CAS NO: | 498-21-5 |
包装: | 1g |
规格: | 98% |
市场价: | 445元 |
分子量: | 132.12 |
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.