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| | ==Cryo-EM structure of cardiac amyloid fibrils from an immunoglobulin light chain (AL) amyloidosis patient.== | | ==Cryo-EM structure of cardiac amyloid fibrils from an immunoglobulin light chain (AL) amyloidosis patient.== |
| - | <StructureSection load='6hud' size='340' side='right'caption='[[6hud]], [[Resolution|resolution]] 4.00Å' scene=''> | + | <SX load='6hud' size='340' side='right' viewer='molstar' caption='[[6hud]], [[Resolution|resolution]] 4.00Å' scene=''> |
| | == Structural highlights == | | == Structural highlights == |
| - | <table><tr><td colspan='2'>[[6hud]] is a 5 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=6HUD OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6HUD FirstGlance]. <br> | + | <table><tr><td colspan='2'>[[6hud]] is a 5 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=6HUD OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6HUD FirstGlance]. <br> |
| - | </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=6hud FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6hud OCA], [http://pdbe.org/6hud PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6hud RCSB], [http://www.ebi.ac.uk/pdbsum/6hud PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6hud ProSAT]</span></td></tr> | + | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 4Å</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=6hud FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6hud OCA], [https://pdbe.org/6hud PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6hud RCSB], [https://www.ebi.ac.uk/pdbsum/6hud PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6hud ProSAT]</span></td></tr> |
| | </table> | | </table> |
| | <div style="background-color:#fffaf0;"> | | <div style="background-color:#fffaf0;"> |
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| | </div> | | </div> |
| | <div class="pdbe-citations 6hud" style="background-color:#fffaf0;"></div> | | <div class="pdbe-citations 6hud" style="background-color:#fffaf0;"></div> |
| | + | |
| | + | ==See Also== |
| | + | *[[Monoclonal Antibodies 3D structures|Monoclonal Antibodies 3D structures]] |
| | == References == | | == References == |
| | <references/> | | <references/> |
| | __TOC__ | | __TOC__ |
| - | </StructureSection> | + | </SX> |
| | [[Category: Homo sapiens]] | | [[Category: Homo sapiens]] |
| | [[Category: Large Structures]] | | [[Category: Large Structures]] |
| - | [[Category: Camilloni, C]] | + | [[Category: Camilloni C]] |
| - | [[Category: Paissoni, C]] | + | [[Category: Paissoni C]] |
| - | [[Category: Amyloid cardiomyopathy]]
| + | |
| - | [[Category: Ex-vivo cryo-em]]
| + | |
| - | [[Category: Helical reconstruction]]
| + | |
| - | [[Category: Immunoglobulin light chain]]
| + | |
| - | [[Category: Protein fibril]]
| + | |
| Structural highlights
Publication Abstract from PubMed
Systemic light chain amyloidosis (AL) is a life-threatening disease caused by aggregation and deposition of monoclonal immunoglobulin light chains (LC) in target organs. Severity of heart involvement is the most important factor determining prognosis. Here, we report the 4.0 A resolution cryo-electron microscopy map and molecular model of amyloid fibrils extracted from the heart of an AL amyloidosis patient with severe amyloid cardiomyopathy. The helical fibrils are composed of a single protofilament, showing typical 4.9 A stacking and cross-beta architecture. Two distinct polypeptide stretches (total of 77 residues) from the LC variable domain (Vl) fit the fibril density. Despite Vl high sequence variability, residues stabilizing the fibril core are conserved through different cardiotoxic Vl, highlighting structural motifs that may be common to misfolding-prone LCs. Our data shed light on the architecture of LC amyloids, correlate amino acid sequences with fibril assembly, providing the grounds for development of innovative medicines.
Cryo-EM structure of cardiac amyloid fibrils from an immunoglobulin light chain AL amyloidosis patient.,Swuec P, Lavatelli F, Tasaki M, Paissoni C, Rognoni P, Maritan M, Brambilla F, Milani P, Mauri P, Camilloni C, Palladini G, Merlini G, Ricagno S, Bolognesi M Nat Commun. 2019 Mar 20;10(1):1269. doi: 10.1038/s41467-019-09133-w. PMID:30894521[1]
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.
See Also
References
- ↑ Swuec P, Lavatelli F, Tasaki M, Paissoni C, Rognoni P, Maritan M, Brambilla F, Milani P, Mauri P, Camilloni C, Palladini G, Merlini G, Ricagno S, Bolognesi M. Cryo-EM structure of cardiac amyloid fibrils from an immunoglobulin light chain AL amyloidosis patient. Nat Commun. 2019 Mar 20;10(1):1269. doi: 10.1038/s41467-019-09133-w. PMID:30894521 doi:http://dx.doi.org/10.1038/s41467-019-09133-w
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