4458 Methylmalonic acid Methylmalonic acid is 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. 516-05-2 487 C4H6O4 White powder. 135°C 679.0 mg/mL Methylmalonic acid 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. Accumulation of Methylmalonic acid in the body has been shown to be toxic. No indication of carcinogenicity to humans (not listed by IARC). This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. Chronically high levels of methylmalonic acid are associated with at least 5 inborn errors of metabolism including: Malonyl CoA decarboxylase deficiency, Malonic Aciduria, Methylmalonate Semialdehyde Dehydrogenase Deficiency, Methylmalonic Aciduria and Methylmalonic Aciduria Due to Cobalamin-Related Disorders. 2014-08-29T06:51:02Z 2026-04-03T00:38:26Z Methylmalonic acid C02170 30860 DB04183 DXX true CC(C(O)=O)C(O)=O C4H6O4 InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8) ZIYVHBGGAOATLY-UHFFFAOYSA-N 118.088 118.02660868 Endogenous Solid HMDB00202 CHEMBL1232416 473 CHEM003364 DTXSID00199549 NS00015152 2-methylpropanedioic acid