Page last updated: 2024-10-06

Xanthinuria Type II

Proteins (37)

ProteinSynonymsTaxonomy
Adenylosuccinate synthetase isozyme 2AMPSase 2; AdSS 2; EC 6.3.4.4; Adenylosuccinate synthetase, acidic isozyme; Adenylosuccinate synthetase, liver isozyme; L-type adenylosuccinate synthetase; IMP--aspartate ligase 2Homo sapiens (human)
cAMP-specific 3',5'-cyclic phosphodiesterase 4DEC 3.1.4.53; DPDE3; PDE43Homo sapiens (human)
Multifunctional protein ADE2Homo sapiens (human)
GMP synthase [glutamine-hydrolyzing] EC 6.3.5.2; GMP synthetase; Glutamine amidotransferaseHomo sapiens (human)
Hypoxanthine-guanine phosphoribosyltransferaseHGPRT; HGPRTase; EC 2.4.2.8Homo sapiens (human)
Xanthine dehydrogenase/oxidaseHomo sapiens (human)
cAMP and cAMP-inhibited cGMP 3',5'-cyclic phosphodiesterase 10AEC 3.1.4.17; EC 3.1.4.35Homo sapiens (human)
Phosphoribosylformylglycinamidine synthaseFGAM synthase; FGAMS; EC 6.3.5.3; Formylglycinamide ribonucleotide amidotransferase; FGAR amidotransferase; FGAR-AT; Formylglycinamide ribotide amidotransferaseHomo sapiens (human)
Cytosolic purine 5'-nucleotidaseEC 3.1.3.5; Cytosolic 5'-nucleotidase IIHomo sapiens (human)
AmidophosphoribosyltransferaseATase; EC 2.4.2.14; Glutamine phosphoribosylpyrophosphate amidotransferase; GPATHomo sapiens (human)
Bis(5'-nucleosyl)-tetraphosphatase [asymmetrical]EC 3.6.1.17; Diadenosine 5',5'''-P1,P4-tetraphosphate asymmetrical hydrolase; Ap4A hydrolase; Ap4Aase; Diadenosine tetraphosphatase; Nucleoside diphosphate-linked moiety X motif 2; Nudix motif 2Homo sapiens (human)
Guanylate kinaseEC 2.7.4.8; GMP kinase; Guanylate kinase 1Homo sapiens (human)
Inosine-5'-monophosphate dehydrogenase 1 IMP dehydrogenase 1; IMPD 1; IMPDH 1; EC 1.1.1.205; IMPDH-IHomo sapiens (human)
Ribonucleoside-diphosphate reductase large subunitEC 1.17.4.1; Ribonucleoside-diphosphate reductase subunit M1; Ribonucleotide reductase large subunitHomo sapiens (human)
Ribonucleoside-diphosphate reductase subunit M2EC 1.17.4.1; Ribonucleotide reductase small chain; Ribonucleotide reductase small subunitHomo sapiens (human)
Bifunctional purine biosynthesis protein ATICAICAR transformylase/inosine monophosphate cyclohydrolase; ATICHomo sapiens (human)
Adenylate cyclase type 2EC 4.6.1.1; ATP pyrophosphate-lyase 2; Adenylate cyclase type II; Adenylyl cyclase 2Homo sapiens (human)
Deoxyguanosine kinase, mitochondrialEC 2.7.1.113; Deoxyadenosine kinase, mitochondrial; 2.7.1.76Homo sapiens (human)
Ectonucleoside triphosphate diphosphohydrolase 8E-NTPDase 8; NTPDase 8; NTPDase8; EC 3.6.1.5Homo sapiens (human)
Guanine deaminaseGuanase; Guanine aminase; EC 3.5.4.3; Guanine aminohydrolase; GAH; p51-nedasinHomo sapiens (human)
Ectonucleoside triphosphate diphosphohydrolase 5NTPDase 5; EC 3.6.1.6; CD39 antigen-like 4; ER-UDPase; Guanosine-diphosphatase ENTPD5; GDPase ENTPD5; 3.6.1.42; Nucleoside diphosphatase; Uridine-diphosphatase ENTPD5; UDPase ENTPD5Homo sapiens (human)
Purine nucleoside phosphorylasePNP; EC 2.4.2.1; Inosine phosphorylase; Inosine-guanosine phosphorylaseHomo sapiens (human)
Adenosine deaminaseEC 3.5.4.4; Adenosine aminohydrolaseHomo sapiens (human)
ThioredoxinTrx; ATL-derived factor; ADF; Surface-associated sulphydryl protein; SASPHomo sapiens (human)
Trifunctional purine biosynthetic protein adenosine-3Homo sapiens (human)
AMP deaminase 1EC 3.5.4.6; AMP deaminase isoform M; Myoadenylate deaminaseHomo sapiens (human)
Guanylate cyclase soluble subunit alpha-2GCS-alpha-2; EC 4.6.1.2Homo sapiens (human)
Adenylate kinase isoenzyme 1AK 1; EC 2.7.4.3; EC 2.7.4.6; ATP-AMP transphosphorylase 1; ATP:AMP phosphotransferase; Adenylate monophosphate kinase; MyokinaseHomo sapiens (human)
Adenylosuccinate lyaseADSL; ASL; EC 4.3.2.2; Adenylosuccinase; ASaseHomo sapiens (human)
Guanylate cyclase soluble subunit beta-1GCS-beta-1; EC 4.6.1.2; Guanylate cyclase soluble subunit beta-3; GCS-beta-3; Soluble guanylate cyclase small subunitHomo sapiens (human)
Inosine triphosphate pyrophosphatase ITPase; Inosine triphosphatase; EC 3.6.1.9; Non-canonical purine NTP pyrophosphatase; Non-standard purine NTP pyrophosphatase; Nucleoside-triphosphate diphosphatase; Nucleoside-triphosphate pyrophosphatase; NTPase; Putative oncogene protein hlc14-06-pHomo sapiens (human)
ADP-sugar pyrophosphataseEC 3.6.1.13; 8-oxo-dGDP phosphatase; 3.6.1.58; Nuclear ATP-synthesis protein NUDIX5; 2.7.7.96; Nucleoside diphosphate-linked moiety X motif 5; Nudix motif 5; hNUDT5; YSA1HHomo sapiens (human)
Adenine phosphoribosyltransferaseAPRT; EC 2.4.2.7Homo sapiens (human)
GMP reductase 1GMPR 1; EC 1.7.1.7; Guanosine 5'-monophosphate oxidoreductase 1; Guanosine monophosphate reductase 1Homo sapiens (human)
Nucleoside diphosphate kinase 6NDK 6; NDP kinase 6; EC 2.7.4.6; Inhibitor of p53-induced apoptosis-alpha; IPIA-alpha; nm23-H6Homo sapiens (human)
Ribose-phosphate pyrophosphokinase 3EC 2.7.6.1; Phosphoribosyl pyrophosphate synthase 1-like 1; PRPS1-like 1; Phosphoribosyl pyrophosphate synthase III; PRS-IIIHomo sapiens (human)
Outer mitochondrial transmembrane helix translocaseEC 7.4.2.-; ATPase family AAA domain-containing protein 1; hATAD1; ThoraseHomo sapiens (human)

