Observations of Altered Intracellular Phosphoribosylpyrophosphate (PP-Ribose-P) in Human Disease

Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 41)


An intricate system of interrelated control mechanisms regulate biochemical reaction sequences. Metabolic pathways are controlled not only by specific activity and inherent kinetic properties of enzymes in the pathway but also by the intracellular concentration of certain essential substrates, activators or inhibitors. PP-ribose-P is an essential substrate of purine, pyrimidine and pyridine biosynthesis. The intracellular concentration of PP-ribose-P represents a balance between its synthesis by PP-ribose-P synthetase and its utilization which is catalyzed by several different phosphoribosyltransferase (PRT) enzymes as well as non-specific phosphatases. Alterations in the rate of synthesis or degradation of PP-ribose-P, whether drug induced or secondary to an inborn error, could potentially change the intracellular concentration of this compound.


Methylene Blue Glycogen Storage Disease Orotic Acid Purine Biosynthesis Essential Substrate 
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  1. Fox, I. H. and Kelley, W. N. 1971. Phophoribosylpyrophosphate in man: Biochemical and clinical signficiance. 74: 424–433.Google Scholar
  2. Greene, M. L. and Seegmiller, J. E. 1969. Erythrocyte 5-phosphoribosyl-1-pyrophosphate (PRPP) in gout: Importance of PRPP in the regulation of human purine synthesis (Abstract). Arth. Rheum. 12: 666–667.Google Scholar
  3. Holmes, E. W., McDonald, J. A., McCord, J. M., Wyngaarden, J. B. and Kelley, W. N. 1973. Human glutamine phosphoribosylpyrophosphate amidotransferase. Kinetic and regulatory properties. J. Biol. Chem. 248: 144–150.PubMedGoogle Scholar
  4. Kelley, W. N., Fox, I. H. and Wyngaarden, J. B. 1970. Regulation of purine biosynthesis in cultured human cells. I. Effects of orotic acid. Biochim. Biophys. Acta. 215: 512–516.CrossRefGoogle Scholar
  5. Kelley, W. N., Greene, M. L., Fox, I. H., Rosenbloom, F. M., Levy, R. I. and Seegmiller, J. E. 1970. Effect of orotic acid on purine and lipoprotein metabolism in man. Metabolism 19: 1025–1035.CrossRefGoogle Scholar
  6. Rosenbloom, F. M., Henderson, J. F., Caldwell, I. C., Kelley, W. N. and Seegmiller, J. E. 1968. Biochemical basis of accelerated purine biosynthesis de novo in human fibroblasts lacking hypoxanthine-guanine phosphoribosyltransferase. J. Biol. Chem. 243: 1166–1173.PubMedGoogle Scholar
  7. Valentine, W. N., Anderson, H. M., Paglia, D. E., Jaffe, E. R., Konrad, P. N. and Harris, S. R. 1972. Studies on human erythrocyte nucleotide metabolism. II. Nonspherocytic hemolytic anemia, high red cell ATP and ribosephosphate phosphoribokinase (Rpk, E.C. deficiency. Blood. 39: 674–684.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1974

Authors and Affiliations

  1. 1.University of Toronto, Canada and Duke University Medical CenterDurhamUSA

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