| Post Translational Modifications | Autophosphorylated as part of the catalytic cycle of phosphodiesterase/pyrophosphatase activity. N-glycosylated. The secreted form is produced through cleavage at Lys-103 by intracellular processing. |
| Function | Nucleotide pyrophosphatase that generates diphosphate (PPi) and functions in bone mineralization and soft tissue calcification by regulating pyrophosphate levels. PPi inhibits bone mineralization and soft tissue calcification by binding to nascent hydroxyapatite crystals, thereby preventing further growth of these crystals. Preferentially hydrolyzes ATP, but can also hydrolyze other nucleoside 5' triphosphates such as GTP, CTP and UTP to their corresponding monophosphates with release of pyrophosphate, as well as diadenosine polyphosphates, and also 3',5'-cAMP to AMP. May also be involved in the regulation of the availability of nucleotide sugars in the endoplasmic reticulum and Golgi, and the regulation of purinergic signaling. Inhibits ectopic joint calcification and maintains articular chondrocytes by repressing hedgehog signaling.it is however unclear whether hedgehog inhibition is direct or indirect. Appears to modulate insulin sensitivity and function. Also involved in melanogenesis. Also able to hydrolyze 2',3'-cGAMP (cyclic GMP-AMP), a second messenger that activates TMEM173/STING and triggers type-I interferon production. 2',3'-cGAMP degradation takes place in the lumen or extracellular space, and not in the cytosol where it is produced.the role of 2',3'-cGAMP hydrolysis is therefore unclear. Not able to hydrolyze the 2',3'-cGAMP linkage isomer 3'-3'-cGAMP. |
| Protein Name | Ectonucleotide Pyrophosphatase/Phosphodiesterase Family Member 1E-Npp 1Alkaline Phosphodiesterase IMembrane Component Chromosome 6 Surface Marker 1Nucleotide DiphosphataseNucleotide PyrophosphataseNppasePhosphodiesterase I/Nucleotide Pyrophosphatase 1Plasma-Cell Membrane Glycoprotein Pc-1 Cleaved Into - Ectonucleotide Pyrophosphatase/Phosphodiesterase Family Member 1 - Secreted Form |
| Database Links | Reactome: R-HSA-196843Reactome: R-HSA-199220 |
| Cellular Localisation | Ectonucleotide Pyrophosphatase/Phosphodiesterase Family Member 1: Cell MembraneSingle-Pass Type Ii Membrane ProteinBasolateral Cell MembraneTargeted To The Basolateral Membrane In Polarized Epithelial Cells And In HepatocytesAnd To Matrix Vesicles In OsteoblastsIn Bile Duct Cells And Cancer CellsLocated To The Apical Cytoplasmic SideEctonucleotide Pyrophosphatase/Phosphodiesterase Family Member 1Secreted Form: SecretedSecreted Following Proteolytic Cleavage |
| Alternative Protein Names | Ectonucleotide Pyrophosphatase/Phosphodiesterase Family Member 1 proteinE-Npp 1 proteinAlkaline Phosphodiesterase I proteinMembrane Component Chromosome 6 Surface Marker 1 proteinNucleotide Diphosphatase proteinNucleotide Pyrophosphatase proteinNppase proteinPhosphodiesterase I/Nucleotide Pyrophosphatase 1 proteinPlasma-Cell Membrane Glycoprotein Pc-1 Cleaved Into - Ectonucleotide Pyrophosphatase/Phosphodiesterase Family Member 1 - Secreted Form proteinENPP1 proteinM6S1 proteinNPPS proteinPC1 proteinPDNP1 protein |
Information sourced from Uniprot.org
