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2-Methylsuccinic acid
本产品不向个人销售，仅用作科学研究，不用于任何人体实验及非科研性质的动物实验。
2-Methylsuccinic acid图片
CAS NO:498-21-5
包装与价格：
包装价格(元)
10mM (in 1mL DMSO)电议
1g电议

产品介绍
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 No.498-21-5
别名甲基丁二酸
Canonical SMILESO=C(O)C(C)CC(O)=O
分子式C5H8O4
分子量132.12
溶解度DMSO : ≥ 100 mg/mL (756.89 mM)
储存条件Store at -20°C
General tipsFor obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.
Shipping ConditionEvaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request
产品描述

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.