2qos

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Template:STRUCTURE 2qos

Contents 1 Crystal structure of complement protein C8 in complex with a peptide containing the C8 binding site on C8 2 Disease 3 Function 4 About this Structure 5 Reference

Crystal structure of complement protein C8 in complex with a peptide containing the C8 binding site on C8

Template:ABSTRACT PUBMED 17692377

Disease

[CO8A_HUMAN] Defects in C8A are a cause of complement component 8 deficiency type 1 (C8D1) [MIM:613790]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.

Function

[CO8A_HUMAN] Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. C8A inserts into the target membrane, but does not form pores by itself.^[1][2]

About this Structure

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

Reference

• Lovelace LL, Chiswell B, Slade DJ, Sodetz JM, Lebioda L. Crystal structure of complement protein C8gamma in complex with a peptide containing the C8gamma binding site on C8alpha: implications for C8gamma ligand binding. Mol Immunol. 2008 Feb;45(3):750-6. Epub 2007 Aug 9. PMID:17692377 doi:10.1016/j.molimm.2007.06.359

1. ↑ Steckel EW, York RG, Monahan JB, Sodetz JM. The eighth component of human complement. Purification and physicochemical characterization of its unusual subunit structure. J Biol Chem. 1980 Dec 25;255(24):11997-2005. PMID:7440581
2. ↑ Hadders MA, Beringer DX, Gros P. Structure of C8alpha-MACPF reveals mechanism of membrane attack in complement immune defense. Science. 2007 Sep 14;317(5844):1552-4. PMID:17872444 doi:317/5844/1552