Abstract
Orotoc acid (OA) an intermediary metabolite of the pyrimidine synthesis pathway is formed from carbamylphosphate (CP) and aspartate (Fig. 1). In the liver these precursors are synthetised mainly in the mitochondrium1,2,3 . Natale and Tremblay4 showed that CP formed by carbamylphosphate synthetase (CPS I) in the mitochondria crosses the mitochondrial membrane into the cytosol where the condensation with aspartate occurs. In addition especially in extrahepatic, fetal or regenerating tissues a cytosolic glutamine dependant carbamylphosphate synthetase (CPS II) exists5,6. Tremblay et al. showed that the synthesis of CP in the mitochondria accounts for a major part of the CP utilized for pyrimidine synthesis by rat liver slices.
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References
G. C. Tremblay, D. E. Crandall, C. E. Knott and M. Alfant, Arch. Biochem. Biophys. 178: 264 (1977).
A. Barton and N. J. Hoogenraad, Eur. J. Biochem. 116: 131 (1981).
A. J. Meijer, J. A. Gimpel, G. Deleeuw, M. E. Tischler, J. Tager and J. R. Williamson, J. Biol. Chem. 253: 2308 (1978).
P. J. Natale and G. C. Tremblay, Biochem. Biophys. Res. Comm. 37: 512 (1969).
M. Mori, H. Ishida and M. Tatibana, Biochemistry 14: 2622 (1975).
M. Tatibana and K. Ito, J. Biol. Chem. 244: 5403 (1969).
A. Russell, B. Levin, V. G. Oberholzer and L. Sinclair, Lancet II: 699 (1962).
L. Kesner, F. L. Aronson, M. Silverman and P. C. Chan, Clin. Chem. 21: 353 (1975).
C. Bachmann and J. P. Colombo, J. Clin. Chem. Clin. Biochem. 18: 293 (1980).
A. Van Gennep, E. J. Van Bree-Blom, J. Grift, P. K. De Brie and S. K. Wadman, Clin. Chim. Acta 104: 227 (1980).
F. X. Coudé, H. Ogier, C. Marsac, A. Munnich, C. Charpentier and J. M. Sandubray, this volume.
K. Shigesada and M. Tatibana, Biochem. Biophys. Res. Comm. 44: 1117 (1971).
F. X. Coudé, L. Sweetman and W. L. Nyhan, J. Clin. Invest. 64: 1544 (1979).
C. Bachmann, Urea Cycle in “Heritable Disorders of Amino Acid Metabolism”, W.L. Nyhan, ed. Wiley, New York (1974)
D. M. Stewart and M. Walser, J Clin. Invest. 66: 484 (1980)
L. Cathelineau, F. P. Petit, F. Coudé and P. P. Kamoun, Biochem. Biophys. Res. Comm. 90: 327 (1979).
J. M. Saudubray, L. Cathelineau, J. M. Laugier, C. Charpentier, J. A. Lejeune and P. Mozziconacci, Acta Paed. Scand. 64: 464 (1975).
S. Arashima and L. Matsuda, Tohoku,J. Exp. Med. 107: 143 (1972).
S. D. Cederbaum, K. N. F. Shaw and M. Valente, Am. J. Hum. Genet. 25: 20a (1973).
A. S. Hassan and J. A. Milner, Arch. Biochem. Biophys. 194: 24 (1979).
J. P. Colombo and C. Bachmann, Enzyme 25: 297 (1980).
H. A. Simmonds, D. R. Webster, D. M. 0. Becroft and C. F. Polter, Eur. J. Clin. Invest. 10: 333 (1980).
C. Bachmann and J. P. Colombo, Eur. J. Pediatr. 134: 109 (1980).
D. R. Webster, H. A. Simmonds, D. M. J. Barry and D. M. 0. Becroft, J. Inherit. Metab. Dis. 4: 27 (1981).
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Bachmann, C., Colombo, J.P. (1982). Orotic Acid in Urine and Hyperammonemia. In: Lowenthal, A., Mori, A., Marescau, B. (eds) Urea Cycle Diseases. Advances in Experimental Medicine and Biology, vol 153. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-6903-6_38
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DOI: https://doi.org/10.1007/978-1-4757-6903-6_38
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