4458 Methylmalonic acid Methylmalonic acid (C4H6O4) is an endogenous human metabolite with an average molecular weight of 118.09 g/mol. At room temperature, this compound exists as a solid. Methylmalonic acid belongs to the dicarboxylic acids and derivatives, a subclass of carboxylic acids and derivatives within the organic compounds. In biological systems, the compound is detected in the liver and kidney tissues. It interacts with one recorded protein target, the methylmalonyl-CoA carboxyltransferase 12S subunit. The compound is found in or released from 14 recorded sources. These include household cleaning and consumer products such as soap detergents, bleaching agents, perfumes within detergents and soaps, and cigar cigarettes. It is also identified in food preservation and building and construction materials, specifically ladders. Additionally, it is associated with electrical and electronic equipment, including electrodes, antennas, coils, conductors or conductive bodies characterised by the conductive materials, and electrically stimulated smoking devices. 516-05-2 487 C4H6O4 118.09 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 2026-08-22T18:14:53Z 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 0.21 HMDB00202 CHEMBL1232416 473 CHEM003364 DTXSID00199549 NS00015152 2-methylpropanedioic acid 74.6 23.5623 10.057853065917067 2 4 2 2.4752703991264853 -2 0 0.17 0.10 1.49e+02 g/l Organic compounds Organic acids and derivatives Carboxylic acids and derivatives Dicarboxylic acids and derivatives 8784 CED0003358 ZIYVHBGGAOATLY-UHFFFAOYSA-N 40000000000000001000000000000000000000000000000000000000000000000000001000000000000000000000000004000000000000000000000000000000000800000000000000000000000000000020000000000000000000000000000000000000010100000000000000000000000000000000000000000000000000000000000040000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000800000000000000000400000000000000000000000000000000 20010006000800000000080000000000000004000000200040000000044000000000800000000001000080000004200084008c00010000442000a000008011000000000000000000200062010000000000400000220000002000000900042000000040000000000000403000002000801000056004110800000010000200440008040000000800020088000000100400024440100000040800000000000041000000004000000008000028001002c0000000000800000000000000000000010000040000000000000000010000000400000000400000000000080000000000080000000000000000000000000000000000004000000000000050000000 776t3gAAAAAQAAAAAwAAAAAAAAAIAAAABwAAAIABBgBgAAAAAQMGAGAAAAAD AQYAKAAAAAMECABoAAAAAwEBCAAoAAAAAwIGACgAAAADBAgAaAAAAAMBAQgA KAAAAAMCCwABAAECAAIDIAIEKAIBBQAFBiAFBygCQgAAAAAXBAAAAAAAAAAW 2026-09-19T04:01:16Z