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3fnv
From Proteopedia
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| - | [[Image:3fnv.png|left|200px]] | ||
| - | + | ==Crystal Structure of Miner1: The Redox-active 2Fe-2S Protein Causative in Wolfram Syndrome 2== | |
| - | + | <StructureSection load='3fnv' size='340' side='right'caption='[[3fnv]], [[Resolution|resolution]] 2.10Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[3fnv]] 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=3FNV OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3FNV FirstGlance]. <br> | |
| - | + | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.1Å</td></tr> | |
| - | + | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=FES:FE2/S2+(INORGANIC)+CLUSTER'>FES</scene></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=3fnv FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3fnv OCA], [https://pdbe.org/3fnv PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3fnv RCSB], [https://www.ebi.ac.uk/pdbsum/3fnv PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3fnv ProSAT]</span></td></tr> |
| - | [[3fnv]] is a 2 chain structure with sequence from [ | + | </table> |
| - | + | == Disease == | |
| - | == | + | [https://www.uniprot.org/uniprot/CISD2_HUMAN CISD2_HUMAN] Defects in CISD2 are the cause of Wolfram syndrome type 2 (WFS2) [MIM:[https://omim.org/entry/604928 604928]. A rare disorder characterized by juvenile-onset insulin-dependent diabetes mellitus with optic atrophy. Other manifestations include diabetes insipidus, sensorineural deafness, dementia, psychiatric illnesses. WFS2 patients additionally show a strong bleeding tendency and gastrointestinal ulceration. Diabetes insipidus may be absent.<ref>PMID:17846994</ref> |
| - | < | + | == Function == |
| + | [https://www.uniprot.org/uniprot/CISD2_HUMAN CISD2_HUMAN] Regulator of autophagy that contributes to antagonize BECN1-mediated cellular autophagy at the endoplasmic reticulum. Participates in the interaction of BCL2 with BECN1 and is required for BCL2-mediated depression of endoplasmic reticulum Ca(2+) stores during autophagy. Contributes to BIK-initiated autophagy, while it is not involved in BIK-dependent activation of caspases. Involved in life span control, probably via its function as regulator of autophagy.<ref>PMID:17846994</ref> <ref>PMID:20010695</ref> | ||
| + | == Evolutionary Conservation == | ||
| + | [[Image:Consurf_key_small.gif|200px|right]] | ||
| + | Check<jmol> | ||
| + | <jmolCheckbox> | ||
| + | <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/fn/3fnv_consurf.spt"</scriptWhenChecked> | ||
| + | <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked> | ||
| + | <text>to colour the structure by Evolutionary Conservation</text> | ||
| + | </jmolCheckbox> | ||
| + | </jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=3fnv ConSurf]. | ||
| + | <div style="clear:both"></div> | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
| - | [[Category: Abresch | + | [[Category: Large Structures]] |
| - | [[Category: Axelrod | + | [[Category: Abresch EC]] |
| - | [[Category: Cohen | + | [[Category: Axelrod HL]] |
| - | [[Category: Conlan | + | [[Category: Cohen AE]] |
| - | [[Category: Jennings | + | [[Category: Conlan AR]] |
| - | [[Category: Nechushtai | + | [[Category: Jennings PA]] |
| - | [[Category: Paddock | + | [[Category: Nechushtai R]] |
| - | [[Category: Yee | + | [[Category: Paddock ML]] |
| - | [[Category: Zuris | + | [[Category: Yee D]] |
| - | + | [[Category: Zuris J]] | |
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Current revision
Crystal Structure of Miner1: The Redox-active 2Fe-2S Protein Causative in Wolfram Syndrome 2
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Categories: Homo sapiens | Large Structures | Abresch EC | Axelrod HL | Cohen AE | Conlan AR | Jennings PA | Nechushtai R | Paddock ML | Yee D | Zuris J

