3erb

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Template:STRUCTURE 3erb

Contents 1 The Crystal Structure of C2b, a Fragment of Complement Component C2 produced during C3-convertase Formation 2 Disease 3 Function 4 About this Structure 5 Reference

The Crystal Structure of C2b, a Fragment of Complement Component C2 produced during C3-convertase Formation

Template:ABSTRACT PUBMED 19237749

Disease

[CO2_HUMAN] Defects in C2 are the cause of complement component 2 deficiency (C2D) [MIM:217000]. A deficiency of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus erythematosus. Skin and joint manifestations are common and renal disease is relatively rare. Patients with complement component 2 deficiency are also reported to have recurrent or invasive infections.^[1][2]

Function

[CO2_HUMAN] Component C2 which is part of the classical pathway of the complement system is cleaved by activated factor C1 into two fragments: C2b and C2a. C2a, a serine protease, then combines with complement factor 4b to generate the C3 or C5 convertase.

About this Structure

3erb is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

• Krishnan V, Xu Y, Macon K, Volanakis JE, Narayana SV. The structure of C2b, a fragment of complement component C2 produced during C3 convertase formation. Acta Crystallogr D Biol Crystallogr. 2009 Mar;65(Pt 3):266-74. Epub 2009, Feb 20. PMID:19237749 doi:10.1107/S0907444909000389

1. ↑ Wetsel RA, Kulics J, Lokki ML, Kiepiela P, Akama H, Johnson CA, Densen P, Colten HR. Type II human complement C2 deficiency. Allele-specific amino acid substitutions (Ser189 --> Phe; Gly444 --> Arg) cause impaired C2 secretion. J Biol Chem. 1996 Mar 8;271(10):5824-31. PMID:8621452
2. ↑ Zhu ZB, Atkinson TP, Volanakis JE. A novel type II complement C2 deficiency allele in an African-American family. J Immunol. 1998 Jul 15;161(2):578-84. PMID:9670930