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.

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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[Isoform 1]: Cell membrane; Single-pass type I membrane protein.; [Isoform 2]: Endoplasmic reticulum membrane; Single-pass type I membrane protein.; [Isoform 3]: Secreted.

ATR family

Expressed in prostate, thymus, ovary, testis, pancreas, colon, heart, kidney, lung, liver, peripheral blood leukocytes, placenta, skeletal muscle, small intestine and spleen.

HGNC: 21732

OMIM: 228600

KEGG: hsa:118429

STRING: 9606.ENSP00000306185

UniGene: Hs.162963