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Overview
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.
Chemical Properties
| Molecular Formula | C5H8O4 |
| Molecular Weight | 132.115 |
| CAS Numbers | 498-21-5 |
| Solubility | DMSO |
| Purity | 98% by HPLC |
| SMILES Code | CC(CC(O)=O)C(O)=O |
Storage and Handling
0C Short Term, -20C Long Term
References
Grosso S, et al. Ethylmalonic encephalopathy further clinical and neuroradiological characterization. J Neurol. 2002 Oct;249[10] 1446-50.