4ihk
From Proteopedia
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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=4ihk FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ihk OCA], [http://pdbe.org/4ihk PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4ihk RCSB], [http://www.ebi.ac.uk/pdbsum/4ihk PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4ihk ProSAT]</span></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=4ihk FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ihk OCA], [http://pdbe.org/4ihk PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4ihk RCSB], [http://www.ebi.ac.uk/pdbsum/4ihk PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4ihk ProSAT]</span></td></tr> | ||
</table> | </table> | ||
| + | <div style="background-color:#fffaf0;"> | ||
| + | == Publication Abstract from PubMed == | ||
| + | Von Willebrand factor A (VWA) domains are versatile protein interaction domains with N and C termini in close proximity placing spatial constraints on overall protein structure. The 1.2 A crystal structures of a collagen VI VWA domain and a disease-causing point mutant show C-terminal extensions that place the N and C termini at opposite ends. This allows a "beads-on-a-string" arrangement of multiple VWA domains as observed for ten N-terminal domains of the collagen VI alpha3 chain. The extension is linked to the core domain by a salt bridge and two hydrophobic patches. Comparison of the wild-type and a muscular dystrophy-associated mutant structure identifies a potential perturbation of a protein interaction interface and indeed, the secretion of mutant collagen VI tetramers is affected. Homology modeling is used to locate a number of disease-associated mutations and analyze their structural impact, which will allow mechanistic analysis of collagen-VI-associated muscular dystrophy phenotypes. | ||
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| + | A structure of a collagen VI VWA domain displays N and C termini at opposite sides of the protein.,Becker AK, Mikolajek H, Paulsson M, Wagener R, Werner JM Structure. 2014 Feb 4;22(2):199-208. doi: 10.1016/j.str.2013.06.028. Epub 2013, Dec 12. PMID:24332716<ref>PMID:24332716</ref> | ||
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| + | From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | ||
| + | </div> | ||
| + | <div class="pdbe-citations 4ihk" style="background-color:#fffaf0;"></div> | ||
==See Also== | ==See Also== | ||
*[[Collagen|Collagen]] | *[[Collagen|Collagen]] | ||
| + | == References == | ||
| + | <references/> | ||
__TOC__ | __TOC__ | ||
</StructureSection> | </StructureSection> | ||
Revision as of 06:56, 5 December 2018
Crystal structure of the Collagen VI alpha3 N5 domain R1061Q
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