Abstract
The juvenile type of neuronal ceroid lipofuscinosis (JNCL) is a recessively inherited, progressive neurodegenerative disease. In this study the levels of the antioxidant factors coenzyme Q 10 (CoQ 10) and vitamin E (alpha-tocopherol) were measured in plasma samples of 29 JNCL patients and compared to 48 healthy controls. A significant reduction of the coenzyme Q 10 level (0.59 ± 0.25 μg/ml) was observed in JNCL patients when compared to control subjects (0.80 ± 0.26 μg/ml). The level of vitamin E was also reduced markedly in JNCL patients when compared to controls (10.4 ± 4.1 and 12.1 ± 4.5 μg/ml, respectively). The low levels of CoQ 10 and vitamin E in JNCL patients may indicate an impaired antioxidant protection in this disease.
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