2fcb

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Template:STRUCTURE 2fcb

Contents 1 HUMAN FC GAMMA RECEPTOR IIB ECTODOMAIN (CD32) 2 Disease 3 Function 4 About this Structure 5 Reference

HUMAN FC GAMMA RECEPTOR IIB ECTODOMAIN (CD32)

Template:ABSTRACT PUBMED 10064577

Disease

[FCG2B_HUMAN] Note=A chromosomal aberration involving FCGR2B is found in a follicular lymphoma. Translocation t(1;22)(q22;q11). The translocation leads to the hyperexpression of the receptor. This may play a role in the tumor progression. Defects in FCGR2B are a cause of susceptibility to systemic lupus erythematosus (SLE) [MIM:152700]. A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.^[1][2]

Function

[FCG2B_HUMAN] Receptor for the Fc region of complexed or aggregated immunoglobulins gamma. Low affinity receptor. Involved in a variety of effector and regulatory functions such as phagocytosis of immune complexes and modulation of antibody production by B-cells. Binding to this receptor results in down-modulation of previous state of cell activation triggered via antigen receptors on B-cells (BCR), T-cells (TCR) or via another Fc receptor. Isoform IIB1 fails to mediate endocytosis or phagocytosis. Isoform IIB2 does not trigger phagocytosis.

About this Structure

2fcb is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

• Sondermann P, Huber R, Jacob U. Crystal structure of the soluble form of the human fcgamma-receptor IIb: a new member of the immunoglobulin superfamily at 1.7 A resolution. EMBO J. 1999 Mar 1;18(5):1095-103. PMID:10064577 doi:10.1093/emboj/18.5.1095

1. ↑ Kyogoku C, Dijstelbloem HM, Tsuchiya N, Hatta Y, Kato H, Yamaguchi A, Fukazawa T, Jansen MD, Hashimoto H, van de Winkel JG, Kallenberg CG, Tokunaga K. Fcgamma receptor gene polymorphisms in Japanese patients with systemic lupus erythematosus: contribution of FCGR2B to genetic susceptibility. Arthritis Rheum. 2002 May;46(5):1242-54. PMID:12115230 doi:10.1002/art.10257
2. ↑ Willcocks LC, Carr EJ, Niederer HA, Rayner TF, Williams TN, Yang W, Scott JA, Urban BC, Peshu N, Vyse TJ, Lau YL, Lyons PA, Smith KG. A defunctioning polymorphism in FCGR2B is associated with protection against malaria but susceptibility to systemic lupus erythematosus. Proc Natl Acad Sci U S A. 2010 Apr 27;107(17):7881-5. doi:, 10.1073/pnas.0915133107. Epub 2010 Apr 12. PMID:20385827 doi:10.1073/pnas.0915133107