信号通路： Purine metabolism;Starch and sucrose metabolism;Riboflavin metabolism;Nicotinate and nicotinamide metabolism;Pantothenate and CoA biosynthesis;
功能： catalytic activity:A dinucleotide + H(2)O = 2 mononucleotides.,catalytic activity:Hydrolytically removes 5'-nucleotides successively from the 3'-hydroxy termini of 3'-hydroxy-terminated oligonucleotides.,caution:It is uncertain whether Met-1 or Met-53 is the initiator.,cofactor:Binds 2 divalent metal cations per subunit.,disease:Defects in ENPP1 are a cause of idiopathic infantile arterial calcification (IIAC) [MIM:208000]; also called generalized arterial calcification of infancy. IIAC is characterized by calcification of the internal elastic lamina of muscular arteries and stenosis due to myointimal proliferation.,disease:Defects in ENPP1 are a cause of increased susceptibility for ossification of the posterior longitudinal ligament of the spine (OPLL) [MIM:602475]. OPLL is a common form of human myelopathy with a prevalence of as much as 4% in a variety of ethnic groups.,disease:Defects in ENPP1 are associated with obesity, glucose intolerance, and type II diabetes non-insulin dependent (NIDDM) [MIM:125853].,domain:The di-leucine motif is required for basolateral targeting in epithelial cells, and for targeting to matrix vesicles derived from mineralizing cells.,enzyme regulation:At low concentrations of ATP, a phosphorylated intermediate is formed which inhibits further hydrolysis.,function: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.,PTM:Autophosphorylated as part of the catalytic cycle of phosphodiesterase/pyrophosphatase activity.,PTM:It has been suggested that the active SMB domain may be permitted considerable disulfide bond heterogeneity or variability, thus two alternate disulfide patterns based on 3D structures are described with 1 disulfide bond conserved in both.,PTM:N-glycosylated.,similarity:Belongs to the nucleotide pyrophosphatase/phosphodiesterase family.,similarity:Contains 2 SMB (somatomedin-B) domains.,subcellular location:Targeted to the basolateral membrane in polarized epithelial cells and in hepatocytes, and to matrix vesicles in osteoblasts. In bile duct cells and cancer cells, located to the apical cytoplasmic side.,subunit:Homodimer; disulfide-linked.,tissue specificity:Expressed in plasma cells and also in a number of non-lymphoid tissues, including the distal convoluted tubule of the kidney, chondrocytes and epididymis.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： [Ectonucleotide pyrophosphatase/phosphodiesterase family member 1]: Cell membrane ; Single-pass type II membrane protein. Basolateral cell membrane ; Single-pass type II membrane protein. Targeted to the basolateral membrane in polarized epithelial cells and in hepatocytes, and to matrix vesicles in osteoblasts (PubMed:11598187). In bile duct cells and cancer cells, located to the apical cytoplasmic side (PubMed:11598187). .; [Ectonucleotide pyrophosphatase/phosphodiesterase family member 1, secreted form]: Secreted . Secreted following proteolytic cleavage. .
组织表达： Expressed in plasma cells and also in a number of non-lymphoid tissues, including the distal convoluted tubule of the kidney, chondrocytes and epididymis (PubMed:9344668). Expressed in melanocytes but not in keratinocytes (PubMed:28964717).
信号通路： Purine metabolism;Starch and sucrose metabolism;Riboflavin metabolism;Nicotinate and nicotinamide metabolism;Pantothenate and CoA biosynthesis;
功能： catalytic activity:A dinucleotide + H(2)O = 2 mononucleotides.,catalytic activity:Hydrolytically removes 5'-nucleotides successively from the 3'-hydroxy termini of 3'-hydroxy-terminated oligonucleotides.,caution:It is uncertain whether Met-1 or Met-53 is the initiator.,cofactor:Binds 2 divalent metal cations per subunit.,disease:Defects in ENPP1 are a cause of idiopathic infantile arterial calcification (IIAC) [MIM:208000]; also called generalized arterial calcification of infancy. IIAC is characterized by calcification of the internal elastic lamina of muscular arteries and stenosis due to myointimal proliferation.,disease:Defects in ENPP1 are a cause of increased susceptibility for ossification of the posterior longitudinal ligament of the spine (OPLL) [MIM:602475]. OPLL is a common form of human myelopathy with a prevalence of as much as 4% in a variety of ethnic groups.,disease:Defects in ENPP1 are associated with obesity, glucose intolerance, and type II diabetes non-insulin dependent (NIDDM) [MIM:125853].,domain:The di-leucine motif is required for basolateral targeting in epithelial cells, and for targeting to matrix vesicles derived from mineralizing cells.,enzyme regulation:At low concentrations of ATP, a phosphorylated intermediate is formed which inhibits further hydrolysis.,function: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.,PTM:Autophosphorylated as part of the catalytic cycle of phosphodiesterase/pyrophosphatase activity.,PTM:It has been suggested that the active SMB domain may be permitted considerable disulfide bond heterogeneity or variability, thus two alternate disulfide patterns based on 3D structures are described with 1 disulfide bond conserved in both.,PTM:N-glycosylated.,similarity:Belongs to the nucleotide pyrophosphatase/phosphodiesterase family.,similarity:Contains 2 SMB (somatomedin-B) domains.,subcellular location:Targeted to the basolateral membrane in polarized epithelial cells and in hepatocytes, and to matrix vesicles in osteoblasts. In bile duct cells and cancer cells, located to the apical cytoplasmic side.,subunit:Homodimer; disulfide-linked.,tissue specificity:Expressed in plasma cells and also in a number of non-lymphoid tissues, including the distal convoluted tubule of the kidney, chondrocytes and epididymis.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： [Ectonucleotide pyrophosphatase/phosphodiesterase family member 1]: Cell membrane ; Single-pass type II membrane protein. Basolateral cell membrane ; Single-pass type II membrane protein. Targeted to the basolateral membrane in polarized epithelial cells and in hepatocytes, and to matrix vesicles in osteoblasts (PubMed:11598187). In bile duct cells and cancer cells, located to the apical cytoplasmic side (PubMed:11598187). .; [Ectonucleotide pyrophosphatase/phosphodiesterase family member 1, secreted form]: Secreted . Secreted following proteolytic cleavage. .
组织表达： Expressed in plasma cells and also in a number of non-lymphoid tissues, including the distal convoluted tubule of the kidney, chondrocytes and epididymis (PubMed:9344668). Expressed in melanocytes but not in keratinocytes (PubMed:28964717).
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