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Pathway Description
Metabolism and Physiological Effects of Orotic acid
Homo sapiens
Metabolic Pathway
Orotic acid (orotate) is classified as a pyrimidinemonocarboxylic acid. Most urinary orotic acid is synthesized in the body, where it arises as an intermediate in the pathway for the synthesis of pyrimidine nucleotides. It originates from l-glutamine, which is obtained from protein sources such as red meat and eggs in the diet. L-glutamine is metabolized to orotate in the liver. L-glutamine is first converted to carbamoyl phosphate then to N-Carbamoyl-L-aspartate and finally to dihydroorotate by the CAD protein (carbamoyl-phosphate synthetase 2, aspartate transcarbamylase, and dihydroorotase). Dihydroorotate is converted to orotate in the mitochondria of the cell via the enzyme dihydroorotate dehydrogenase. Orotate can enter the bloodstream where it exerts detrimental effects on other systems. A build up of orotate in the body leads to acidosis which can have detrimental effects on other systems in the body causing renal failure, neurotoxicity, endothelial dysfunction and hypertension.
References
Metabolism and Physiological Effects of Orotic acid References
Choi YJ, Yoon Y, Lee KY, Kang YP, Lim DK, Kwon SW, Kang KW, Lee SM, Lee BH: Orotic acid induces hypertension associated with impaired endothelial nitric oxide synthesis. Toxicol Sci. 2015 Apr;144(2):307-17. doi: 10.1093/toxsci/kfv003. Epub 2015 Jan 19.
Pubmed: 25601987
Fonteh, Aliah L. (2018) "Orotic Aciduria," Fidei et Veritatis: The Liberty University Journal of Graduate Research: Vol. 2 : Iss. 1 , Article 1.
Available at: https://digitalcommons.liberty.edu/fidei_et_veritatis/vol2/iss1/1
Kanehisa M, Furumichi M, Sato Y, Ishiguro-Watanabe M, Tanabe M: KEGG: integrating viruses and cellular organisms. Nucleic Acids Res. 2021 Jan 8;49(D1):D545-D551. doi: 10.1093/nar/gkaa970.
Wishart DS, Feunang YD, Marcu A, Guo AC, Liang K, Vazquez-Fresno R, Sajed T, Johnson D, Li C, Karu N, Sayeeda Z, Lo E, Assempour N, Berjanskii M, Singhal S, Arndt D, Liang Y, Badran H, Grant J, Serra-Cayuela A, Liu Y, Mandal R, Neveu V, Pon A, Knox C, Wilson M, Manach C, Scalbert A: HMDB 4.0: the human metabolome database for 2018. Nucleic Acids Res. 2018 Jan 4;46(D1):D608-D617. doi: 10.1093/nar/gkx1089.
Pubmed: 29140435
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