Rabbit anti-Human ENPP1 Polyclonal Antibody | anti-ENPP1 antibody
ENPP1, NT (ENPP1, M6S1, NPPS, PC1, PDNP1, Ectonucleotide pyrophosphatase/phosphodiesterase family member 1, Membrane component chromosome 6 surface marker 1, Phosphodiesterase I/nucleotide pyrophosphatase 1, Plasma-cell membrane glycoprotein PC-1, Alkalin
Purified by Protein A affinity chromatography.
Purified by Protein A affinity chromatography.
Dilution: ELISA: 1:1,000
Western Blot: 1:100-500
NCBI and Uniprot Product Information
NCBI Description
This gene is a member of the ecto-nucleotide pyrophosphatase/phosphodiesterase (ENPP) family. The encoded protein is a type II transmembrane glycoprotein comprising two identical disulfide-bonded subunits. This protein has broad specificity and cleaves a variety of substrates, including phosphodiester bonds of nucleotides and nucleotide sugars and pyrophosphate bonds of nucleotides and nucleotide sugars. This protein may function to hydrolyze nucleoside 5' triphosphates to their corresponding monophosphates and may also hydrolyze diadenosine polyphosphates. Mutations in this gene have been associated with 'idiopathic' infantile arterial calcification, ossification of the posterior longitudinal ligament of the spine (OPLL), and insulin resistance. [provided by RefSeq, Jul 2008]
Uniprot Description
Function: By generating PPi, plays a role in regulating pyrophosphate levels, and functions in bone mineralization and soft tissue calcification. PPi inhibits mineralization by binding to nascent hydroxyapatite (HA) crystals, thereby preventing further growth of these crystals. 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. Ref.10
Catalytic activity: Hydrolytically removes 5'-nucleotides successively from the 3'-hydroxy termini of 3'-hydroxy-terminated oligonucleotides.A dinucleotide + H2O = 2 mononucleotides.
Cofactor: Binds 2 divalent metal cations per subunit
By similarity.
Enzyme regulation: At low concentrations of ATP, a phosphorylated intermediate is formed which inhibits further hydrolysis.
Subunit structure: The secreted form is monomeric
By similarity. Homodimer; disulfide-linked. Interacts with INSR. Ref.10
Subcellular location: Cell membrane; Single-pass type II membrane protein. Basolateral cell membrane; Single-pass type II membrane protein. Secreted
By similarity. Note: The proteolytically processed form is secreted
By similarity. 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. Ref.11 Ref.12
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. Ref.9
Domain: The di-leucine motif is required for basolateral targeting in epithelial cells, and for targeting to matrix vesicles derived from mineralizing cells
By similarity.
Post-translational modification: Autophosphorylated as part of the catalytic cycle of phosphodiesterase/pyrophosphatase activity.N-glycosylated.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.The interchain disulfide bonding resides in the transmembrane segment
By similarity.A secreted form is produced through cleavage at Lys-103 by intracellular processing
By similarity.
Involvement in disease: Ossification of the posterior longitudinal ligament of the spine (OPLL) [MIM:602475]: A calcification of the posterior longitudinal ligament of the spinal column, usually at the level of the cervical spine. Patients with OPLL frequently present with a severe myelopathy that can lead to tetraparesis.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.16Arterial calcification of infancy, generalized, 1 (GACI1) [MIM:208000]: A severe autosomal recessive disorder characterized by calcification of the internal elastic lamina of muscular arteries and stenosis due to myointimal proliferation. The disorder is often fatal within the first 6 months of life because of myocardial ischemia resulting in refractory heart failure.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.17 Ref.18 Ref.20 Ref.24Diabetes mellitus, non-insulin-dependent (NIDDM) [MIM:125853]: A multifactorial disorder of glucose homeostasis caused by a lack of sensitivity to the body's own insulin. Affected individuals usually have an obese body habitus and manifestations of a metabolic syndrome characterized by diabetes, insulin resistance, hypertension and hypertriglyceridemia. The disease results in long-term complications that affect the eyes, kidneys, nerves, and blood vessels.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry. Ref.19Hypophosphatemic rickets, autosomal recessive, 2 (ARHR2) [MIM:613312]: A hereditary form of hypophosphatemic rickets, a disorder of proximal renal tubule function that causes phosphate loss, hypophosphatemia and skeletal deformities, including rickets and osteomalacia unresponsive to vitamin D. Symptoms are bone pain, fractures and growth abnormalities.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.21 Ref.22
Sequence similarities: Belongs to the nucleotide pyrophosphatase/phosphodiesterase family.Contains 2 SMB (somatomedin-B) domains.
Caution: It is uncertain whether Met-1 or Met-53 is the initiator.
Sequence caution: The sequence AAA63237.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.The sequence AAH59375.2 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.The sequence BAA02054.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.
Research Articles on ENPP1
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Product Notes
The ENPP1 enpp1 (Catalog #AAA649905) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The ENPP1, NT (ENPP1, M6S1, NPPS, PC1, PDNP1, Ectonucleotide pyrophosphatase/phosphodiesterase family member 1, Membrane component chromosome 6 surface marker 1, Phosphodiesterase I/nucleotide pyrophosphatase 1, Plasma-cell membrane glycoprotein PC-1, Alkalin reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's ENPP1 can be used in a range of immunoassay formats including, but not limited to, ELISA (EL/EIA), Western Blot (WB). Suitable for use in Western Blot, ELISA Dilution: ELISA: 1:1,000 Western Blot: 1:100-500. Researchers should empirically determine the suitability of the ENPP1 enpp1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ENPP1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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