Mastrogiacomo F, Kish S J
Human Neurochemical Pathology Laboratory, Clarke Institute of Psychiatry, Toronto, Ontario, Canada.
Ann Neurol. 1994 May;35(5):624-6. doi: 10.1002/ana.410350519.
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.
我们测定了12例患有橄榄体脑桥小脑萎缩1型脊髓小脑共济失调患者死后大脑中硫胺素焦磷酸依赖性酶α-酮戊二酸脱氢酶复合体的活性。在缺乏硫胺素焦磷酸的情况下测得的α-酮戊二酸脱氢酶复合体活性在橄榄体脑桥小脑萎缩的小脑皮质中显著降低（-72%）。这种关键的三羧酸循环限速酶活性降低可能会损害小脑能量代谢和兴奋性氨基酸合成，从而导致橄榄体脑桥小脑萎缩的脑功能障碍。
