View source for 7u15


==TMEM106B(120-254) singlet amyloid fibril from frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP) type B case 2 (case 7).==
<StructureSection load='7u15' size='340' side='right'caption='[[7u15]], [[Resolution|resolution]] 3.00&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=7U15 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=7U15 FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 3&#8491;</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</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=7u15 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=7u15 OCA], [https://pdbe.org/7u15 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=7u15 RCSB], [https://www.ebi.ac.uk/pdbsum/7u15 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=7u15 ProSAT]</span></td></tr>
</table>
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
Misfolding and aggregation of disease-specific proteins, resulting in the formation of filamentous cellular inclusions, is a hallmark of neurodegenerative disease with characteristic filament structures, or conformers, defining each proteinopathy. Here we show that a previously unsolved amyloid fibril composed of a 135 amino acid C-terminal fragment of TMEM106B is a common finding in distinct human neurodegenerative diseases, including cases characterized by abnormal aggregation of TDP-43, tau, or alpha-synuclein protein. A combination of cryoelectron microscopy and mass spectrometry was used to solve the structures of TMEM106B fibrils at a resolution of 2.7 A from postmortem human brain tissue afflicted with frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP, n = 8), progressive supranuclear palsy (PSP, n = 2), or dementia with Lewy bodies (DLB, n = 1). The commonality of abundant amyloid fibrils composed of TMEM106B, a lysosomal/endosomal protein, to a broad range of debilitating human disorders indicates a shared fibrillization pathway that may initiate or accelerate neurodegeneration.

Homotypic fibrillization of TMEM106B across diverse neurodegenerative diseases.,Chang A, Xiang X, Wang J, Lee C, Arakhamia T, Simjanoska M, Wang C, Carlomagno Y, Zhang G, Dhingra S, Thierry M, Perneel J, Heeman B, Forgrave LM, DeTure M, DeMarco ML, Cook CN, Rademakers R, Dickson DW, Petrucelli L, Stowell MHB, Mackenzie IRA, Fitzpatrick AWP Cell. 2022 Mar 1. pii: S0092-8674(22)00259-8. doi: 10.1016/j.cell.2022.02.026. PMID:35247328<ref>PMID:35247328</ref>

From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
<div class="pdbe-citations 7u15" style="background-color:#fffaf0;"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Large Structures]]
[[Category: Arakhamia T]]
[[Category: Carlomagno Y]]
[[Category: Chang A]]
[[Category: Cook CN]]
[[Category: DeMarco ML]]
[[Category: DeTure M]]
[[Category: Dhingra S]]
[[Category: Dickson D]]
[[Category: Fitzpatrick AWP]]
[[Category: Forgrave LM]]
[[Category: Heeman B]]
[[Category: Lee C]]
[[Category: Mackenzie IRA]]
[[Category: Perneel J]]
[[Category: Petrucelli L]]
[[Category: Rademakers R]]
[[Category: Simjanoska M]]
[[Category: Stowell MHB]]
[[Category: Thierry M]]
[[Category: Wang C]]
[[Category: Wang J]]
[[Category: Xiang X]]
[[Category: Zhang G]]