ENPP3

Ectonucleotide pyrophosphatase/phosphodiesterase 3 (ENPP3), also known as B10, NPP3, PDNP3, CD203c or PD-IBETA, is a 150 kDa type II transmembrane glycoprotein that belongs to the nucleotide pyrophosphatase/phosphodiesterase family. These proteins are involved in hydrolysis of extracellular nucleotides. These ectoenzymes also possess ATPase and ATP pyrophosphatase activitie.

The extracellular domain of ENPP3 is comprised of a catalytic domain, a nuclease-like domain and a somatomedin B-like domain. ENPP3 can cleave a variety of extracellular phosphodiester and phosphosulfate bonds as well as intracellular nucleotides involved in glycosyltransferase activity. This protein is expressed on multiple cells that include activated basophils, mast cells during allergic reactions, cardiac muscle, hepatocytes, and epithelial cells. It is upregulated in colon and bile duct carcinomas. Moreover, the expression of NPP3 has been reported in numerous tumor cells, including colon cancer, renal cell carcinoma (RCC), neoplastic cells of the bile duct, acute basophilic leukemia and neoplastic mast cells. The unique expression pattern of ENPP3 makes it an appealing target for cancer therapy, and an anti-ENPP3 antibody- drug conjugate, AGS16F, is currently being evaluated in a Phase I clinical trial for RCC.

Gene ID: 5169
UniProt ID: O14638