Compounds (38)

CompoundDescription
phosphoric acidconcise etchant is 37% H3PO4
Orthophosphate
adenineA purine base and a fundamental unit of ADENINE NUCLEOTIDES.
ammonium hydroxideThe hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
glutamic acidA non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.
NADH
Adenosine TriphosphateAn adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
glutamineA non-essential amino acid present abundantly throughout the body and is involved in many metabolic processes. It is synthesized from GLUTAMIC ACID and AMMONIA. It is the principal carrier of NITROGEN in the body and is an important energy source for many cells.
adenosine diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
adenosine monophosphateAdenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.
adenosineA nucleoside that is composed of ADENINE and D-RIBOSE. Adenosine or adenosine derivatives play many important biological roles in addition to being components of DNA and RNA. Adenosine itself is a neurotransmitter.
phosphoribosyl pyrophosphateThe key substance in the biosynthesis of histidine, tryptophan, and purine and pyrimidine nucleotides.
Hydrogen PeroxideA strong oxidizing agent used in aqueous solution as a ripening agent, bleach, and topical anti-infective. It is relatively unstable and solutions deteriorate over time unless stabilized by the addition of acetanilide or similar organic materials.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
Adenosine Diphosphate RiboseAn ester formed between the aldehydic carbon of RIBOSE and the terminal phosphate of ADENOSINE DIPHOSPHATE. It is produced by the hydrolysis of nicotinamide-adenine dinucleotide (NAD) by a variety of enzymes, some of which transfer an ADP-ribosyl group to target proteins.
phosphoribosyl-N-formylglycineamide
glycineamide ribonucleotidestructure
uric acidAn oxidation product, via XANTHINE OXIDASE, of oxypurines such as XANTHINE and HYPOXANTHINE. It is the final oxidation product of purine catabolism in humans and primates, whereas in most other mammals URATE OXIDASE further oxidizes it to ALLANTOIN.
2'-deoxy-5'-adenosine monophosphateRN given refers to parent cpd
2'-deoxyadenosinewas (MH) DEOXYADENOSINE (68-89)
2'-deoxyadenosine triphosphateRN given refers to unlabeled parent cpd
ribose 1-phosphateRN given refers to (D)-isomer
2-deoxyribose 1-phosphate
NADA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
xanthosine
xanthosine 5'-triphosphateRN given refers to parent cpd
xanthineA purine base found in most body tissues and fluids, certain plants, and some urinary calculi. It is an intermediate in the degradation of adenosine monophosphate to uric acid, being formed by oxidation of hypoxanthine. The methylated xanthine compounds caffeine, theobromine, and theophylline and their derivatives are used in medicine for their bronchodilator effects. (Dorland, 28th ed)
saicaradenosylsuccinase catalyzes its conversion to AICAR
aminoimidazole ribotide
carboxyaminoimidazole ribotidereacts with alanosine to form antimetabolite; substrate for EC 4.1.1.21; structure in second source
5-formamidoimidazole-4-carboxamide ribotidepurine precursor
creolinfrom refined coal tar oils
Cyclic AMPAn adenine nucleotide containing one phosphate group which is esterified to both the 3'- and 5'-positions of the sugar moiety. It is a second messenger and a key intracellular regulator, functioning as a mediator of activity for a number of hormones, including epinephrine, glucagon, and ACTH.
pyrophosphate
aica ribonucleotidepurine precursor that has antineoplastic activity
aminoimidazole carboxamideAn imidazole derivative which is a metabolite of the antineoplastic agents BIC and DIC. By itself, or as the ribonucleotide, it is used as a condensation agent in the preparation of nucleosides and nucleotides. Compounded with orotic acid, it is used to treat liver diseases.