Methylmalonic Acid

DB04183ExperimentalSmall molecule

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... This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA.

Formula / weight: C₄H₆O₄ · 118.088 g/mol (avg)
Code names: NSC-25201

Resolves to

Compound

UNII8LL8S712J7CAS516-05-2ChEMBLCHEMBL1232416ChEBI30860PubChem487KEGGC02170

Structure

InChIKeyZIYVHBGGAOATLY-UHFFFAOYSA-NSMILESCC(C(O)=O)C(O)=O

Targets

Q8GBW6Methylmalonyl-CoA carboxyltransferase 12S subunit

What you can answer from here — as of September 16, 2026

1Protein targetEach mapped to UniProt, with action and pharmacological action

Listed above

2+ReferencesStructured and connected to the statements they support

Which drugs share a target with Methylmalonic Acid, and which of those have an active Phase 3 trial?

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