Formulation: A solid
Formal Name: 2-methyl-butanedioic acid
Purity: ≥95%
Formula Markup: C5H8O4
Formula Weight: 132,1
Shelf life (days): 1460
CAS Number: 498-21-5
Notes: Methylsuccinic acid is a dicarboxylic acid and metabolite of the essential amino acid L-isoleucine and branched-chain amino acid L-alloisoleucine (Item No. 34904).{67310} It is formed from L-isoleucine and L-alloisoleucine via an R-2-oxo-3-methylvaleric acid intermediate. Urinary levels of methylsuccinic acid are increased in patients with ethylmalonic encephalopathy, an inborn error of metabolism characterized by developmental delay, hypotonia, vascular instability, petechiae, acrocyanosis, chronic diarrhea, and lactic acidemia. Urinary levels of methylsuccinic acid are also increased in patients with short-chain acyl-CoA dehydrogenase deficiency or type 2 diabetes.{67311,67312} It has been found in urban and industrial aerosols of fine particulate matter less than 2.5 µm (PM2.5).{67313}
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