2qqh

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[[Image:2qqh.png|left|200px]]
 
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{{STRUCTURE_2qqh| PDB=2qqh | SCENE= }}
{{STRUCTURE_2qqh| PDB=2qqh | SCENE= }}
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===Structure of C8a-MACPF reveals mechanism of membrane attack in complement immune defense===
===Structure of C8a-MACPF reveals mechanism of membrane attack in complement immune defense===
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{{ABSTRACT_PUBMED_17872444}}
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{{ABSTRACT_PUBMED_17872444}}
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==Disease==
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[[http://www.uniprot.org/uniprot/CO8A_HUMAN CO8A_HUMAN]] Defects in C8A are a cause of complement component 8 deficiency type 1 (C8D1) [MIM:[http://omim.org/entry/613790 613790]]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.
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==Function==
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[[http://www.uniprot.org/uniprot/CO8A_HUMAN 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.<ref>PMID:7440581</ref><ref>PMID:17872444</ref>
==About this Structure==
==About this Structure==
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==Reference==
==Reference==
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<ref group="xtra">PMID:017872444</ref><references group="xtra"/>
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<ref group="xtra">PMID:017872444</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Gros, P.]]
[[Category: Gros, P.]]

Revision as of 06:14, 25 March 2013

Template:STRUCTURE 2qqh

Contents

Structure of C8a-MACPF reveals mechanism of membrane attack in complement immune defense

Template:ABSTRACT PUBMED 17872444

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

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

Reference

  • 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
  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

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