ANTR2_HUMAN » Anthrax toxin receptor 2

ANTR2_HUMAN » Anthrax toxin receptor 2
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
ANTR2_HUMAN » Anthrax toxin receptor 2 » Capillary morphogenesis gene 2 protein;CMG-2;
Hydrophobic Thickness 37.6 ± 2.4 Å
Tilt Angle 1 ± 1°
ΔGtransfer -51.6 kcal/mol
ΔGfold -23.6 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, HMDB
Topology Out
TM Segments 318-340 (312-341)
Pathways none
PDB 1sht (38-217), 1shu (38-218), 1tzn (a/b...=38-218), 1t6b (Y=40-212)
OPM none
Complexes none
Interactions

LRP6, Complex: ANTR2:LRP6, PubMed

Domains

AA: 44-212, PDBID: 1SHT, Subunit X, Seq Identity:100%, von Willebrand factor type A domain

AA: 216-317, PDBID: 1SHU, Subunit X, Seq Identity:100%, Anthrax receptor extracellular domain

AA: 394-484, Anthrax receptor C-terminus region

UniProt annotation for ANTR2_HUMAN » Anthrax toxin receptor 2
FUNCTION: Necessary for cellular interactions with laminin and the extracellular matrix.

SUBUNIT: Binds laminin, and possibly also collagen type IV. Binds to the protective antigen (PA) of Bacillus anthracis in a divalent cation-dependent manner, with the following preference: calcium > manganese > magnesium > zinc. Binding of PA leads to heptamerization of the receptor-PA complex.

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

DOMAIN: Binding to PA seems to be effected through the VWA domain.

DISEASE: Hyaline fibromatosis syndrome (HFS) OMIM: An autosomal recessive syndrome characterized by abnormal growth of hyalinized fibrous tissue usually affecting subcutaneous regions on the scalp, ears, neck, face, hands, and feet. The lesions appear as pearly papules or fleshy nodules. Additional features include gingival hypertrophy, progressive joint contractures resulting in severe limitation of mobility, osteopenia, and osteoporosis. Disease severity is variable. Some individuals manifest symptoms in infancy and have additional visceral or systemic involvement. Hyaline deposits in multiple organs, recurrent infections and intractable diarrhea often lead to early death. Surviving children may suffer from severely reduced mobility due to joint contractures. Other patients have later onset of a milder disorder affecting only the face and digits. mutations affecting the gene represented in this entry.

MISCELLANEOUS: Upon binding of the protective antigen (PA) of Bacillus 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.

UniProt features for ANTR2_HUMAN » Anthrax toxin receptor 2
SIGNAL 1 33 Potential.
CHAIN 34 489 Anthrax toxin receptor 2.
DOMAIN 44 213 VWFA.
DISULFID 39 218
Amino Acid Sequence for ANTR2_HUMAN » Anthrax toxin receptor 2
MVAERSPARS PGSWLFPGLW LLVLSGPGGL LRAQEQPSCR RAFDLYFVLD KSGSVANNWI EIYNFVQQLA ERFVSPEMRL SFIVFSSQAT IILPLTGDRG KISKGLEDLK RVSPVGETYI HEGLKLANEQ IQKAGGLKTS SIIIALTDGK LDGLVPSYAE KEAKISRSLG ASVYCVGVLD FEQAQLERIA DSKEQVFPVK GGFQALKGII NSILAQSCTE ILELQPSSVC VGEEFQIVLS GRGFMLGSRN GSVLCTYTVN ETYTTSVKPV SVQLNSMLCP APILNKAGET LDVSVSFNGG KSVISGSLIV TATECSNGIA AIIVILVLLL LLGIGLMWWF WPLCCKVVIK DPPPPPPPAP KEEEEEPLPT KKWPTVDASY YGGRGVGGIK RMEVRWGDKG STEEGARLEK AKNAVVKIPE ETEEPIRPRP PRPKPTHQPP QTKWYTPIKG RLDALWALLR RQYDRVSLMR PQEGDEVCIW ECIEKELTA