Observations of Altered Intracellular Phosphoribosylpyrophosphate (PP-Ribose-P) in Human Disease
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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.
KeywordsMethylene Blue Glycogen Storage Disease Orotic Acid Purine Biosynthesis Essential Substrate
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