2i6q

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== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[2i6q]] is a 1 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=2I6Q OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2I6Q FirstGlance]. <br>
<table><tr><td colspan='2'>[[2i6q]] is a 1 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=2I6Q OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2I6Q FirstGlance]. <br>
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</td></tr><tr><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=MLI:MALONATE+ION'>MLI</scene>, <scene name='pdbligand=MN:MANGANESE+(II)+ION'>MN</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=FUC:ALPHA-L-FUCOSE'>FUC</scene><br>
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</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=MLI:MALONATE+ION'>MLI</scene>, <scene name='pdbligand=MN:MANGANESE+(II)+ION'>MN</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=FUC:ALPHA-L-FUCOSE'>FUC</scene></td></tr>
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<tr><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2i6s|2i6s]]</td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2i6s|2i6s]]</td></tr>
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<tr><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">C2a ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">C2a ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
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<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Classical-complement-pathway_C3/C5_convertase Classical-complement-pathway C3/C5 convertase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.4.21.43 3.4.21.43] </span></td></tr>
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<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Classical-complement-pathway_C3/C5_convertase Classical-complement-pathway C3/C5 convertase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.4.21.43 3.4.21.43] </span></td></tr>
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<tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2i6q FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2i6q OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2i6q RCSB], [http://www.ebi.ac.uk/pdbsum/2i6q PDBsum]</span></td></tr>
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<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=2i6q FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2i6q OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2i6q RCSB], [http://www.ebi.ac.uk/pdbsum/2i6q PDBsum]</span></td></tr>
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<table>
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</table>
== Disease ==
== Disease ==
[[http://www.uniprot.org/uniprot/CO2_HUMAN CO2_HUMAN]] Defects in C2 are the cause of complement component 2 deficiency (C2D) [MIM:[http://omim.org/entry/217000 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.<ref>PMID:8621452</ref> <ref>PMID:9670930</ref>
[[http://www.uniprot.org/uniprot/CO2_HUMAN CO2_HUMAN]] Defects in C2 are the cause of complement component 2 deficiency (C2D) [MIM:[http://omim.org/entry/217000 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.<ref>PMID:8621452</ref> <ref>PMID:9670930</ref>
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[[Category: Classical-complement-pathway C3/C5 convertase]]
[[Category: Classical-complement-pathway C3/C5 convertase]]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Daha, M R.]]
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[[Category: Daha, M R]]
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[[Category: Gros, P.]]
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[[Category: Gros, P]]
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[[Category: Hemrika, W.]]
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[[Category: Hemrika, W]]
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[[Category: Huizinga, E G.]]
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[[Category: Huizinga, E G]]
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[[Category: Milder, F J.]]
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[[Category: Milder, F J]]
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[[Category: Raaijmakers, H C.A.]]
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[[Category: Raaijmakers, H C.A]]
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[[Category: Romijn, R A.]]
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[[Category: Romijn, R A]]
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[[Category: Roos, A.]]
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[[Category: Roos, A]]
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[[Category: Schouten, A.]]
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[[Category: Schouten, A]]
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[[Category: Vandeputte, D A.A.]]
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[[Category: Vandeputte, D A.A]]
[[Category: Hydrolase]]
[[Category: Hydrolase]]
[[Category: Serine protease domain]]
[[Category: Serine protease domain]]
[[Category: Von willebrand factor-a domain]]
[[Category: Von willebrand factor-a domain]]

Revision as of 08:03, 16 January 2015

Complement component C2a

2i6q, resolution 2.10Å

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