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PDBsum entry 2wii
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Immune system
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PDB id
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2wii
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Contents |
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640 a.a.
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896 a.a.
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245 a.a.
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References listed in PDB file
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Key reference
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Title
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Structure of complement fragment c3b-Factor h and implications for host protection by complement regulators.
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Authors
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J.Wu,
Y.Q.Wu,
D.Ricklin,
B.J.Janssen,
J.D.Lambris,
P.Gros.
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Ref.
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Nat Immunol, 2009,
10,
728-733.
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PubMed id
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Abstract
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Factor H (FH) is an abundant regulator of complement activation and protects
host cells from self-attack by complement. Here we provide insight into the
regulatory activity of FH by solving the crystal structure of the first four
domains of FH in complex with its target, complement fragment C3b. FH interacted
with multiple domains of C3b, covering a large, extended surface area. The
structure indicated that FH destabilizes the C3 convertase by competition and
electrostatic repulsion and that FH enables proteolytic degradation of C3b by
providing a binding platform for protease factor I while stabilizing the overall
domain arrangement of C3b. Our results offer general models for complement
regulation and provide structural explanations for disease-related mutations in
the genes encoding both FH and C3b.
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