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

  • I. H. Fox
  • W. N. Kelley
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 41 B)

Abstract

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.

Keywords

Methylene Blue Glycogen Storage Disease Orotic Acid Purine Biosynthesis Essential Substrate 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

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Copyright information

© Springer Science+Business Media New York 1974

Authors and Affiliations

  • I. H. Fox
    • 1
  • W. N. Kelley
    • 1
  1. 1.University of Toronto, Canada and Duke University Medical CenterDurhamUSA

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