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ENPP1 Involved primarily in ATP hydrolysis at the plasma membrane. Plays a role in regulating pyrophosphate levels, and functions in bone mineralization and soft tissue calcification. In vitro, has a broad specificity, hydrolyzing other nucleoside 5' triphosphates such as GTP, CTP, TTP and UTP to their corresponding monophosphates with release of pyrophosphate and 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. Appears to modulate insulin sensitivity. Homodimer; disulfide-linked. Interacts with INSR. Expressed in plasma cells and also in a number of non-lymphoid tissues, including the distal convoluted tubule of the kidney, chondrocytes and epididymis. At low concentrations of ATP, a phosphorylated intermediate is formed which inhibits further hydrolysis. Belongs to the nucleotide pyrophosphatase/phosphodiesterase family. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - starch and sucrose; Cofactor and Vitamin Metabolism - nicotinate and nicotinamide; Cofactor and Vitamin Metabolism - pantothenate and CoA biosynthesis; Cofactor and Vitamin Metabolism - riboflavin; EC; EC; Membrane protein, integral; Motility/polarity/chemotaxis; Nucleotide Metabolism - purine; Phosphatase (non-protein); Phosphodiesterase
Chromosomal Location of Human Ortholog: 6q23.2
Cellular Component: cell surface; extracellular space; integral to membrane; integral to plasma membrane; lysosomal membrane; plasma membrane
Molecular Function: 3'-phosphoadenosine 5'-phosphosulfate binding; ATP binding; calcium ion binding; insulin receptor binding; nucleoside-triphosphate diphosphatase activity; nucleotide diphosphatase activity; phosphodiesterase I activity; protein binding; protein homodimerization activity; zinc ion binding
Biological Process: 3'-phosphoadenosine 5'-phosphosulfate metabolic process; ATP metabolic process; cellular phosphate ion homeostasis; cellular response to insulin stimulus; generation of precursor metabolites and energy; inorganic diphosphate transport; negative regulation of cell growth; negative regulation of fat cell differentiation; negative regulation of glucose import; negative regulation of glycogen biosynthetic process; negative regulation of insulin receptor signaling pathway; negative regulation of protein amino acid autophosphorylation; nucleoside triphosphate catabolic process; phosphate metabolic process; regulation of bone mineralization; riboflavin metabolic process; sequestering of triacylglycerol
Disease: Arterial Calcification, Generalized, Of Infancy, 1; Cole Disease; Diabetes Mellitus, Noninsulin-dependent; Hypophosphatemic Rickets, Autosomal Recessive, 2; Obesity
Reference #:  P22413 (UniProtKB)
Alt. Names/Synonyms: alkaline phosphodiesterase 1; Alkaline phosphodiesterase I; ARHR2; E-NPP 1; ectonucleotide pyrophosphatase/phosphodiesterase 1; Ectonucleotide pyrophosphatase/phosphodiesterase family member 1; ENPP1; Ly-41 antigen; M6S1; Membrane component chromosome 6 surface marker 1; membrane component, chromosome 6, surface marker 1; NPP1; NPPase; NPPS; Nucleotide pyrophosphatase; PC-1; PC1; PCA1; PDNP1; Phosphodiesterase I/nucleotide pyrophosphatase 1; plasma-cell membrane glycoprotein 1; Plasma-cell membrane glycoprotein PC-1
Gene Symbols: ENPP1
Molecular weight: 104,924 Da
Basal Isoelectric point: 6.76  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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