Abstract
PRPP is a substrate for the enzyme glutamine-PRPP amidotransferase catalyzing the first rate-limiting step of purine nucleotide synthesis de novo (1). Evidence has been obtained that PRPP is an important regulator of this pathway (2–4). A possible role of increased PRPP availability in the enhancement of de novo purine synthesis in primary metabolic gout, a purine overproduction disease, has been suggested by the demonstration of an increased PRPP turnover in gouty subjects (5) and of an increased rate of PRPP formation in erythrocytes and cultured fibroblasts from such patients (6–8).
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© 1974 Plenum Press, New York
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Sperling, O., Persky-Brosh, S., Boer, P., de Vries, A. (1974). Mutant Phosphoribosylpyrophosphate Synthetase in Two Gouty Siblings with Excessive Purine Production. In: Sperling, O., De Vries, A., Wyngaarden, J.B. (eds) Purine Metabolism in Man. Advances in Experimental Medicine and Biology, vol 41A. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-3294-7_36
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DOI: https://doi.org/10.1007/978-1-4684-3294-7_36
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