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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.

| Pack Size | Price | Availability | Quantity |
|---|---|---|---|
| 1 g | $29 | In Stock | |
| 1 mL x 10 mM (in DMSO) | $29 | In Stock |
| Description | 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. |
| Synonyms | Pyrotartaric Acid, Methylbutanedioic acid |
| Molecular Weight | 132.11 |
| Formula | C5H8O4 |
| Cas No. | 498-21-5 |
| Smiles | C(CC(O)=O)(C(O)=O)C |
| Relative Density. | 1.4105 g/cm3 |
| Color | White |
| Appearance | Solid |
| Storage | keep away from moisture | Powder: -20°C for 3 years | In solvent: -80°C for 1 year | Shipping with blue ice/Shipping at ambient temperature. | |||||||||||||||||||||||||||||||||||
| Solubility Information | DMSO: 55 mg/mL (416.3 mM), Sonication is recommended. | |||||||||||||||||||||||||||||||||||
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DMSO
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