Bettendorff L, Goessens G, Sluse F E
Laboratory of Neurochemistry, University of Liège, Belgium.
Mol Cell Biochem. 1997 Sep;174(1-2):121-4.
Culture of neuroblastoma cells in the presence of low thiamine concentration (16 nM) and of the transport inhibitor amprolium leads to the appearance of signs of necrosis: the chromatin condenses, the oxygen consumption decreases and is uncoupled, the mitochondrial cristae are disorganized, the thiamine diphosphate-dependent dehydrogenase activities are impaired. When 10 microM thiamine are added to these cells, the basal respiration increases, the coupled respiration is restored and mitochondrial morphology is recovered within 1 h. Addition of succinate, which is oxidized via a thiamine diphosphate-independent dehydrogenase, to digitonin-permeabilized cells immediately restores a coupled respiration. Our results suggest that the slowing of the citric acid cycle is the cause of the biochemical lesion induced by severe thiamine deficiency and that part of the mitochondria remain functional.
在低硫胺素浓度(16 nM)和转运抑制剂氨丙啉存在的情况下培养神经母细胞瘤细胞,会出现坏死迹象:染色质浓缩,耗氧量减少且解偶联,线粒体嵴紊乱,硫胺素二磷酸依赖性脱氢酶活性受损。当向这些细胞中添加10 microM硫胺素时,基础呼吸增加,偶联呼吸恢复,线粒体形态在1小时内恢复。向经洋地黄皂苷通透处理的细胞中添加通过不依赖硫胺素二磷酸的脱氢酶氧化的琥珀酸,可立即恢复偶联呼吸。我们的结果表明,柠檬酸循环的减慢是严重硫胺素缺乏引起的生化损伤的原因,并且部分线粒体仍保持功能。