概述

  • 产品名称Anti-ENPP1抗体
    参阅全部 ENPP1 一抗
  • 描述
    山羊多克隆抗体to ENPP1
  • 经测试应用适用于: IHC-Fr, IHC-P, WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat
  • 免疫原

    Synthetic peptide:

    KTHLPTFSQED

    , corresponding to C terminal amino acids 915-925 of Human ENPP1

  • 阳性对照
    • Human liver and pancreas tissue sections. Lysate prepared from transfected HEK293 cells transiently expressing ENPP1.

性能

应用

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.

应用 Ab评论 说明
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).

靶标

  • 功能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.
  • 组织特异性Expressed in plasma cells and also in a number of non-lymphoid tissues, including the distal convoluted tubule of the kidney, chondrocytes and epididymis.
  • 疾病相关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.
  • 序列相似性Belongs to the nucleotide pyrophosphatase/phosphodiesterase family.
    Contains 2 SMB (somatomedin-B) domains.
  • 结构域The di-leucine motif is required for basolateral targeting in epithelial cells, and for targeting to matrix vesicles derived from mineralizing cells.
  • 翻译后修饰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.
  • 细胞定位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
  • 数据库链接
  • 别名
    • Alkaline phosphodiesterase 1 antibody
    • ARHR2 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
    • 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

Anti-ENPP1 antibody 图像

  • 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?)

Anti-ENPP1 antibody (ab40003)参考文献

This product has been referenced in:
  • 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) »
  • Foster BL  et al. Central role of pyrophosphate in acellular cementum formation. PLoS One 7:e38393 (2012). IHC-P ; Mouse . Read more (PubMed: 22675556) »

See all 4 Publications for this product

Product Wall

Application Immunohistochemistry (Frozen sections)
Blocking step Serum as blocking agent for 2 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 23°C
Sample Mouse Tissue sections (Brain)
Specification Brain
Permeabilization Yes - Triton
Fixative Paraformaldehyde
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提交于 Aug 14 2014

Abreviews
Abcam has not validated the combination of species/application used in this Abreview.
Application Western blot
Sample Rat Cell lysate - whole cell (Osteoblast)
Loading amount 20 µg
Specification Osteoblast
Blocking step Milk as blocking agent for 2 hour(s) and 0 minute(s) · Concentration: 5%
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提交于 Apr 02 2007

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