5jtw: Difference between revisions

<table><tr><td colspan='2'>[[5jtw]] is a 6 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. This structure supersedes the now removed PDB entry [http://oca.weizmann.ac.il/oca-bin/send-pdb?obs=1&id=4xam 4xam]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5JTW OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=5JTW FirstGlance]. <br>

<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=5jtw FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5jtw OCA], [https://pdbe.org/5jtw PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=5jtw RCSB], [https://www.ebi.ac.uk/pdbsum/5jtw PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=5jtw ProSAT]</span></td></tr>

[https://www.uniprot.org/uniprot/CO4A_HUMAN CO4A_HUMAN] Defects in C4A are the cause of complement component 4A deficiency (C4AD) [MIM:[https://omim.org/entry/614380 614380]. A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus with or without associated glomerulonephritis.<ref>PMID:8473511</ref>  Defects in C4A are a cause of susceptibility to systemic lupus erythematosus (SLE) [MIM:[https://omim.org/entry/152700 152700]. A chronic, inflammatory and often febrile multisystemic disorder of connective tissue. It affects principally the skin, joints, kidneys and serosal membranes. It is thought to represent a failure of the regulatory mechanisms of the autoimmune system. Note=Interindividual copy-number variation (CNV) of complement component C4 and associated polymorphisms result in different susceptibilities to SLE. The risk of SLE susceptibility has been shown to be significantly increased among subjects with only two copies of total C4. A high copy number is a protective factor against SLE.<ref>PMID:17503323</ref>  

[https://www.uniprot.org/uniprot/CO4A_HUMAN CO4A_HUMAN] C4 plays a central role in the activation of the classical pathway of the complement system. It is processed by activated C1 which removes from the alpha chain the C4a anaphylatoxin. The remaining alpha chain fragment C4b is the major activation product and is an essential subunit of the C3 convertase (C4b2a) and the C5 convertase (C3bC4b2a) enzymes of the classical complement pathway.  Derived from proteolytic degradation of complement C4, C4a anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes.

[[Category: Andersen GR]]

[[Category: Croll TI]]