2-Methylsuccinic acid - CAS 498-21-5

2-Methylsuccinic acid (Pyrotartaric Acid) is a normal metabolite found in human fluids. Increased urinary levels of 2-Methylsuccinic acid (Pyrotartaric 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.

Endogenous Metabolite

Metabolism

472

0.9931

C5H8O4

132.11

C(CC(O)=O)(C(O)=O)C

-20°C

Ice Packs

Natural Product