辅酶Q10和维生素E在青少年神经元蜡样脂褐质沉积症（JNCL）中可能作用的评估。

Evaluation of the possible role of coenzyme Q10 and vitamin E in juvenile neuronal ceroid-lipofuscinosis (JNCL).

作者信息

Westermarck T, Aberg L, Santavuori P, Antila E, Edlund P, Atroshi F

机构信息

Children's Hospital, University of Helsinki, Finland.

出版信息

Mol Aspects Med. 1997;18 Suppl:S259-62. doi: 10.1016/s0098-2997(97)00025-3.

DOI:10.1016/s0098-2997(97)00025-3

PMID:9266533

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 Q10 (CoQ10) 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 Q10 level (0.59 +/- 0.25 microgram/ml) was observed in JNCL patients when compared to control subjects (0.80 +/- 0.26 microgram/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 micrograms/ml, respectively). The low levels of CoQ10 and vitamin E in JNCL patients may indicate an impaired antioxidant protection in this disease.

摘要

青少年型神经元蜡样脂褐质沉积症（JNCL）是一种隐性遗传的进行性神经退行性疾病。在本研究中，对29例JNCL患者的血浆样本中抗氧化因子辅酶Q10（CoQ10）和维生素E（α-生育酚）的水平进行了测定，并与48名健康对照者进行了比较。与对照受试者（0.80±0.26微克/毫升）相比，JNCL患者的辅酶Q10水平显著降低（0.59±0.25微克/毫升）。与对照组相比，JNCL患者的维生素E水平也明显降低（分别为10.4±4.1和12.1±4.5微克/毫升）。JNCL患者中CoQ10和维生素E水平较低可能表明该疾病中抗氧化保护受损。