CEREBELLAR ALPHA-KETOGLUTARATE DEHYDROGENASE-ACTIVITY IS REDUCED IN SPINOCEREBELLAR ATAXIA TYPE-1

被引：13

作者：

MASTROGIACOMO, F ^{[1
]}

KISH, SJ ^{[1
]}

机构：

[1] CLARKE INST PSYCHIAT, HUMAN NEUROCHEM PATHOL LAB, TORONTO M5T 1R8, ON, CANADA

来源：

ANNALS OF NEUROLOGY | 1994年 / 35卷 / 05期

关键词：

D O I：

10.1002/ana.410350519

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

We measured the activity of the thiamine pyrophosphate-dependent enzyme alpha-ketoglutarate dehydrogenase complex in postmortem brain of 12 patients with the spinocerebellar ataxia type 1 form of olivopontocerebellar atrophy. alpha-Ketoglutarate dehydrogenase complex activity measured in the absence of thiamine pyrophosphate was markedly reduced (-72%) in olivopontocerebellar atrophy cerebellar cortex. Decreased activity of this key rate-limiting Krebs cycle enzyme could compromise cerebellar energy metabolism and excitatory amino acid synthesis and thereby contribute to the brain dysfunction of olivopontocerebellar atrophy.

引用

页码：624 / 626

页数：3

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