6t8v: Difference between revisions

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<StructureSection load='6t8v' size='340' side='right'caption='[[6t8v]], [[Resolution|resolution]] 2.29&Aring;' scene=''>
<StructureSection load='6t8v' size='340' side='right'caption='[[6t8v]], [[Resolution|resolution]] 2.29&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[6t8v]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6T8V OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6T8V FirstGlance]. <br>
<table><tr><td colspan='2'>[[6t8v]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6T8V OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6T8V FirstGlance]. <br>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=MVK:4-[(2~{S})-1-[(5,7-dimethyl-1~{H}-indol-4-yl)methyl]piperidin-2-yl]benzoic+acid'>MVK</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</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]] 2.29&#8491;</td></tr>
<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>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=MVK:4-[(2~{S})-1-[(5,7-dimethyl-1~{H}-indol-4-yl)methyl]piperidin-2-yl]benzoic+acid'>MVK</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></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=6t8v FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6t8v OCA], [http://pdbe.org/6t8v PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6t8v RCSB], [http://www.ebi.ac.uk/pdbsum/6t8v PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6t8v ProSAT]</span></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=6t8v FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6t8v OCA], [https://pdbe.org/6t8v PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6t8v RCSB], [https://www.ebi.ac.uk/pdbsum/6t8v PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6t8v ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
[[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>
[https://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:[https://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>  
== Function ==
== Function ==
[[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.  
[https://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.
<div style="background-color:#fffaf0;">
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
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__TOC__
__TOC__
</StructureSection>
</StructureSection>
[[Category: Alternative-complement-pathway C3/C5 convertase]]
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Large Structures]]
[[Category: Adams, C]]
[[Category: Adams C]]
[[Category: Adams, C M]]
[[Category: Adams CM]]
[[Category: Anderson, K]]
[[Category: Anderson K]]
[[Category: April, M]]
[[Category: April M]]
[[Category: Argikar, U A]]
[[Category: Argikar UA]]
[[Category: Capparelli, M]]
[[Category: Capparelli M]]
[[Category: Crowley, M]]
[[Category: Crowley M]]
[[Category: Cumin, F]]
[[Category: Cumin F]]
[[Category: Ding, J]]
[[Category: De Erkenez A]]
[[Category: Eder, J]]
[[Category: Ding J]]
[[Category: Ehara, T]]
[[Category: Eder J]]
[[Category: Erbel, P]]
[[Category: Ehara T]]
[[Category: Erkenez, A De]]
[[Category: Erbel P]]
[[Category: Fang, L]]
[[Category: Fang L]]
[[Category: Feifel, R]]
[[Category: Feifel R]]
[[Category: Ferrara, L]]
[[Category: Ferrara L]]
[[Category: Flohr, S]]
[[Category: Flohr S]]
[[Category: Forster, C]]
[[Category: Forster C]]
[[Category: Gerhartz, B]]
[[Category: Gerhartz B]]
[[Category: Gradoux, N]]
[[Category: Gradoux N]]
[[Category: Harrison, R A]]
[[Category: Harrison RA]]
[[Category: Jaffee, B D]]
[[Category: Jaffee BD]]
[[Category: Jendza, K]]
[[Category: Jendza K]]
[[Category: Karki, R G]]
[[Category: Karki RG]]
[[Category: Kawanami, T]]
[[Category: Kawanami T]]
[[Category: Klosowski, D W]]
[[Category: Klosowski DW]]
[[Category: Lee, W]]
[[Category: Lee W]]
[[Category: Liao, S M]]
[[Category: Liao S-M]]
[[Category: Long, D]]
[[Category: Long D]]
[[Category: Maibaum, J]]
[[Category: Mac Sweeney A]]
[[Category: Mainolfi, N]]
[[Category: Maibaum J]]
[[Category: Mogi, M]]
[[Category: Mainolfi N]]
[[Category: Powers, J]]
[[Category: Mogi M]]
[[Category: Prentiss, M]]
[[Category: Powers J]]
[[Category: Ramage, P]]
[[Category: Prentiss M]]
[[Category: Ramqaj, R]]
[[Category: Ramage P]]
[[Category: Schubart, A]]
[[Category: Ramqaj R]]
[[Category: Sedrani, R]]
[[Category: Schubart A]]
[[Category: Sellner, H]]
[[Category: Sedrani R]]
[[Category: Serrano-Wu, M]]
[[Category: Sellner H]]
[[Category: Sirockin, F]]
[[Category: Serrano-Wu M]]
[[Category: Smith, T M]]
[[Category: Sirockin F]]
[[Category: Solovay, C]]
[[Category: Smith TM]]
[[Category: Sweeney, A Mac]]
[[Category: Solovay C]]
[[Category: Valeur, E]]
[[Category: Valeur E]]
[[Category: Vogg, B]]
[[Category: Vogg B]]
[[Category: Wiesmann, C]]
[[Category: Wiesmann C]]
[[Category: Yang, L]]
[[Category: Yang L]]
[[Category: Zhang, C]]
[[Category: Zhang C]]
[[Category: Complement immune]]
[[Category: Hydrolase]]
[[Category: Inhibitor]]

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