7kwz

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==TDP-43 LCD amyloid fibrils==
==TDP-43 LCD amyloid fibrils==
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<StructureSection load='7kwz' size='340' side='right'caption='[[7kwz]]' scene=''>
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<StructureSection load='7kwz' size='340' side='right'caption='[[7kwz]], [[Resolution|resolution]] 3.20&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=7KWZ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=7KWZ FirstGlance]. <br>
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<table><tr><td colspan='2'>[[7kwz]] is a 5 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=7KWZ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=7KWZ FirstGlance]. <br>
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</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=7kwz FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=7kwz OCA], [https://pdbe.org/7kwz PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=7kwz RCSB], [https://www.ebi.ac.uk/pdbsum/7kwz PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=7kwz ProSAT]</span></td></tr>
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</td></tr><tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">TARDBP, TDP43 ([https://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</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=7kwz FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=7kwz OCA], [https://pdbe.org/7kwz PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=7kwz RCSB], [https://www.ebi.ac.uk/pdbsum/7kwz PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=7kwz ProSAT]</span></td></tr>
</table>
</table>
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== Disease ==
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[[https://www.uniprot.org/uniprot/TADBP_HUMAN TADBP_HUMAN]] Defects in TARDBP are the cause of amyotrophic lateral sclerosis type 10 (ALS10) [MIM:[https://omim.org/entry/612069 612069]]. ALS is a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of ALS is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.<ref>PMID:20740007</ref> <ref>PMID:18288693</ref> <ref>PMID:18438952</ref> <ref>PMID:18396105</ref> <ref>PMID:18372902</ref> <ref>PMID:18309045</ref> <ref>PMID:19350673</ref> <ref>PMID:19224587</ref> <ref>PMID:19655382</ref> <ref>PMID:19695877</ref> <ref>PMID:21220647</ref> <ref>PMID:21418058</ref> <ref>PMID:22456481</ref>
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== Function ==
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[[https://www.uniprot.org/uniprot/TADBP_HUMAN TADBP_HUMAN]] DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.<ref>PMID:17481916</ref> <ref>PMID:11285240</ref>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Amyotrophic lateral sclerosis and several other neurodegenerative diseases are associated with brain deposits of amyloid-like aggregates formed by the C-terminal fragments of TDP-43 that contain the low complexity domain of the protein. Here, we report the cryo-EM structure of amyloid formed from the entire TDP-43 low complexity domain in vitro at pH 4. This structure reveals single protofilament fibrils containing a large (139-residue), tightly packed core. While the C-terminal part of this core region is largely planar and characterized by a small proportion of hydrophobic amino acids, the N-terminal region contains numerous hydrophobic residues and has a non-planar backbone conformation, resulting in rugged surfaces of fibril ends. The structural features found in these fibrils differ from those previously found for fibrils generated from short protein fragments. The present atomic model for TDP-43 LCD fibrils provides insight into potential structural perturbations caused by phosphorylation and disease-related mutations.
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Cryo-EM structure of amyloid fibrils formed by the entire low complexity domain of TDP-43.,Li Q, Babinchak WM, Surewicz WK Nat Commun. 2021 Mar 12;12(1):1620. doi: 10.1038/s41467-021-21912-y. PMID:33712624<ref>PMID:33712624</ref>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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<div class="pdbe-citations 7kwz" style="background-color:#fffaf0;"></div>
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== References ==
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<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Human]]
[[Category: Large Structures]]
[[Category: Large Structures]]
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[[Category: Babinchak WM]]
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[[Category: Babinchak, W M]]
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[[Category: Li Q]]
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[[Category: Li, Q]]
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[[Category: Surewicz WK]]
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[[Category: Surewicz, W K]]
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[[Category: Amyloid]]
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[[Category: Amyotropic lateral sclerosis]]
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[[Category: Neurodegenerative disease]]
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[[Category: Protein fibril]]
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[[Category: Tdp-43]]

Revision as of 06:54, 24 March 2021

TDP-43 LCD amyloid fibrils

PDB ID 7kwz

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