Butterworth R F, Kril J J, Harper C G
Neuroscience Research Unit, Hôpital Saint-Luc (University of Montreal), Quebec, Canada.
Alcohol Clin Exp Res. 1993 Oct;17(5):1084-8. doi: 10.1111/j.1530-0277.1993.tb05668.x.
Chronic alcoholism results in thiamine deficiency as a consequence of poor nutrition, impaired absorption, and decreased phosphorylation to the enzyme cofactor form of the vitamin, thiamine pyrophosphate (TPP). Results of this study demonstrate significant reductions of TPP-dependent enzymes [pyruvate dehydrogenase complex, alpha-ketoglutarate dehydrogenase (alpha KGDH), and transketolase] in autopsied cerebellar vermis samples from alcoholic patients with the clinical and neuropathologically confirmed diagnosis of Wernicke-Korsakoff Syndrome (WKS). Enzyme activities in brain samples from alcoholics without WKS were within normal limits and activities of a nonthiamine-dependent enzyme, glutamate dehydrogenase, were not significantly different from control values in brain samples from alcoholics with or without WKS. These findings provide evidence, for the first time, of a direct implication of TPP-related metabolic processes in the pathogenesis of WKS. Decreased activities of alpha KGDH could be the trigger for a sequence of metabolic events resulting in energy compromise, and ultimately neuronal death in this syndrome.
慢性酒精中毒会导致硫胺素缺乏,这是营养状况差、吸收受损以及该维生素转化为酶辅因子形式(硫胺素焦磷酸,TPP)的磷酸化作用降低的结果。本研究结果表明,在临床和神经病理学确诊为韦尼克-科尔萨科夫综合征(WKS)的酒精性患者尸检小脑蚓部样本中,TPP依赖性酶[丙酮酸脱氢酶复合体、α-酮戊二酸脱氢酶(αKGDH)和转酮醇酶]显著减少。无WKS的酒精性患者脑样本中的酶活性在正常范围内,且一种非硫胺素依赖性酶谷氨酸脱氢酶的活性在有或无WKS的酒精性患者脑样本中与对照值无显著差异。这些发现首次为TPP相关代谢过程直接参与WKS发病机制提供了证据。αKGDH活性降低可能是一系列代谢事件的触发因素,导致能量供应不足,最终造成该综合征中的神经元死亡。