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| ==Human Adenovirus type 21 knob in complex with domains SCR1 and SCR2 of CD46 (membrane cofactor protein, MCP)== | | ==Human Adenovirus type 21 knob in complex with domains SCR1 and SCR2 of CD46 (membrane cofactor protein, MCP)== |
| <StructureSection load='3l89' size='340' side='right' caption='[[3l89]], [[Resolution|resolution]] 3.50Å' scene=''> | | <StructureSection load='3l89' size='340' side='right'caption='[[3l89]], [[Resolution|resolution]] 3.50Å' scene=''> |
| == Structural highlights == | | == Structural highlights == |
| <table><tr><td colspan='2'>[[3l89]] is a 24 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human] and [http://en.wikipedia.org/wiki/Human_adenovirus_21 Human adenovirus 21]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3L89 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3L89 FirstGlance]. <br> | | <table><tr><td colspan='2'>[[3l89]] is a 24 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [https://en.wikipedia.org/wiki/Human_adenovirus_21 Human adenovirus 21]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3L89 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3L89 FirstGlance]. <br> |
| </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene></td></tr> | | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 3.5Å</td></tr> |
| <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2o39|2o39]], [[1ckl|1ckl]], [[3l88|3l88]]</td></tr>
| | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene></td></tr> |
| <tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">32608, L5 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=32608 Human adenovirus 21]), 9606, CD46, MCP, MIC10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr> | | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3l89 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3l89 OCA], [https://pdbe.org/3l89 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3l89 RCSB], [https://www.ebi.ac.uk/pdbsum/3l89 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3l89 ProSAT]</span></td></tr> |
| <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=3l89 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3l89 OCA], [http://pdbe.org/3l89 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=3l89 RCSB], [http://www.ebi.ac.uk/pdbsum/3l89 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=3l89 ProSAT]</span></td></tr> | |
| </table> | | </table> |
| == Disease ==
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| [[http://www.uniprot.org/uniprot/MCP_HUMAN MCP_HUMAN]] Defects in CD46 are a cause of susceptibility to hemolytic uremic syndrome atypical type 2 (AHUS2) [MIM:[http://omim.org/entry/612922 612922]]. An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Note=Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype. Patients with CD46 mutations seem to have an overall better prognosis compared to patients carrying CFH mutations.<ref>PMID:14615110</ref> <ref>PMID:14566051</ref> <ref>PMID:16621965</ref> <ref>PMID:16386793</ref> <ref>PMID:20513133</ref>
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| == Function == | | == Function == |
| [[http://www.uniprot.org/uniprot/MCP_HUMAN MCP_HUMAN]] Acts as a cofactor for complement factor I, a serine protease which protects autologous cells against complement-mediated injury by cleaving C3b and C4b deposited on host tissue. May be involved in the fusion of the spermatozoa with the oocyte during fertilization. Also acts as a costimulatory factor for T-cells which induces the differentiation of CD4+ into T-regulatory 1 cells. T-regulatory 1 cells suppress immune responses by secreting interleukin-10, and therefore are thought to prevent autoimmunity. A number of viral and bacterial pathogens seem to exploit this property and directly induce an immunosuppressive phenotype in T-cells by binding to CD46.<ref>PMID:10843656</ref> <ref>PMID:12540904</ref> | | [https://www.uniprot.org/uniprot/Q2KS96_9ADEN Q2KS96_9ADEN] |
| == Evolutionary Conservation == | | == Evolutionary Conservation == |
| [[Image:Consurf_key_small.gif|200px|right]] | | [[Image:Consurf_key_small.gif|200px|right]] |
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| __TOC__ | | __TOC__ |
| </StructureSection> | | </StructureSection> |
| [[Category: Human]] | | [[Category: Homo sapiens]] |
| [[Category: Human adenovirus 21]] | | [[Category: Human adenovirus 21]] |
| [[Category: Cupelli, K]] | | [[Category: Large Structures]] |
| [[Category: Stehle, T]] | | [[Category: Cupelli K]] |
| [[Category: Adenovirus]] | | [[Category: Stehle T]] |
| [[Category: Ccp]]
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| [[Category: Complement control protein]]
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| [[Category: Complement pathway]]
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| [[Category: Fiber knob]]
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| [[Category: Glycoprotein]]
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| [[Category: Host-virus interaction]]
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| [[Category: Immune response]]
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| [[Category: Innate immunity]]
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| [[Category: Mcp]]
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| [[Category: Membrane cofactor protein]]
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| [[Category: Scr]]
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| [[Category: Short consensus repeat]]
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| [[Category: Sushi2]]
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| [[Category: Viral protein]]
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| [[Category: Viral protein-protein binding complex]]
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| [[Category: Virus receptor complex]]
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