Factor B structure provides insights into activation of the central protease of the complement system

Nat Struct Mol Biol. 2007 Mar;14(3):224-8. doi: 10.1038/nsmb1210. Epub 2007 Feb 25.

Abstract

Factor B is the central protease of the complement system of immune defense. Here, we present the crystal structure of human factor B at 2.3-A resolution, which reveals how the five-domain proenzyme is kept securely inactive. The canonical activation helix of the Von Willebrand factor A (VWA) domain is displaced by a helix from the preceding domain linker. The two helices conformationally link the scissile-activation peptide and the metal ion-dependent adhesion site required for binding of the ligand C3b. The data suggest that C3b binding displaces the three N-terminal control domains and reshuffles the two central helices. Reshuffling of the helices releases the scissile bond for final proteolytic activation and generates a new interface between the VWA domain and the serine protease domain. This allosteric mechanism is crucial for tight regulation of the complement-amplification step in the immune response.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Catalytic Domain
  • Complement C3-C5 Convertases / chemistry
  • Complement Factor B / chemistry*
  • Complement Factor B / metabolism*
  • Complement System Proteins / immunology*
  • Crystallography, X-Ray
  • Enzyme Activation
  • Humans
  • Models, Molecular
  • Protein Structure, Secondary
  • Protein Structure, Tertiary
  • Regulatory Sequences, Nucleic Acid / genetics
  • Structure-Activity Relationship
  • von Willebrand Factor / chemistry

Substances

  • von Willebrand Factor
  • Complement System Proteins
  • Complement C3-C5 Convertases
  • Complement Factor B

Associated data

  • PDB/2OK5