Krentz A J, Williams A C, Nattrass M
Diabetic Clinic, General Hospital, Birmingham, UK.
Diabetes Res. 1991 Feb;16(2):93-9.
In this study, circulating concentrations of intermediary metabolites were measured in eight non-obese subjects with motor neurone disease in the basal (postabsorptive) state, and after a 75 g oral glucose challenge. Eight healthy subjects of similar age and body mass index served as controls. Basal pyruvate concentration was significantly elevated in the subjects with motor neurone disease (p less than 0.02). After oral glucose ingestion, overall levels of pyruvate (p less than 0.01) and lactate (p less than 0.05) were significantly higher in these subjects. Blood glucose concentrations fulfilled the criteria diagnostic of impaired glucose tolerance in six of the eight subjects with motor neurone disease (WHO, 1985). Cumulative insulin levels were slightly higher in these subjects and peak insulin response was delayed (120 min vs. 60 min) relative to the healthy controls. Circulating concentrations of alanine, glycerol, non-esterified fatty acids and total ketone bodies were similar between groups. These results confirm that impaired glucose tolerance is a common feature of motor neurone disease. Furthermore, our data indicate disordered regulation of both pyruvate and lactate metabolism, consistent with reports of defective skeletal muscle pyruvate oxidation in individuals with this disorder. In contrast, our results indicate that the regulation of lipolysis and ketone body metabolism is unimpaired in motor neurone disease.
在本研究中，我们测量了8名非肥胖运动神经元病患者在基础（空腹）状态下以及口服75 g葡萄糖后的中间代谢产物循环浓度。8名年龄和体重指数相近的健康受试者作为对照。运动神经元病患者的基础丙酮酸浓度显著升高（p<0.02）。口服葡萄糖后，这些患者的丙酮酸（p<0.01）和乳酸（p<0.05）总体水平显著更高。8名运动神经元病患者中有6人的血糖浓度符合葡萄糖耐量受损的诊断标准（世界卫生组织，1985年）。这些患者的累积胰岛素水平略高，且相对于健康对照，胰岛素反应峰值延迟（120分钟对60分钟）。两组之间丙氨酸、甘油、非酯化脂肪酸和总酮体的循环浓度相似。这些结果证实葡萄糖耐量受损是运动神经元病的一个常见特征。此外，我们的数据表明丙酮酸和乳酸代谢调节紊乱，这与该疾病患者骨骼肌丙酮酸氧化缺陷的报道一致。相比之下，我们的结果表明运动神经元病患者的脂肪分解和酮体代谢调节未受损。
