Munujos P, Coll-Cantí J, Beleta J, González-Sastre F, Gella F J
Department of Biochemistry and Molecular Biology, Universitat Autònoma de Barcelona, Hospital de Sant Pau de Barcelona, Catalonia, Spain.
Clin Chim Acta. 1996 Nov 15;255(1):13-25. doi: 10.1016/0009-8981(96)06385-1.
Pyrithiamine-induced thiamin deficiency has been used in rat as an experimental form of Wernicke-Korsakoff encephalopathy, a disease associated with chronic alcoholism. Although the main etiological factor is known to be the lack of thiamin, the biochemical mechanisms involved in the pathogenesis remain unclear. Thiamin-dependent enzymes were studied in brain mitochondria: alpha-ketoglutarate dehydrogenase activity exhibited 40% reduction, whereas pyruvate dehydrogenase did not change significantly. Polarographic recordings of mitochondrial respiration revealed a decreased State 3, when using pyruvate/malate, alpha-ketoglutarate or glutamine as a substrate, but the respiration rates remained unchanged with glutamate or succinate. This fall in pyruvate oxidation may be due to the impairment of alpha-ketoglutarate dehydrogenase, which follows pyruvate dehydrogenase in the metabolic pathway. A time course of lactate concentration showed dramatic increases in thalamus, mid brain, hypothalamus and colliculli, consistent with the anatomopathological findings. No increases were found before the onset of neurological symptoms.
吡硫胺诱导的硫胺素缺乏已被用于大鼠,作为韦尼克-科尔萨科夫脑病的一种实验形式,该疾病与慢性酒精中毒有关。尽管已知主要病因是硫胺素缺乏,但发病机制中涉及的生化机制仍不清楚。对脑线粒体中的硫胺素依赖性酶进行了研究:α-酮戊二酸脱氢酶活性降低了40%,而丙酮酸脱氢酶没有明显变化。线粒体呼吸的极谱记录显示,当使用丙酮酸/苹果酸、α-酮戊二酸或谷氨酰胺作为底物时,状态3呼吸速率降低,但使用谷氨酸或琥珀酸时呼吸速率保持不变。丙酮酸氧化的下降可能是由于α-酮戊二酸脱氢酶受损,该酶在代谢途径中位于丙酮酸脱氢酶之后。乳酸浓度的时间进程显示,丘脑、中脑、下丘脑和小脑的乳酸浓度显著增加,这与解剖病理学发现一致。在神经症状出现之前没有发现增加。