ANTXR2 Antibody

Code CSB-PA001833GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
Alternative Names
ANTXR2; CMG2; Anthrax toxin receptor 2; Capillary morphogenesis gene 2 protein; CMG-2
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Human ANTXR2
Immunogen Species
Homo sapiens (Human)
Isotype
IgG
Purification Method
Antigen Affinity purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
ELISA,IHC
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Necessary for cellular interactions with laminin and the extracellular matrix.; (Microbial infection) Receptor for the protective antigen (PA) of B.anthracis. Binding of PA leads to heptamerization of the receptor-PA complex. Upon binding of the PA of B.anthracis, the complex moves to glycosphingolipid-rich lipid rafts, where it is internalized via a clathrin-dependent pathway. In the endosomal membrane, at pH under 7, the complex then rearranges and forms a pore allowing the other components of anthrax toxin to escape to the cytoplasm.
Gene References into Functions
  1. A decreased CMG2 expression is a negative prognostic factor for soft tissue sarcoma patients. PMID: 29215551
  2. Results identified Gly116Val mutation in ANTXR2 associated with hyaline fibromatosis syndrome. PMID: 28103792
  3. Stability of domain 4 of the anthrax toxin protective antigen and the effect of the VWA domain of CMG2 on stability PMID: 27874231
  4. study examines 4 cases with clinical features of hyaline fibromatosis syndrome; identified a previously unreported splice junction mutation (c.946-2A-->G in intron 11) and a recurrent founder mutation ( c.1074delT) PMID: 27174544
  5. This study reports a novel association between ANTXR2 and ankylosing spondylitis in the Han Chinese. PMID: 26728147
  6. Case Report: systemic hyalinosis with a heterozygous mutation in CMG2. PMID: 26885603
  7. The association between ANTXR2 rs4333130 and ankylosing spondylitis was independent of HLA-B27 status. Clinical disease severity scores (BASDAI and BASFI) and pain score were higher in ANTXR2 rs4333130 CT genotype. PMID: 26590821
  8. Roles of Anthrax Toxin Receptor 2 in Anthrax Toxin Membrane Insertion and Pore Formation PMID: 26805886
  9. Anthrax Susceptibility: Human Genetic Polymorphisms Modulating ANTXR2 Expression PMID: 26703731
  10. miR-124 might induce autophagy to participate in AS by targeting ANTXR2, which might be implicated in pathological process of AS. PMID: 25736362
  11. The disulfide bond Cys255-Cys279 in the immunoglobulin-like domain of ANTXR2 is required for membrane insertion of anthrax protective antigen pore. PMID: 26107617
  12. CMG2 glycosylation provides a buffer towards genetic variation by promoting folding of the protein in the ER lumen. PMID: 25781883
  13. Results show that CMG2 has been shown to be able to regulate the proliferation and tubule formation of endothelial cells, but not the migration. PMID: 24993339
  14. Ankylosing spondylitis is associated with the anthrax toxin receptor 2 gene. PMID: 25169729
  15. data suggested that cellular cholesterol regulated ANTXR2-dependent activation of MMP-2 via ERK1/2 phosphorylation in neuroglioma U251 cell. PMID: 24924630
  16. Fused the ANTXR2 ectodomain to the C-terminus of bacterial Trigger Factor (TF), and the fusion protein was overly expressed as a dominant soluble protein in E coli. PMID: 24380801
  17. Silencing CMG2 using targeted siRNAs provided almost complete protection against anthrax lethal toxin-induced cytotoxicity and death in murine and human macrophages. PMID: 24742682
  18. Loss of CMG2 expression is associated with breast cancer progression. PMID: 24667935
  19. A protective antigen mutation increases the pH threshold of anthrax toxin receptor 2-mediated pore formation PMID: 24641616
  20. Investigated the kinetics of binding as a function of pH to the full-length monomeric anthrax protective antigen(PA)and to two variants to CMG2. Results suggest that for the full-length PA, low pH increases the binding affinity. PMID: 22855243
  21. 2 novel ANTXR2 mutations were identified in patients with hyaline fibromatosis syndrome PMID: 22383261
  22. ANTXR2 and IL-1R2 polymorphisms are not associated with ankylosing spondylitis in Chinese Han population. PMID: 20652271
  23. results reveal extensive diversity in cell lethality dependent on anthrax toxin PA-mediated toxin binding and uptake, and identify individual differences in CMG2 expression level as a determinant of this diversity PMID: 22315420
  24. ANTXR2 might not be a susceptibility gene of Ankylosing Spondylitis in Chinese Han. PMID: 22118297
  25. Missense mutations in the extracellular von Willebrand domain of CMG2 lead to protein folding defects. PMID: 21328543
  26. the molecular basis (spectrum of ANTXR2 mutations) of infantile systemic hyalinosis and juvenile hyaline fibromatosis in two unrelated Egyptian families PMID: 20331448
  27. Divalent metal ion coordination by residue T118 of anthrax toxin receptor 2 is not essential for protective antigen binding. PMID: 17183731
  28. Cytoplasmic delivery of lethal factor by anthrax toxin receptor 2 was mediated by cathepsin B. PMID: 19858192
  29. identification and characterization of juvenile hyaline fibromatosis and infantile systemic hyalinosis disease-causing mutations in the capillary morphogenesis factor-2 gene PMID: 12973667
  30. identification of 15 different mutations in the gene encoding capillary morphogenesis protein 2 in 17 families with juvenile hyaline fibromatosis or infantile systemic hyalinosis PMID: 14508707
  31. structures provide a template to begin probing the high-affinity capillary morphogenesis protein 2-protective antigen interaction and may facilitate understanding of toxin assembly/internalization PMID: 15079089
  32. crystal structure of the complex with anthrax toxin PMID: 15243628
  33. Molecular dynamics simulations explain the great strength that the protective antigen-capillary morphogenesis gene 2 complex achieves through extraordinary coordination of a divalent cation. PMID: 16473908
  34. Results suggest that, in vivo, slow folding, rather than misfolding, is responsible for ER retention, and that systemic hyalinosis can be qualified as a conformational disease. PMID: 19191226

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Involvement in disease
Hyaline fibromatosis syndrome (HFS)
Subcellular Location
[Isoform 1]: Cell membrane; Single-pass type I membrane protein.; [Isoform 2]: Endoplasmic reticulum membrane; Single-pass type I membrane protein.; [Isoform 3]: Secreted.
Protein Families
ATR family
Tissue Specificity
Expressed in prostate, thymus, ovary, testis, pancreas, colon, heart, kidney, lung, liver, peripheral blood leukocytes, placenta, skeletal muscle, small intestine and spleen.
Database Links

HGNC: 21732

OMIM: 228600

KEGG: hsa:118429

STRING: 9606.ENSP00000306185

UniGene: Hs.162963

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