4346 T3D4292 Orotic acid Orotic acid is a minor dietary constituent. Indeed, until it was realized that it could be synthesized by humans, orotic acid was known as vitamin B-13. The richest dietary sources are cow's milk and other dairy products as well as root vegetables such as carrots and beets. Dietary intake probably contributes to a basal rate of orotic acid excretion in urine because fasting decreases excretion by ~50%. However, it is now apparent that most urinary orotic acid is synthesized in the body, where it arises as an intermediate in the pathway for the synthesis of pyrimidine nucleotides. Orotic acid is converted to UMP by UMP synthase, a multifunctional protein with both orotate phosphoribosyltransferase and orotidylate decarboxylase activity. The most frequently observed inborn error of pyrimidine nucleotide synthesis is a mutation of the multifunctional protein UMP synthase. This disorder prevents the conversion of orotic acid to UMP and thus to other pyrimidines. As a result, plasma orotic acid accumulates to high concentrations, and increased quantities appear in the urine. Indeed, urinary orotic acid is so markedly increased in individuals harboring a mutation in UMP synthase that orotic acid crystals can form in the urine. The urinary concentration of orotic acid in homozygotes can be of the order of millimoles per millimole creatinine. By comparison, the urinary level in unaffected individuals is ~ 1 umol/mmol creatinine. (A3380). 65-86-1 967 C5H4N2O4 156.1 White powder. 345.5°C 1.82 mg/mL at 18°C 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 orotic acid are associated with at least 4 inborn errors of metabolism including: Argininemia, Citrullinemia Type I, Purine nucleoside phosphorylase deficiency and Orotic Aciduria. 2014-08-29T06:16:18Z 2026-05-14T17:32:45Z Orotic acid C00295 16742 OROTATE DB02262 ORO true OC(=O)C1=CC(=O)NC(=O)N1 C5H4N2O4 InChI=1S/C5H4N2O4/c8-3-1-2(4(9)10)6-5(11)7-3/h1H,(H,9,10)(H2,6,7,8,11) PXQPEWDEAKTCGB-UHFFFAOYSA-N 156.0963 156.017106626 Endogenous Solid -0.83 HMDB00226 CHEMBL1235017 942 <p>Paul Rambacher, Siegfried Make, &#8220;Process for preparing orotic acid.&#8221; U.S. Patent US4062847, issued November, 1960.</p> CHEM003252 DTXSID0025814 NS00003862 2,6-dioxo-1,2,3,6-tetrahydropyrimidine-4-carboxylic acid 95.5 33.27460000000001 12.461507679606417 1 4 3 2.827266331449678 -6.031557162374663 -1 1 -0.89 -1.54 4.51e+00 g/l