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。
产品描述
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.
Cas No.
498-21-5
分子式
C5H8O4
分子量
132.115
别名
Pyrotartaric Acid;甲基丁二酸;Methylbutanedioic acid;2-Methylsuccinic acid
储存和溶解度
DMSO:200 mM
Powder: -20°C for 3 years
In solvent: -80°C for 2 years