Overview

  • Product name

  • Description

    Rabbit polyclonal to ENPP1/PC1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Mouse
    Predicted to work with: Rat, Human
  • Immunogen

    Synthetic peptide within Human ENPP1/PC1 aa 270-320 conjugated to keyhole limpet haemocyanin. The exact sequence is proprietary.
    Sequence:

    ESHGIIDNKMYDPKMNASFSLKSKEKFNPEWYKGEPIWVTAKYQGLKSGT F


    Database link: P22413

  • Positive control

    • Mouse kidney lysate.
  • General notes

     This product was previously labelled as ENPP1

     

Properties

Applications

Our Abpromise guarantee covers the use of ab217368 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/100 - 1/1000. Predicted molecular weight: 105 kDa.

Target

  • 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.
  • 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.
  • Involvement in 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.
    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 similarities

    Belongs to the nucleotide pyrophosphatase/phosphodiesterase family.
    Contains 2 SMB (somatomedin-B) domains.
  • Domain

    The di-leucine motif is required for basolateral targeting in epithelial cells, and for targeting to matrix vesicles derived from mineralizing cells.
  • Post-translational
    modifications

    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.
  • Cellular localization

    Membrane. 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
  • Database links

  • Alternative names

    • Alkaline phosphodiesterase 1 antibody
    • ARHR2 antibody
    • COLED antibody
    • E-NPP 1 antibody
    • Ectonucleotide pyrophosphatase/phosphodiesterase 1 antibody
    • Ectonucleotide pyrophosphatase/phosphodiesterase family member 1 antibody
    • ENPP1 antibody
    • ENPP1_HUMAN antibody
    • Ly 41 antigen antibody
    • M6S1 antibody
    • Membrane component chromosome 6 surface marker 1 antibody
    • NPP1 antibody
    • NPPase antibody
    • NPPS antibody
    • Nucleotide pyrophosphatase antibody
    • PC 1 antibody
    • PC-1 antibody
    • PCA1 antibody
    • PDNP1 antibody
    • Phosphodiesterase I/nucleotide pyrophosphatase 1 antibody
    • Plasma cell membrane glycoprotein 1 antibody
    • Plasma-cell membrane glycoprotein PC-1 antibody
    see all

Images

  • Anti-ENPP1/PC1 antibody (ab217368) at 1/300 dilution + Mouse kidney lysate

    Secondary
    Goat Anti-Goat Anti-Rabbit IgG Antibody (H+L), HRP Conjugated at 1/5000 dilution

    Predicted band size: 105 kDa

References

ab217368 has not yet been referenced specifically in any publications.

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