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@ARTICLE{Simonetti:282909,
author = {Simonetti, Francesca and Zhong, Weijia and Hutten, Saskia
and Uliana, Federico and Schifferer, Martina and Rezaei, Ali
and Ramirez, Lisa Marie and Hochmair, Janine and Sankar,
Rithika and Gopalan, Anusha and Kielisch, Fridolin and
Riemenschneider, Henrick and Ruf, Viktoria and Schmidt,
Carla and Simons, Mikael and Zweckstetter, Markus and
Wegmann, Susanne and Lashley, Tammaryn and Polymenidou,
Magdalini and Edbauer, Dieter and Dormann, Dorothee},
title = {{D}irect interaction between {TDP}-43 and {T}au promotes
their co-condensation, while suppressing {T}au fibril
formation and seeding.},
journal = {The EMBO journal},
volume = {44},
number = {24},
issn = {0261-4189},
address = {[London]},
publisher = {Nature Publishing Group UK},
reportid = {DZNE-2025-01370},
pages = {7395 - 7433},
year = {2025},
abstract = {Neuronal aggregates of Tau are a hallmark of Alzheimer's
disease (AD), but more than half of the patients exhibit
additional TDP-43 inclusions, while some have co-aggregates
of the two proteins. The presence of such co-aggregates is
associated with increased disease severity, although whether
there is a causal relationship remains unclear. Here, we
demonstrate that Tau and TDP-43 mutually promote each
other's condensation through direct interaction in vitro,
forming irregularly-shaped or multiphasic co-condensates
with lower TDP-43 mobility, but higher Tau mobility. While
Tau promotes TDP-43 aggregation in vitro, TDP-43 suppresses
formation of Tau fibrils and instead causes formation of
oligomeric Tau and Tau/TDP-43 species. These co-assemblies
hinder Tau seeding in a biosensor assay specific for
proteopathic Tau seeds. Consistent with these data,
insoluble material extracted from AD patient brains with
Tau/TDP-43 co-aggregates exhibits reduced Tau seeding
compared to AD patient brains with Tau aggregates only. In
contrast, patient-derived extracts from AD patient brains
with Tau/TDP-43 co-aggregates are highly potent in seeding
new TDP-43 aggregates in a TDP-43 reporter cell line. Our
results suggest that direct interaction between TDP-43 and
Tau may suppress Tau pathology, while promoting TDP-43
pathology in Alzheimer's disease patients.},
keywords = {tau Proteins: metabolism / tau Proteins: genetics / Humans
/ DNA-Binding Proteins: metabolism / DNA-Binding Proteins:
genetics / Alzheimer Disease: metabolism / Alzheimer
Disease: pathology / Alzheimer Disease: genetics / Protein
Aggregation, Pathological: metabolism / Brain: metabolism /
Brain: pathology / Protein Aggregates / Protein Binding /
Alzheimer’s Disease (Other) / Phase Separation (Other) /
Seeding (Other) / TDP-43 (Other) / Tau (Other) / tau
Proteins (NLM Chemicals) / DNA-Binding Proteins (NLM
Chemicals) / TARDBP protein, human (NLM Chemicals) / MAPT
protein, human (NLM Chemicals) / Protein Aggregates (NLM
Chemicals)},
cin = {AG Edbauer / AG Misgeld / AG Zweckstetter / AG Wegmann /
Clinical Research (Munich)},
ddc = {570},
cid = {I:(DE-2719)1110004 / I:(DE-2719)1110000-4 /
I:(DE-2719)1410001 / I:(DE-2719)1810006 /
I:(DE-2719)1111015},
pnm = {352 - Disease Mechanisms (POF4-352) / 351 - Brain Function
(POF4-351) / 353 - Clinical and Health Care Research
(POF4-353)},
pid = {G:(DE-HGF)POF4-352 / G:(DE-HGF)POF4-351 /
G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:41107545},
doi = {10.1038/s44318-025-00590-2},
url = {https://pub.dzne.de/record/282909},
}