4cbn

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Template:STRUCTURE 4cbn

Contents

Crystal structure of Complement Factor D mutant R202A after conventional refinement

Template:ABSTRACT PUBMED 24598742

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

4cbn is a 2 chain structure. Full crystallographic information is available from OCA.

Reference

  • Forneris F, Burnley BT, Gros P. Ensemble refinement shows conformational flexibility in crystal structures of human complement factor D. Acta Crystallogr D Biol Crystallogr. 2014 Mar;70(Pt 3):733-43. doi:, 10.1107/S1399004713032549. Epub 2014 Feb 15. PMID:24598742 doi:http://dx.doi.org/10.1107/S1399004713032549

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