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Record Information
Version1.0
StatusDetected and Quantified
Creation Date2020-12-10 18:36:45 UTC
Update Date2024-04-30 19:32:06 UTC
Metabolite IDMMDBc0000392
Metabolite Identification
Common NameMethylsuccinic acid
DescriptionMethylsuccinic acid (CAS: 498-21-5) is a normal metabolite found in human fluids. Increased urinary levels of methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy (OMIM: 602473 ), 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 (PMID:12382164 ). The underlying biochemical defect involves isoleucine catabolism (PMID:9667231 ). Methylsuccinic acid levels were found to have decreased in the urine of animals under D-serine-induced nephrotoxicity (D-serine causes selective necrosis of the proximal straight tubules in the rat kidney) (PMID:15596249 ). Moreover, methylsuccinic acid is found to be associated with ethylmalonic encephalopathy, isovaleric acidemia, and medium-chain acyl-CoA dehydrogenase deficiency, which are also inborn errors of metabolism.
Structure
Synonyms
Molecular FormulaC5H8O4
Average Mass132.115
Monoisotopic Mass132.042258738
IUPAC Name(2S)-2-methylbutanedioic acid
Traditional Name(2S)-2-methylbutanedioic acid
CAS Registry NumberNot Available
SMILES
C[C@@H](CC(O)=O)C(O)=O
InChI Identifier
InChI=1S/C5H8O4/c1-3(5(8)9)2-4(6)7/h3H,2H2,1H3,(H,6,7)(H,8,9)/t3-/m0/s1
InChI KeyWXUAQHNMJWJLTG-VKHMYHEASA-N