ITPase (inosine triphosphate pyrophosphatase) is also known as putative oncogene protein hlc14-06-p or ITPA (inosine triphosphatase (nucleoside triphosphate pyrophosphatase)) and is a 194 amino acid protein. ITPase is abundantly expressed in heart, liver, sex glands, thyroid and adrenal gland, and is localized to the cytoplasm in the cell. ITPase catalyzes the pyrophosphohydrolysis of both ITP (inosine triphosphate) and dITP (deoxyinosine triphosphate) to IMP (inosine monophosphate) and diphosphate. IMP can be used as a substrate for purine nucleotide pathways. IMP can be phosphorylated to ITP, and ITPase can regulate the concentration of ITP in the cell by converting ITP back to IMP. Defects in ITPase result in ITPase deficiency which is thought to be inherited and is characterized by an over-accumulation of ITP in erythocytes, leukocytes and fibroblasts.
