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anthrax toxin receptor 2 (capillary morphogenesis gene 2 protein, CMG-2, ANTXR2, CMG2)
Function:
1) role in cellular interactions with laminin & extracellular matrix
2) binds laminin, & possibly collagen type 5
Structure:
1) belongs to the ATR family
2) 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:
1) 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
2) defects in ANTXR2 are the cause of infantile systemic hyalinosis
3) defects in ANTXR2 are the cause of juvenile hyaline fibromatosis
General
cell surface receptor
glycoprotein
Properties
SIZE: MW = 54 kD
entity length = 489 aa
COMPARTMENT: endoplasmic reticulum
MOTIF: signal sequence {1-33}
cysteine residue {C39}
MODIFICATION: cysteine residue {C218}
VWFA domain {44-213}
cysteine residue {C218}
MODIFICATION: cysteine residue {C39}
N-glycosylation site {N250}
N-glycosylation site {N260}
transmembrane domain {319-341}
proline-rich region
SITE: 352-360
MOTIF: proline residue (SEVERAL)
glutamate-rich region {362-366}
MOTIF: glutamate residue (SEVERAL)
Database Correlations
OMIM correlations
UniProt P58335
References
UniProt :accession P58335