anthrax toxin receptor 2 (capillary morphogenesis gene 2 protein, CMG-2, ANTXR2, CMG2)

Function

• role in cellular interactions with laminin & extracellular matrix
• binds laminin, & possibly collagen type 5

Structure

• belongs to the ATR family
• contains 1 VWFA domain

Compartment

• isoform 1: cell membrane
• isoform 2: endoplasmic reticulum
• isoform 3: secreted protein

Alternative splicing

named isoforms=4

Expression

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

Pathology

• binds protective antigen (PA) of Bacillus anthracis (divalent cation-dependent manner,preference: Ca+2 > Mn+2 > Mg+2 > Zn+2
  • binding of PA leads to heptamerization of the receptor-PA complex
  • binding to PA seems to be effected through the VWA domain
  • upon binding of the protective antigen the complex moves to glycosphingolipid-rich lipid rafts, where it is internalized via a clathrin-dependent pathway
• defects in ANTXR2 are the cause of infantile systemic hyalinosis
• defects in ANTXR2 are the cause of juvenile hyaline fibromatosis

More general terms

• cell surface receptor
• glycoprotein

Database

• OMIM: https://mirror.omim.org/entry/228600
• OMIM: https://mirror.omim.org/entry/236490
• OMIM: https://mirror.omim.org/entry/608041
• UniProt: http://www.uniprot.org/uniprot/P58335.html