Poulton K R, Rossi M L
Department of Neuropathology, Midland Centre for Neurosurgery and Neurology, Smethwick, West Midlands, UK.
Funct Neurol. 1993 Jan-Feb;8(1):33-42.
Conversion of glucose to fructose via sorbitol depends upon the enzymes aldose reductase and sorbitol dehydrogenase and is called the polyol pathway. It is particularly active in muscle from patients with X-linked muscular dystrophies (15). This investigation shows enhanced metabolism of glucose to fructose in muscle from patients with ALS. Evidence is also presented showing increased activities of ketohexokinase and F-1-P splitting aldolase, which suggests that further metabolism of fructose may occur via a fructolytic pathway. Investigation of protein glycation, by an adapted fructosamine assay, in post mortem muscle, sural nerve and blood indicates that there is an increased concentration of glucose in muscle and nerve in the period prior to sampling, but blood glucose concentrations were within normal limits. The implications of fructolysis and the relationship of altered glucose metabolism in ALS are discussed.
葡萄糖通过山梨醇转化为果糖依赖于醛糖还原酶和山梨醇脱氢酶，这一过程称为多元醇途径。它在患有X连锁肌营养不良症患者的肌肉中特别活跃（15）。本研究表明，肌萎缩侧索硬化症（ALS）患者肌肉中葡萄糖向果糖的代谢增强。同时也有证据表明，酮己糖激酶和F-1-P裂解醛缩酶的活性增加，这表明果糖可能通过糖酵解途径进一步代谢。通过改良的果糖胺测定法对死后肌肉、腓肠神经和血液中的蛋白质糖基化进行研究表明，在采样前肌肉和神经中的葡萄糖浓度升高，但血糖浓度在正常范围内。本文讨论了糖酵解的意义以及ALS中葡萄糖代谢改变的关系。
