, they also dose-dependently destabilized preformed falphaS. With such potent anti-fibrillogenic as well as fibril-destabilizing activities, these compounds could be useful in the treatment and prevention of LBD and MSA.

Topics: alpha-Synuclein; Antioxidants; Benzothiazoles; beta Carotene; Coenzymes; Humans; Lewy Body Disease; Microscopy, Atomic Force; Microscopy, Electron; Microscopy, Fluorescence; Neurofibrils; Neuroprotective Agents; Parkinson Disease; Thiazoles; Ubiquinone; Vitamin A; Vitamins

The purpose of the present study was to compare serum levels of coenzyme Q(10) (CoQ(10)) and the coenzyme Q(10) cholesterol (CoQ(10)/cholesterol) ratio in 18 patients with Lewy body disease (LBD) with 20 matched controls. The mean serum coenzyme Q10 levels in patients with LBD were significantly reduced with respect to control group, however, no differences were found in CoQ(10)/cholesterol ratio between LBD patients and control group. There was no correlation among CoQ(10) and CoQ(10)/cholesterol ratio with age, age of onset, body mass index, duration of the disease or scores of the Mini Mental State Examination, UPDRS and Hoehn and Yahr stage. These results suggest the involvement of this enzimatic system in the pathogenic mechanism of LBD.

Topics: Age Factors; Age of Onset; Aged; Apoptosis; Body Mass Index; Cholesterol; Coenzymes; Cytochrome c Group; Down-Regulation; Female; Humans; Lewy Body Disease; Male; Nerve Tissue Proteins; Neuropsychological Tests; Oxidative Stress; Synucleins; Ubiquinone