2.4.2.1 1-methyladenosine + phosphate - 2.4.2.1 1-methylguanosine + phosphate - 2.4.2.1 2'-deoxyguanosine + phosphate - 2.4.2.1 2-fluoro-2-deoxyadenosine + phosphate a toxic prodrug 2.4.2.1 5'-deoxy-5'-methylthioadenosine + phosphate - 2.4.2.1 5'-methylthioinosine + phosphate - 2.4.2.1 6-mercaptopurine-2'-deoxyriboside + phosphate - 2.4.2.1 7-methylguanine + alpha-D-ribose 1-phosphate - 2.4.2.1 7-methylguanosine + phosphate - 2.4.2.1 7-methylguanosine + phosphate low activity 2.4.2.1 8-azaguanine + alpha-D-ribose 1-phosphate - 2.4.2.1 8-azaguanosine + phosphate - 2.4.2.1 9-beta-D-arabinosyl-2-fluoroadenine + phosphate i.e. fludarabine 2.4.2.1 9-[2-deoxy-beta-D-ribofuranosyl]-6-methylpurine + phosphate a toxic prodrug 2.4.2.1 9-[6-deoxy-beta-L-talofuranosyl]-2-fluoro-adenine + phosphate a toxic prodrug, substrate of enzyme mutant M64V, the drug product has anti-tumor activity 2.4.2.1 9-[6-deoxy-beta-L-talofuranosyl]-6-methylpurine + phosphate a toxic prodrug, substrate of enzyme mutant M64V, the drug product has anti-tumor activity 2.4.2.1 9-[beta-D-arabinofuranosyl]-2-fluoro-adenine + phosphate a toxic prodrug 2.4.2.1 9-[beta-L-lyxofuranosyl]-2-fluoro-adenine + phosphate a toxic prodrug, substrate of enzyme mutant M64V, the drug product has anti-tumor activity 2.4.2.1 adenosine + phosphate - 2.4.2.1 adenosine + phosphate very low activity 2.4.2.1 adenosine + phosphate low activity 2.4.2.1 guanine + alpha-D-ribose 1-phosphate - 2.4.2.1 guanosine + phosphate - 2.4.2.1 inosine + phosphate - 2.4.2.1 inosine + phosphate complete conversion 2.4.2.1 additional information - 2.4.2.1 additional information in fish skin the enzyme plays a key role in the deposition of guanine and hypoxanthine crystals 2.4.2.1 additional information cellular function seems concerned primarily with nucleoside breakdown 2.4.2.1 additional information children lacking PNP activity exhibit severe T cell immunodeficiency while maintaining normal or exaggerated B cell function. The PNP deficiency results in very low uric acid concentrations and high concentrations of the nucleoside substrate of PNP in plasma and urine 2.4.2.1 additional information in intact cells, the enzyme functions in the direction of phosphorolysis, leading to degradation of purine nucleosides via coupling with guanase and xanthine oxidase. PNP deficiency is a rare disorder associated with an autosomal recessive form of cellular, but not humoral, immunodeficiency, and comprises about 4% of all cases of severe combined immunodeficiency. Mutations identified in cases of PNP deficiency 2.4.2.1 additional information key enzyme in purine salvage pathway 2.4.2.1 additional information enzyme plays a key role in the purine salvage pathway. PNP deficiency in humans leads to an impairment of T-cell function, usually with no apparent effects on B-cell function 2.4.2.1 additional information the enzyme is specific for adenine nucleosides which includes adenosine analogs modified in the aglycone, pentose or both moieties 2.4.2.1 additional information in cultured cells (CFs, GMCs, PGVSMCs), isolated perfused kidneys, and in vivo, N6-etheno-adenosine is converted to N6-etheno-adenine. This reaction is abolished by 8-aminoguanine. When kidneys are not inserted into the perfusion system, N6-etheno-ATP is not metabolized. The conversions are inhibited by the PNPase inhibitor forodesine 2.4.2.1 nicotinamide riboside + phosphate - 2.4.2.1 purine deoxyribonucleoside + phosphate - 2.4.2.1 purine ribonucleoside + phosphate - 2.4.2.1 xanthosine + phosphate -