2rd7
From Proteopedia
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{{STRUCTURE_2rd7| PDB=2rd7 | SCENE= }} | {{STRUCTURE_2rd7| PDB=2rd7 | SCENE= }} | ||
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===Human Complement Membrane Attack Proteins Share a Common Fold with Bacterial Cytolysins=== | ===Human Complement Membrane Attack Proteins Share a Common Fold with Bacterial Cytolysins=== | ||
+ | ==Disease== | ||
+ | [[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== | ||
+ | [[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> [[http://www.uniprot.org/uniprot/CO8G_HUMAN CO8G_HUMAN]] C8 is a constituent of the membrane attack complex. C8 binds to the C5B-7 complex, forming the C5B-8 complex. C5-B8 binds C9 and acts as a catalyst in the polymerization of C9. The gamma subunit seems to be able to bind retinol. | ||
==About this Structure== | ==About this Structure== | ||
[[2rd7]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2RD7 OCA]. | [[2rd7]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2RD7 OCA]. | ||
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+ | ==Reference== | ||
+ | <references group="xtra"/><references/> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Chruszcz, M.]] | [[Category: Chruszcz, M.]] |
Revision as of 02:51, 25 March 2013
Contents |
Human Complement Membrane Attack Proteins Share a Common Fold with Bacterial Cytolysins
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] [CO8G_HUMAN] C8 is a constituent of the membrane attack complex. C8 binds to the C5B-7 complex, forming the C5B-8 complex. C5-B8 binds C9 and acts as a catalyst in the polymerization of C9. The gamma subunit seems to be able to bind retinol.
About this Structure
2rd7 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- ↑ 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
- ↑ 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
Categories: Homo sapiens | Chruszcz, M. | Lebioda, L. | Lovelace, L L. | Minor, W. | Slade, D J. | Sodetz, J M. | Cleavage on pair of basic residue | Complement alternate pathway | Complement pathway | Cytolysis | Egf-like domain | Glycoprotein | Immune response | Immune system | Innate immunity | Membrane attack complex | Membrane attack system | Secreted