Metabolites

2-Methylsuccinic acid View larger

2-Methylsuccinic acid

AT5231

CAS 498-21-5

More details

Molarity Calculation Cart®

HOW TO ORDER

$25.00

$25.00 per g

Data sheet

Molecular FormulaC5H8O4
Molecular Weight132.115
CAS Numbers498-21-5
Storage Condition0C Short Term, -20C Long Term
SolubilityDMSO
Purity98% by HPLC
SMILES CodeCC(CC(O)=O)C(O)=O
ReferencesGrosso S, et al. Ethylmalonic encephalopathy further clinical and neuroradiological characterization. J Neurol. 2002 Oct;249[10] 1446-50.

More info

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.