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| - | | + | #REDIRECT [[6rav]] This PDB entry is obsolete and replaced by 6rav |
| - | ==Complement factor B protease domain in complex with the reversible inhibitor 4-((2S,4S)-4-ethoxy-1-((5-methoxy-7-methyl-1H-indol-4-yl)methyl)piperidin-2-yl)benzoic acid==
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| - | <StructureSection load='6qsv' size='340' side='right'caption='[[6qsv]], [[Resolution|resolution]] 1.70Å' scene=''>
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| - | == Structural highlights ==
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| - | <table><tr><td colspan='2'>[[6qsv]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6QSV OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6QSV FirstGlance]. <br>
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| - | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=JGQ:4-[(2~{S},4~{S})-4-ethoxy-1-[(5-methoxy-7-methyl-1~{H}-indol-4-yl)methyl]piperidin-2-yl]benzoic+acid'>JGQ</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></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/Alternative-complement-pathway_C3/C5_convertase Alternative-complement-pathway C3/C5 convertase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.4.21.47 3.4.21.47] </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=6qsv FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6qsv OCA], [http://pdbe.org/6qsv PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6qsv RCSB], [http://www.ebi.ac.uk/pdbsum/6qsv PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6qsv ProSAT]</span></td></tr>
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| - | </table>
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| - | == Disease ==
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| - | [[http://www.uniprot.org/uniprot/CFAB_HUMAN CFAB_HUMAN]] Defects in CFB are a cause of susceptibility to hemolytic uremic syndrome atypical type 4 (AHUS4) [MIM:[http://omim.org/entry/612924 612924]]. 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.<ref>PMID:17182750</ref> <ref>PMID:20513133</ref>
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| - | == Function ==
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| - | [[http://www.uniprot.org/uniprot/CFAB_HUMAN CFAB_HUMAN]] Factor B which is part of the alternate pathway of the complement system is cleaved by factor D into 2 fragments: Ba and Bb. Bb, a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase. It has also been implicated in proliferation and differentiation of preactivated B-lymphocytes, rapid spreading of peripheral blood monocytes, stimulation of lymphocyte blastogenesis and lysis of erythrocytes. Ba inhibits the proliferation of preactivated B-lymphocytes.
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| - | == References ==
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| - | <references/>
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| - | __TOC__
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| - | </StructureSection>
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| - | [[Category: Alternative-complement-pathway C3/C5 convertase]]
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| - | [[Category: Large Structures]]
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| - | [[Category: Adams, C M]]
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| - | [[Category: Anderson, K]]
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| - | [[Category: Argikar, U]]
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| - | [[Category: Crowley, M]]
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| - | [[Category: Cumin, F]]
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| - | [[Category: Eder, J]]
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| - | [[Category: Ehara, T]]
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| - | [[Category: Erbel, P]]
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| - | [[Category: Flohr, S]]
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| - | [[Category: Gerhartz, B]]
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| - | [[Category: Harrison, R]]
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| - | [[Category: Hughes, N]]
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| - | [[Category: Jaffee, B]]
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| - | [[Category: Karki, R]]
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| - | [[Category: Maibaum, J]]
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| - | [[Category: Mainolfi, N]]
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| - | [[Category: Mogi, M]]
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| - | [[Category: Sedrani, R]]
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| - | [[Category: Sellner, H]]
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| - | [[Category: Sirockin, F]]
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| - | [[Category: Smith, T M]]
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| - | [[Category: Sweeney, A Mac]]
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| - | [[Category: Valeur, E]]
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| - | [[Category: Wiesmann, C]]
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| - | [[Category: C3 convertase]]
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| - | [[Category: Complement]]
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| - | [[Category: Hydrolase]]
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| - | [[Category: Immune]]
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| - | [[Category: Inhibitor]]
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