2008年9月23日 · In male probands with the mild phenotype, detection of high activity or lack of allosteric regulation of the PRS-I enzyme (PRS-I enzyme assay) establishes the diagnosis. …

5-磷酸核糖-1α-焦磷酸（Phosphoribosyl pyrophosphate，缩写PRPP）是一种核糖衍生物。 它是核糖C1的活化形式，由核糖-5-磷酸与ATP在核糖磷酸焦磷酸激酶催化下生成。

Phosphoribosyl diphosphate (PRPP) is an important intermediate in cellular metabolism. PRPP is synthesized by PRPP synthase, as follows: ribose 5-phosphate + ATP → PRPP + AMP. …

5-Phosphoribosyl-1-pyrophosphate (PRPP) is a key intermediate in nucleotide biosynthesis. It is required for de novo synthesis of purine and pyrimidine nucleotides and the salvage pathways, …

The disease is due to overactivity of ribose-phosphate pyrophosphokinase 1 (PRS-I), an enzyme that catalyzes the synthesis of PRPP, a cofactor involved in the synthesis of purine and …

PRPP is synthesized from MgATP and ribose-5-P in an allosterically regulated reaction catalyzed by independently active and structurally homologous PRS isoforms encoded by separable …

Phosphoribosylpyrophosphate(PRPP) synthetase(PRS) catalyzes the formation of PRPP from ATP and ribose-5-phosphate. PRPP is an important substrate for the synthesis of purine, …

2008年10月21日 · Phosphoribosylpyrophosphate synthetase (PRS) deficiency, an X-linked disorder, is a phenotypic continuum comprising three disorders previously thought to be …

Phosphoribosylpyrophosphate (PRPP) synthetase (PRS; EC 2.7.6.1) superactivity (also called Phosphoribosyl Pyrophosphate Synthetase – PRS– overactivity) is an X chromosome-linked …

The operation of additional determinants of rates of PRPP synthesis in human cells is suggested by: (1) multiple PRS isoforms with distinctive physical and kinetic properties; (2) nearly …