Chemicals and Drugs

Methylmalonic Acid

A malonic acid derivative which is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

A malonic acid derivative which is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA.

Chemical Identity

Chemical Information

Molecular Formula
C4H6O4
Molecular Weight
118.09 g/mol
CAS Registry No.
516-05-2
IUPAC Name
2-methylpropanedioic acid
InChIKey
ZIYVHBGGAOATLY-UHFFFAOYSA-N
SMILES
CC(C(=O)O)C(=O)O
MeSH Unique ID
D008764
Source
PubChem CID 487

MeSH Record

Classification

Broader headings

Related Concepts

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MeSH Record

Synonyms

1 entry terms
  • Acid, Methylmalonic

MeSH Record

Aspects Covered

25 allowable subheadings

Indexed with the subheadings administration & dosage, adverse effects, agonists, analogs & derivatives, analysis, antagonists & inhibitors, blood, cerebrospinal fluid, chemical synthesis, chemistry, classification, economics, history, immunology, isolation & purification, metabolism, pharmacokinetics, pharmacology, poisoning, radiation effects, standards, supply & distribution, therapeutic use, toxicity, urine.

MeSH Record

Indexing Annotation

/biosyn /physiol permitted

MeSH Record

History Note

91(75); was see under MALONATES 1975-90

MeSH Record

Previous Indexing

  • Malonates (1974)

MeSH Hierarchy

Tree Number

AMA Style

References

  1. National Library of Medicine. Methylmalonic Acid. Medical Subject Headings (MeSH). 2026. Unique ID D008764. http://id.nlm.nih.gov/mesh/2026/D008764
  2. Methylmalonic Acid. In: Wikipedia. https://en.wikipedia.org/wiki/Methylmalonic_acid
  3. Methylmalonic Acid. In: Wikidata. https://www.wikidata.org/wiki/Q239598
  4. Methylmalonic Acid. In: PubChem Compound. CID 487. https://pubchem.ncbi.nlm.nih.gov/compound/487