6h03: Difference between revisions

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-'''Unreleased structure'''
+{{Large structure}}
+==OPEN CONFORMATION OF THE MEMBRANE ATTACK COMPLEX==
-The entry 6h03 is ON HOLD  until Paper Publication
+<StructureSection load='6h03' size='340' side='right' caption='[[6h03]], [[Resolution|resolution]] 5.60&Aring;' scene=''>
+== Structural highlights ==
-Authors:
+<table><tr><td colspan='2'>[[6h03]] is a 24 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6H03 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6H03 FirstGlance]. <br>
+</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene></td></tr>
-Description:
+<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[6h04|6h04]]</td></tr>
-[[Category: Unreleased Structures]]
+<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6h03 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6h03 OCA], [http://pdbe.org/6h03 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6h03 RCSB], [http://www.ebi.ac.uk/pdbsum/6h03 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6h03 ProSAT]</span></td></tr>
+</table>
+{{Large structure}}
+== Disease ==
+[[http://www.uniprot.org/uniprot/CO6_HUMAN CO6_HUMAN]] Defects in C6 are the cause of complement component 6 deficiency (C6D) [MIM:[http://omim.org/entry/612446 612446]]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. [[http://www.uniprot.org/uniprot/CO7_HUMAN CO7_HUMAN]] Immunodeficiency due to a late component of complement deficiency. Disease susceptibility is associated with variations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/CO9_HUMAN CO9_HUMAN]] Age-related macular degeneration;Immunodeficiency due to a late component of complements deficiency. Disease susceptibility is associated with variations affecting the gene represented in this entry.  Disease susceptibility is associated with variations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/CO8B_HUMAN CO8B_HUMAN]] Immunodeficiency due to a late component of complements deficiency. Disease susceptibility is associated with variations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/CO5_HUMAN CO5_HUMAN]] Defects in C5 are the cause of complement component 5 deficiency (C5D) [MIM:[http://omim.org/entry/609536 609536]]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.  Note=An association study of C5 haplotypes and genotypes in individuals with chronic hepatitis C virus infection shows that individuals homozygous for the C5_1 haplotype have a significantly higher stage of liver fibrosis than individuals carrying at least 1 other allele (PubMed:15995705). [[http://www.uniprot.org/uniprot/CO8A_HUMAN CO8A_HUMAN]] Defects in C8A are a cause of complement component 8 deficiency type 1 (C8D1) [MIM:[http://omim.org/entry/613790 613790]]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.
+== Function ==
+[[http://www.uniprot.org/uniprot/CO6_HUMAN CO6_HUMAN]] Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. [[http://www.uniprot.org/uniprot/CO7_HUMAN CO7_HUMAN]] Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. C7 serves as a membrane anchor. [[http://www.uniprot.org/uniprot/CO9_HUMAN CO9_HUMAN]] Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. C9 is the pore-forming subunit of the MAC. [[http://www.uniprot.org/uniprot/CO8B_HUMAN CO8B_HUMAN]] Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. [[http://www.uniprot.org/uniprot/CO5_HUMAN CO5_HUMAN]] Activation of C5 by a C5 convertase initiates the spontaneous assembly of the late complement components, C5-C9, into the membrane attack complex. C5b has a transient binding site for C6. The C5b-C6 complex is the foundation upon which the lytic complex is assembled.  Derived from proteolytic degradation of complement C5, C5 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. C5a also stimulates the locomotion of polymorphonuclear leukocytes (chemokinesis) and direct their migration toward sites of inflammation (chemotaxis). [[http://www.uniprot.org/uniprot/CO8G_HUMAN CO8G_HUMAN]] C8 is a constituent of the membrane attack complex. C8 binds to the C5B-7 complex, forming the C5B-8 complex. C5-B8 binds C9 and acts as a catalyst in the polymerization of C9. The gamma subunit seems to be able to bind retinol. [[http://www.uniprot.org/uniprot/CO8A_HUMAN CO8A_HUMAN]] Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. C8A inserts into the target membrane, but does not form pores by itself.<ref>PMID:7440581</ref> <ref>PMID:17872444</ref>
+== References ==
+<references/>
+__TOC__
+</StructureSection>
+[[Category: Homo sapiens]]
+[[Category: Boyd, C M]]
+[[Category: Bubeck, D]]
+[[Category: Gardner, S]]
+[[Category: Joseph, A P]]
+[[Category: Menny, A]]
+[[Category: Serna, M]]
+[[Category: Topf, M]]
+[[Category: C5b9]]
+[[Category: Immune system]]