青少年帕金森病患者单核血细胞中的GTP环化水解酶I活性

GTP cyclohydrolase I activity in mononuclear blood cells in juvenile parkinsonism.

作者信息

Ichinose H, Ohye T, Yokochi M, Fujita K, Nagatsu T

机构信息

Institute for Comprehensive Medical Science, School of Medicine, Fujita Health University, Aichi, Japan.

出版信息

Neurosci Lett. 1995 May 5;190(2):140-2. doi: 10.1016/0304-3940(95)11511-t.

DOI:10.1016/0304-3940(95)11511-t

PMID:7644124

Abstract

GTP cyclohydrolase I activity in mononuclear blood cells from patients with juvenile parkinsonism (JP) was found to be normal compared to healthy controls. The normal activity in JP contrasts strongly with the decreased activity of 2-20% normal levels in hereditary progressive dystonia with marked diurnal fluctuation (HPD) or dopa responsive dystonia (DRD). The result indicates that the decreased dopamine level in the basal ganglia in JP is not due to decreased activity of GTP cyclohydrolase I, the enzyme for the biosynthesis of the tetrahydrobiopterin cofactor of tyrosine hydroxylase (TH), and the enzyme activity in mononuclear blood cells could be a reliable method for differential diagnosis between JP and HPD/DRD.

摘要

与健康对照组相比，青少年帕金森病（JP）患者单核血细胞中的鸟苷三磷酸环化水解酶I活性被发现是正常的。JP患者的正常活性与遗传性进行性肌张力障碍伴明显昼夜波动（HPD）或多巴反应性肌张力障碍（DRD）中2%-20%正常水平的活性降低形成强烈对比。结果表明，JP患者基底节中多巴胺水平降低并非由于鸟苷三磷酸环化水解酶I活性降低，该酶是酪氨酸羟化酶（TH）四氢生物蝶呤辅因子生物合成的酶，单核血细胞中的酶活性可能是JP与HPD/DRD鉴别诊断的可靠方法。