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1kun

From Proteopedia

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{{Seed}}
 
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[[Image:1kun.png|left|200px]]
 
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==SOLUTION STRUCTURE OF THE HUMAN ALPHA3-CHAIN TYPE VI COLLAGEN C-TERMINAL KUNITZ DOMAIN, NMR, 20 STRUCTURES==
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The line below this paragraph, containing "STRUCTURE_1kun", creates the "Structure Box" on the page.
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<StructureSection load='1kun' size='340' side='right'caption='[[1kun]]' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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<table><tr><td colspan='2'>[[1kun]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1KUN OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1KUN FirstGlance]. <br>
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or leave the SCENE parameter empty for the default display.
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1kun FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1kun OCA], [https://pdbe.org/1kun PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1kun RCSB], [https://www.ebi.ac.uk/pdbsum/1kun PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1kun ProSAT]</span></td></tr>
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{{STRUCTURE_1kun| PDB=1kun | SCENE= }}
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/CO6A3_HUMAN CO6A3_HUMAN] Defects in COL6A3 are a cause of Bethlem myopathy (BM) [MIM:[https://omim.org/entry/158810 158810]. BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles.<ref>PMID:11992252</ref> <ref>PMID:9536084</ref> <ref>PMID:10399756</ref> <ref>PMID:15689448</ref> <ref>PMID:17886299</ref> Defects in COL6A3 are a cause of Ullrich congenital muscular dystrophy (UCMD) [MIM:[https://omim.org/entry/254090 254090]; also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy.<ref>PMID:11992252</ref> <ref>PMID:15689448</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/CO6A3_HUMAN CO6A3_HUMAN] Collagen VI acts as a cell-binding protein.
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/ku/1kun_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</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=1kun ConSurf].
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<div style="clear:both"></div>
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===SOLUTION STRUCTURE OF THE HUMAN ALPHA3-CHAIN TYPE VI COLLAGEN C-TERMINAL KUNITZ DOMAIN, NMR, 20 STRUCTURES===
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==See Also==
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*[[Collagen 3D structures|Collagen 3D structures]]
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== References ==
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<references/>
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The line below this paragraph, {{ABSTRACT_PUBMED_9265624}}, adds the Publication Abstract to the page
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__TOC__
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(as it appears on PubMed at http://www.pubmed.gov), where 9265624 is the PubMed ID number.
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</StructureSection>
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-->
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{{ABSTRACT_PUBMED_9265624}}
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==About this Structure==
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1KUN is a 1 chain structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1KUN OCA].
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==Reference==
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<ref group="xtra">PMID:9265624</ref><references group="xtra"/>
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Bjorn, S.]]
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[[Category: Large Structures]]
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[[Category: James, T L.]]
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[[Category: Bjorn S]]
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[[Category: Led, J J.]]
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[[Category: James TL]]
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[[Category: Norris, K.]]
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[[Category: Led JJ]]
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[[Category: Olsen, O.]]
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[[Category: Norris K]]
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[[Category: Petersen, L.]]
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[[Category: Olsen O]]
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[[Category: Sorensen, M D.]]
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[[Category: Petersen L]]
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[[Category: Collagen type vi fragment]]
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[[Category: Sorensen MD]]
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[[Category: Connective tissue]]
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[[Category: Extracellular matrix]]
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[[Category: Kunitz-type domain]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Feb 17 01:57:21 2009''
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Current revision

SOLUTION STRUCTURE OF THE HUMAN ALPHA3-CHAIN TYPE VI COLLAGEN C-TERMINAL KUNITZ DOMAIN, NMR, 20 STRUCTURES

PDB ID 1kun

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