6t8v: Difference between revisions
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<StructureSection load='6t8v' size='340' side='right'caption='[[6t8v]], [[Resolution|resolution]] 2.29Å' scene=''> | <StructureSection load='6t8v' size='340' side='right'caption='[[6t8v]], [[Resolution|resolution]] 2.29Å' 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 [ | <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><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.29Å</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'>[ | <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 == | ||
[ | [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 == | ||
[ | [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: | [[Category: Homo sapiens]] | ||
[[Category: Large Structures]] | [[Category: Large Structures]] | ||
[[Category: Adams | [[Category: Adams C]] | ||
[[Category: Adams | [[Category: Adams CM]] | ||
[[Category: Anderson | [[Category: Anderson K]] | ||
[[Category: April | [[Category: April M]] | ||
[[Category: Argikar | [[Category: Argikar UA]] | ||
[[Category: Capparelli | [[Category: Capparelli M]] | ||
[[Category: Crowley | [[Category: Crowley M]] | ||
[[Category: Cumin | [[Category: Cumin F]] | ||
[[Category: Ding | [[Category: De Erkenez A]] | ||
[[Category: Eder | [[Category: Ding J]] | ||
[[Category: Ehara | [[Category: Eder J]] | ||
[[Category: Erbel | [[Category: Ehara T]] | ||
[[Category: Erbel P]] | |||
[[Category: Fang | [[Category: Fang L]] | ||
[[Category: Feifel | [[Category: Feifel R]] | ||
[[Category: Ferrara | [[Category: Ferrara L]] | ||
[[Category: Flohr | [[Category: Flohr S]] | ||
[[Category: Forster | [[Category: Forster C]] | ||
[[Category: Gerhartz | [[Category: Gerhartz B]] | ||
[[Category: Gradoux | [[Category: Gradoux N]] | ||
[[Category: Harrison | [[Category: Harrison RA]] | ||
[[Category: Jaffee | [[Category: Jaffee BD]] | ||
[[Category: Jendza | [[Category: Jendza K]] | ||
[[Category: Karki | [[Category: Karki RG]] | ||
[[Category: Kawanami | [[Category: Kawanami T]] | ||
[[Category: Klosowski | [[Category: Klosowski DW]] | ||
[[Category: Lee | [[Category: Lee W]] | ||
[[Category: Liao | [[Category: Liao S-M]] | ||
[[Category: Long | [[Category: Long D]] | ||
[[Category: Maibaum | [[Category: Mac Sweeney A]] | ||
[[Category: Mainolfi | [[Category: Maibaum J]] | ||
[[Category: Mogi | [[Category: Mainolfi N]] | ||
[[Category: Powers | [[Category: Mogi M]] | ||
[[Category: Prentiss | [[Category: Powers J]] | ||
[[Category: Ramage | [[Category: Prentiss M]] | ||
[[Category: Ramqaj | [[Category: Ramage P]] | ||
[[Category: Schubart | [[Category: Ramqaj R]] | ||
[[Category: Sedrani | [[Category: Schubart A]] | ||
[[Category: Sellner | [[Category: Sedrani R]] | ||
[[Category: Serrano-Wu | [[Category: Sellner H]] | ||
[[Category: Sirockin | [[Category: Serrano-Wu M]] | ||
[[Category: Smith | [[Category: Sirockin F]] | ||
[[Category: Solovay | [[Category: Smith TM]] | ||
[[Category: Solovay C]] | |||
[[Category: Valeur | [[Category: Valeur E]] | ||
[[Category: Vogg | [[Category: Vogg B]] | ||
[[Category: Wiesmann | [[Category: Wiesmann C]] | ||
[[Category: Yang | [[Category: Yang L]] | ||
[[Category: Zhang | [[Category: Zhang C]] | ||