Purine Nucleoside Phosphorylase Deficiency
Metabolites
1-(5-Phospho-D-ribosyl)-5-amino-4-imidazolecarboxylate
Formula: C9H14N3O9P (339.04676440000003)
CAS ID: 6001-14-5
1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole
Formula: C13H19N4O12P (454.07370640000005)
CAS ID: 3031-95-6
1-(5'-Phosphoribosyl)-5-amino-4-imidazolecarboxamide
Formula: C9H15N4O8P (338.062748)
CAS ID: 3031-94-5
1-(5'-Phosphoribosyl)-5-formamido-4-imidazolecarboxamide
Formula: C10H15N4O9P (366.057663)
CAS ID: 13018-54-7
Enzyme
| EC Number | name | full name | note |
|---|---|---|---|
| 2.7.-.- | |||
| 1.17.1.4 | xanthine dehydrogenase | xanthine:NAD+ oxidoreductase | |
| 1.17.3.2 | xanthine oxidase | xanthine:oxygen oxidoreductase | |
| 1.17.4.1 | ribonucleoside-diphosphate reductase | 2'-deoxyribonucleoside-5'-diphosphate:thioredoxin-disulfide 2'-oxidoreductase | |
| 2.1.2.2 | phosphoribosylglycinamide formyltransferase 1 | 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide N-formyltransferase | |
| 2.1.2.3 | phosphoribosylaminoimidazolecarboxamide formyltransferase | 10-formyltetrahydrofolate:5'-phosphoribosyl-5-amino-4-imidazole-carboxamide N-formyltransferase | |
| 2.4.2.14 | amidophosphoribosyltransferase | 5-phospho-beta-D-ribosylamine:diphosphate phospho-alpha-D-ribosyltransferase (glutamate-amidating) | |
| 3.1.4.35 | 3',5'-cyclic-GMP phosphodiesterase | 3',5'-cyclic-GMP 5'-nucleotidohydrolase | |
| 3.1.4.53 | 3',5'-cyclic-AMP phosphodiesterase | 3',5'-cyclic-AMP 5'-nucleotidohydrolase | |
| 3.5.4.10 | IMP cyclohydrolase | IMP 1,2-hydrolase (decyclizing) | |
| 4.1.1.21 | phosphoribosylaminoimidazole carboxylase | 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxylate carboxy-lyase [5-amino-1-(5-phospho-D-ribosyl)imidazole-forming] | |
| 4.3.2.2 | adenylosuccinate lyase | N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming) | |
| 6.3.2.6 | phosphoribosylaminoimidazolesuccinocarboxamide synthase | 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxylate:L-aspartate ligase (ADP-forming) | |
| 6.3.3.1 | phosphoribosylformylglycinamidine cyclo-ligase | 2-(Formamido)-N1-(5-phosphoribosyl)acetamidine cyclo-ligase (ADP-forming) | |
| 6.3.4.13 | phosphoribosylamine-glycine ligase | 5-phospho-D-ribosylamine:glycine ligase (ADP-forming) | |
| 6.3.4.4 | adenylosuccinate synthase | IMP:L-aspartate ligase (GDP-forming) | |
| 6.3.5.2 | GMP synthase (glutamine-hydrolysing) | xanthosine-5'-phosphate:L-glutamine amido-ligase (AMP-forming) | |
| 6.3.5.3 | phosphoribosylformylglycinamidine synthase | N2-formyl-N1-(5-phospho-D-ribosyl)glycinamide:L-glutamine amido-ligase (ADP-forming) |
Proteins
| Protein ID | name | full name |
|---|