2-Methylsuccinicacid是人体液中的一种正常代谢物，是乙基丙二酸脑病的主要生化指标。
CAS：498-21-5
分子式：C5H8O4
分子量：132.12
纯度：98%
存储：Store at -20°C

Background:

2-Methylsuccinic acid is a normal metabolite in human fluids and the main biochemical measurable features in ethylmalonic encephalopathy.

2-Methylsuccinic acid is a normal metabolite in human fluids and the main biochemical measurable features in ethylmalonic encephalopathy[1]. The underlying biochemical defect involves isoleucine catabolism[2].

[1]. Grosso S, et al. Ethylmalonic encephalopathy: further clinical and neuroradiological characterization. J Neurol. 2002 Oct;249(10):1446-50. [2]. Nowaczyk MJ, et al. Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism. Metabolism. 1998 Jul;47(7):836-9.