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@ARTICLE{GarciaMorato:163198,
author = {Garcia Morato, Jorge and Hans, Friederike and von Zweydorf,
Felix and Feederle, Regina and Elsässer, Simon J and
Skodras, Angelos and Gloeckner, Christian Johannes and
Buratti, Emanuele and Neumann, Manuela and Kahle, Philipp},
title = {{S}irtuin-1 sensitive lysine-136 acetylation drives phase
separation and pathological aggregation of {TDP}-43.},
journal = {Nature Communications},
volume = {13},
number = {1},
issn = {2041-1723},
address = {[London]},
publisher = {Nature Publishing Group UK},
reportid = {DZNE-2022-00033},
pages = {1223},
year = {2022},
abstract = {Trans-activation response DNA-binding protein of 43 kDa
(TDP-43) regulates RNA processing and forms
neuropathological aggregates in patients with amyotrophic
lateral sclerosis and frontotemporal lobar degeneration.
Investigating TDP-43 post-translational modifications, we
discovered that K84 acetylation reduced nuclear import
whereas K136 acetylation impaired RNA binding and splicing
capabilities of TDP-43. Such failure of RNA interaction
triggered TDP-43 phase separation mediated by the C-terminal
low complexity domain, leading to the formation of insoluble
aggregates with pathologically phosphorylated and
ubiquitinated TDP-43. Introduction of acetyl-lysine at the
identified sites via amber suppression confirmed the results
from site-directed mutagenesis. K84-acetylated TDP-43 showed
cytoplasmic mislocalization, and the aggregation propensity
of K136-acetylated TDP-43 was confirmed. We generated
antibodies selective for TDP-43 acetylated at these lysines,
and found that sirtuin-1 can potently deacetylate
K136-acetylated TDP-43 and reduce its aggregation
propensity. Thus, distinct lysine acetylations modulate
nuclear import, RNA binding and phase separation of TDP-43,
suggesting regulatory mechanisms for TDP-43 pathogenesis.},
keywords = {Acetylation / Amyotrophic Lateral Sclerosis: metabolism /
DNA-Binding Proteins: metabolism / Humans / Lysine:
metabolism / Protein Aggregation, Pathological: metabolism /
Protein Processing, Post-Translational / RNA: metabolism /
Sirtuin 1: genetics / Sirtuin 1: metabolism},
cin = {AG Gasser 1 / AG Kahle 2 / AG Gloeckner 2 / AG Feederle /
AG Jucker / AG Neumann},
ddc = {500},
cid = {I:(DE-2719)1210000 / I:(DE-2719)1210000-4 /
I:(DE-2719)1210007 / I:(DE-2719)1140004 / I:(DE-2719)1210001
/ I:(DE-2719)1210003},
pnm = {353 - Clinical and Health Care Research (POF4-353) / 352 -
Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-353 / G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pmc = {pmc:PMC8907366},
pubmed = {pmid:35264561},
doi = {10.1038/s41467-022-28822-7},
url = {https://pub.dzne.de/record/163198},
}