6ic3: Difference between revisions
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==AL amyloid fibril from a lambda 1 light chain== | |||
<SX load='6ic3' size='340' side='right' viewer='molstar' caption='[[6ic3]], [[Resolution|resolution]] 3.30Å' scene=''> | |||
== Structural highlights == | |||
<table><tr><td colspan='2'>[[6ic3]] is a 8 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6IC3 OCA]. For a <b>guided tour on the structure components</b> use [http://proteopedia.org/fgij/fg.htm?mol=6IC3 FirstGlance]. <br> | |||
</td></tr><tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://proteopedia.org/fgij/fg.htm?mol=6ic3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6ic3 OCA], [http://pdbe.org/6ic3 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6ic3 RCSB], [http://www.ebi.ac.uk/pdbsum/6ic3 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6ic3 ProSAT]</span></td></tr> | |||
</table> | |||
<div style="background-color:#fffaf0;"> | |||
== Publication Abstract from PubMed == | |||
Amyloid fibrils derived from antibody light chains are key pathogenic agents in systemic AL amyloidosis. They can be deposited in multiple organs but cardiac amyloid is the major risk factor of mortality. Here we report the structure of a lambda1 AL amyloid fibril from an explanted human heart at a resolution of 3.3 A which we determined using cryo-electron microscopy. The fibril core consists of a 91-residue segment presenting an all-beta fold with ten mutagenic changes compared to the germ line. The conformation differs substantially from natively folded light chains: a rotational switch around the intramolecular disulphide bond being the crucial structural rearrangement underlying fibril formation. Our structure provides insight into the mechanism of protein misfolding and the role of patient-specific mutations in pathogenicity. | |||
Cryo-EM structure of a light chain-derived amyloid fibril from a patient with systemic AL amyloidosis.,Radamaker L, Lin YH, Annamalai K, Huhn S, Hegenbart U, Schonland SO, Fritz G, Schmidt M, Fandrich M Nat Commun. 2019 Mar 20;10(1):1103. doi: 10.1038/s41467-019-09032-0. PMID:30894526<ref>PMID:30894526</ref> | |||
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | |||
[[Category: | </div> | ||
<div class="pdbe-citations 6ic3" style="background-color:#fffaf0;"></div> | |||
== References == | |||
<references/> | |||
__TOC__ | |||
</SX> | |||
[[Category: Homo sapiens]] | |||
[[Category: Large Structures]] | |||
[[Category: Faendrich, M]] | |||
[[Category: Fritz, G]] | |||
[[Category: Radamaker, L]] | |||
[[Category: Schmidt, M]] | |||
[[Category: Amyloid fibril]] | |||
[[Category: Antibody]] | |||
[[Category: Beta sheet]] | |||
[[Category: Heart]] | |||
[[Category: Protein fibril]] | |||