Product nameAnti-ENPP1 antibody
See all ENPP1 primary antibodies
DescriptionGoat polyclonal to ENPP1
Tested applicationsSuitable for: IHC-Fr, IHC-P, WBmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat
- Human liver and pancreas tissue sections. Lysate prepared from transfected HEK293 cells transiently expressing ENPP1.
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage bufferpH: 7.30
Preservative: 0.02% Sodium azide
Constituents: Tris buffered saline, 0.5% BSA
Concentration information loading...
PurityImmunogen affinity purified
Purification notesPurified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Our Abpromise guarantee covers the use of ab40003 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-Fr||1/250. PubMed: 23225236|
|IHC-P||Use at an assay dependent concentration.|
|WB||Use a concentration of 1 - 3 µg/ml. Detects a band of approximately 100 kDa (predicted molecular weight: 105 kDa).|
FunctionInvolved 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.
Tissue specificityExpressed in plasma cells and also in a number of non-lymphoid tissues, including the distal convoluted tubule of the kidney, chondrocytes and epididymis.
Involvement in diseaseDefects 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.
Defects in ENPP1 are the cause of arterial calcification of infancy, generalized, type 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.
Defects in ENPP1 are associated with obesity, glucose intolerance, and type II diabetes non-insulin dependent (NIDDM) [MIM:125853].
Defects in ENPP1 are the cause of rickets hypophosphatemic autosomal recessive type 2 (ARHR2) [MIM:613312]. ARHR2 is 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.
Sequence similaritiesBelongs to the nucleotide pyrophosphatase/phosphodiesterase family.
Contains 2 SMB (somatomedin-B) domains.
DomainThe di-leucine motif is required for basolateral targeting in epithelial cells, and for targeting to matrix vesicles derived from mineralizing cells.
modificationsAutophosphorylated as part of the catalytic cycle of phosphodiesterase/pyrophosphatase activity.
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.
Cellular localizationMembrane. Basolateral cell membrane. 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.
- Information by UniProt
- Alkaline phosphodiesterase 1 antibody
- ARHR2 antibody
- COLED antibody
Ab40003 (1ug/ml) staining human ENPP1 in human liver tissue by immunohistochemistry using paraffin embedded tissue. Microwaved antigen retrieval with citrate buffer pH 6, HRP-staining.
Anti-ENPP1 antibody (ab40003) at 3 µg/ml + lysate prepared from transfected HEK293 cells, transiently expressing ENPP1
Predicted band size: 105 kDa
Observed band size: 100 kDa why is the actual band size different from the predicted?
This product has been referenced in:
- Pérez de Lara MJ et al. Increased Ap4A levels and ecto-nucleotidase activity in glaucomatous mice retina. Purinergic Signal 14:259-270 (2018). Read more (PubMed: 29948577) »
- Aliagas E et al. Ecto-nucleotidases distribution in human cyclic and postmenopausic endometrium. Purinergic Signal 9:227-37 (2013). IHC-Fr ; Human . Read more (PubMed: 23225236) »