4497 Propionylcarnitine Propionylcarnitine is present in high abundance in the urine of patients with Methylmalonyl-CoA mutase (MUT) deficiency, (together with Methylmalonic acid). MUT is a mitochondrial enzyme that catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA. 17298-37-2 107738 C10H19NO4 White powder. 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. 2014-08-29T06:51:18Z 2026-04-03T00:20:24Z C03017 28867 true CCC(=O)OC(CC([O-])=O)C[N+](C)(C)C C10H19NO4 InChI=1S/C10H19NO4/c1-5-10(14)15-8(6-9(12)13)7-11(2,3)4/h8H,5-7H2,1-4H3 UFAHZIUFPNSHSL-UHFFFAOYSA-N 217.2622 217.131408101 Endogenous Solid HMDB00824 CHEMBL2074916 96904 CHEM003403 NS00015030 66.43 77.26370000000001 22.721274988631023 7 3 0 4.185139998188834 -7.054133284063387 0 0 -2.27 -3.25 1.52e-01 g/l