Orotic acid

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Orotic acid
2D structure for Orotic acid
Chemical Name	2,6-dioxo-3H-pyrimidine-4-carboxylic acid
Chemical Formula	C₅H₄N₂O₄
CAS Number	65-86-1
Chemical Information	HMDB00226
Biochemical Taxonomy	Keto-Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Pyrimidine Metabolism
Biofluid Location	Blood Urine
Tissue Location	Pancreas Liver
Normal Biofluid Concentrations	Blood: 5.0 +/- 0.4 uM Urine: 0.0007+/-0.0005 umol/mmol creatinine Urine: 0.0011+/-0.0008 umol/mmol creatinine Urine: 0.0013+/-0.0008 umol/mmol creatinine Urine: 0.0015+/-0.0012 umol/mmol creatinine Urine: 1.05 +/- 1.22 umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Patient with Canavan disease
Other (Monogenic Disorders)	Lysinuric protein intolerance OMIM: 222700 N-acetylglutamate synthase deficiency OMIM: 237310
Abnormal Biofluid Concentrations	Blood (Patient with Canavan disease): 0.94 +/- 0.78 umol/L
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
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. (PMID: 17513443)