2xw9
From Proteopedia
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{{STRUCTURE_2xw9| PDB=2xw9 | SCENE= }} | {{STRUCTURE_2xw9| PDB=2xw9 | SCENE= }} | ||
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===Crystal Structure of Complement Factor D mutant S183A=== | ===Crystal Structure of Complement Factor D mutant S183A=== | ||
+ | {{ABSTRACT_PUBMED_21205667}} | ||
+ | ==Disease== | ||
+ | [[http://www.uniprot.org/uniprot/CFAD_HUMAN CFAD_HUMAN]] Defects in CFD are the cause of complement factor D deficiency (CFDD) [MIM:[http://omim.org/entry/613912 613912]]. CFDD is an immunologic disorder characterized by increased susceptibility to bacterial infections, particularly Neisseria infections, due to a defect in the alternative complement pathway. | ||
- | + | ==Function== | |
- | + | [[http://www.uniprot.org/uniprot/CFAD_HUMAN CFAD_HUMAN]] Factor D cleaves factor B when the latter is complexed with factor C3b, activating the C3bbb complex, which then becomes the C3 convertase of the alternate pathway. Its function is homologous to that of C1s in the classical pathway. | |
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==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
- | <ref group="xtra">PMID:021205667</ref><references group="xtra"/> | + | <ref group="xtra">PMID:021205667</ref><references group="xtra"/><references/> |
[[Category: Complement factor D]] | [[Category: Complement factor D]] | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] |
Revision as of 06:38, 25 March 2013
Contents |
Crystal Structure of Complement Factor D mutant S183A
Template:ABSTRACT PUBMED 21205667
Disease
[CFAD_HUMAN] Defects in CFD are the cause of complement factor D deficiency (CFDD) [MIM:613912]. CFDD is an immunologic disorder characterized by increased susceptibility to bacterial infections, particularly Neisseria infections, due to a defect in the alternative complement pathway.
Function
[CFAD_HUMAN] Factor D cleaves factor B when the latter is complexed with factor C3b, activating the C3bbb complex, which then becomes the C3 convertase of the alternate pathway. Its function is homologous to that of C1s in the classical pathway.
About this Structure
2xw9 is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Forneris F, Ricklin D, Wu J, Tzekou A, Wallace RS, Lambris JD, Gros P. Structures of C3b in complex with factors B and D give insight into complement convertase formation. Science. 2010 Dec 24;330(6012):1816-20. PMID:21205667 doi:10.1126/science.1195821