ITPA Pyrophosphatase that hydrolyzes the non-canonical purine nucleotides inosine triphosphate (ITP), deoxyinosine triphosphate (dITP) as well as 2'-deoxy-N-6-hydroxylaminopurine triposphate (dHAPTP) and xanthosine 5'-triphosphate (XTP) to their respective monophosphate derivatives. The enzyme does not distinguish between the deoxy- and ribose forms. Probably excludes non-canonical purines from RNA and DNA precursor pools, thus preventing their incorporation into RNA and DNA and avoiding chromosomal lesions. Belongs to the HAM1 NTPase family. Ubiquitous. Highly expressed in heart, liver, sex glands, thyroid and adrenal gland. 3 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: EC; Hydrolase; Nucleotide Metabolism - purine; Nucleotide Metabolism - pyrimidine; Xenobiotic Metabolism - drug metabolism - other enzymes
Chromosomal Location of Human Ortholog: 20p13
Cellular Component:  cytosol
Molecular Function:  dITP diphosphatase activity; identical protein binding; metal ion binding; NADH pyrophosphatase activity; nucleotide binding
Biological Process:  chromosome organization; deoxyribonucleoside triphosphate catabolic process; ITP catabolic process; purine nucleotide catabolic process
Disease: Epileptic Encephalopathy, Early Infantile, 35; Inosine Triphosphatase Deficiency
Reference #:  Q9BY32 (UniProtKB)
Alt. Names/Synonyms: C20orf37; dJ794I6.3; HLC14-06-P; Inosine triphosphatase; inosine triphosphatase (nucleoside triphosphate pyrophosphatase); inosine triphosphatase-A; Inosine triphosphate pyrophosphatase; inosine triphosphate pyrophosphohydrolase; ITPA; ITPase; My049 protein; nucleoside triphosphate diphosphatase; Putative oncogene protein hlc14-06-p
Gene Symbols: ITPA
Molecular weight: 21,446 Da
Basal Isoelectric point: 5.5  Predict pI for various phosphorylation states
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Protein Structure Not Found.

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