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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 |
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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. |
Alias | Pyrotartaric Acid, Methylbutanedioic acid |
Molecular Weight | 132.115 |
Formula | C5H8O4 |
Cas No. | 498-21-5 |
Smiles | CC(CC(O)=O)C(O)=O |
Relative Density. | 1.4105 g/cm3 |
Storage | Powder: -20°C for 3 years | In solvent: -80°C for 1 year | Shipping with blue ice. | |||||||||||||||||||||||||||||||||||
Solubility Information | DMSO: 55 mg/mL (416.3 mM) | |||||||||||||||||||||||||||||||||||
Solution Preparation Table | ||||||||||||||||||||||||||||||||||||
DMSO
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