4e0s
From Proteopedia
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{{STRUCTURE_4e0s| PDB=4e0s | SCENE= }} | {{STRUCTURE_4e0s| PDB=4e0s | SCENE= }} | ||
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===Crystal Structure of C5b-6=== | ===Crystal Structure of C5b-6=== | ||
| + | {{ABSTRACT_PUBMED_22500023}} | ||
| - | + | ==Disease== | |
| + | [[http://www.uniprot.org/uniprot/CO5_HUMAN CO5_HUMAN]] Defects in C5 are the cause of complement component 5 deficiency (C5D) [MIM:[http://omim.org/entry/609536 609536]]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. Note=An association study of C5 haplotypes and genotypes in individuals with chronic hepatitis C virus infection shows that individuals homozygous for the C5_1 haplotype have a significantly higher stage of liver fibrosis than individuals carrying at least 1 other allele (PubMed:15995705). [[http://www.uniprot.org/uniprot/CO6_HUMAN CO6_HUMAN]] Defects in C6 are the cause of complement component 6 deficiency (C6D) [MIM:[http://omim.org/entry/612446 612446]]. 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/CO5_HUMAN CO5_HUMAN]] Activation of C5 by a C5 convertase initiates the spontaneous assembly of the late complement components, C5-C9, into the membrane attack complex. C5b has a transient binding site for C6. The C5b-C6 complex is the foundation upon which the lytic complex is assembled. Derived from proteolytic degradation of complement C5, C5 anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes. C5a also stimulates the locomotion of polymorphonuclear leukocytes (chemokinesis) and direct their migration toward sites of inflammation (chemotaxis). [[http://www.uniprot.org/uniprot/CO6_HUMAN CO6_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. | ||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
| - | <ref group="xtra">PMID:022500023</ref><references group="xtra"/> | + | <ref group="xtra">PMID:022500023</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Aleshin, A E.]] | [[Category: Aleshin, A E.]] | ||
Revision as of 04:20, 25 March 2013
Contents |
Crystal Structure of C5b-6
Template:ABSTRACT PUBMED 22500023
Disease
[CO5_HUMAN] Defects in C5 are the cause of complement component 5 deficiency (C5D) [MIM:609536]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. Note=An association study of C5 haplotypes and genotypes in individuals with chronic hepatitis C virus infection shows that individuals homozygous for the C5_1 haplotype have a significantly higher stage of liver fibrosis than individuals carrying at least 1 other allele (PubMed:15995705). [CO6_HUMAN] Defects in C6 are the cause of complement component 6 deficiency (C6D) [MIM:612446]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.
Function
[CO5_HUMAN] Activation of C5 by a C5 convertase initiates the spontaneous assembly of the late complement components, C5-C9, into the membrane attack complex. C5b has a transient binding site for C6. The C5b-C6 complex is the foundation upon which the lytic complex is assembled. Derived from proteolytic degradation of complement C5, C5 anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes. C5a also stimulates the locomotion of polymorphonuclear leukocytes (chemokinesis) and direct their migration toward sites of inflammation (chemotaxis). [CO6_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.
About this Structure
4e0s is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Aleshin AE, Discipio RG, Stec B, Liddington RC. Crystal structure of C5b-6 suggests a structural basis for priming the assembly of the Membrane Attack Complex (MAC). J Biol Chem. 2012 Apr 12. PMID:22500023 doi:10.1074/jbc.M112.361121
