2-Methylsuccinic acid 是乙基丙二酸脑病的主要生化指标，是人体液中的正常代谢物。

Methylsuccinic acid is a normal metabolite found in human fluids. Increased urinary levels of Methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy, a rare metabolic disorder with an autosomal recessive mode of inheritance that is clinically characterized by neuromotor delay, hyperlactic acidemia, recurrent petechiae, orthostatic acrocyanosis, and chronic diarrhea.

498-21-5

C5H8O4

132.115

Pyrotartaric Acid;甲基丁二酸;Methylbutanedioic acid;2-Methylsuccinic acid

DMSO:200 mM
Powder: -20°C for 3 years
In solvent: -80°C for 2 years