4d9r

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Template:STRUCTURE 4d9r

Contents

Inhibiting Alternative Pathway Complement Activation by Targeting the Exosite on Factor D

Template:ABSTRACT PUBMED 22362762

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

4d9r is a 6 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • Katschke KJ, Wu P, Ganesan R, Kelley RF, Mathieu MA, Hass PE, Murray J, Kirchhofer D, Wiesmann C, van Lookeren Campagne M. Inhibiting alternative pathway complement activation by targeting the exosite of factor D. J Biol Chem. 2012 Feb 23. PMID:22362762 doi:10.1074/jbc.M112.345082

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