Unknown

Dataset Information

0

Nucleolin Rescues TDP-43 Toxicity in Yeast and Human Cell Models.


ABSTRACT: TDP-43 is a nuclear protein involved in pivotal processes, extensively studied for its implication in neurodegenerative disorders. TDP-43 cytosolic inclusions are a common neuropathologic hallmark in amyotrophic lateral sclerosis (ALS) and related diseases, and it is now established that TDP-43 misfolding and aggregation play a key role in their etiopathology. TDP-43 neurotoxic mechanisms are not yet clarified, but the identification of proteins able to modulate TDP-43-mediated damage may be promising therapeutic targets for TDP-43 proteinopathies. Here we show by the use of refined yeast models that the nucleolar protein nucleolin (NCL) acts as a potent suppressor of TDP-43 toxicity, restoring cell viability. We provide evidence that NCL co-expression is able to alleviate TDP-43-induced damage also in human cells, further supporting its beneficial effects in a more consistent pathophysiological context. Presented data suggest that NCL could promote TDP-43 nuclear retention, reducing the formation of toxic cytosolic TDP-43 inclusions.

SUBMITTER: Peggion C 

PROVIDER: S-EPMC8072491 | biostudies-literature |

REPOSITORIES: biostudies-literature

Similar Datasets

2021-03-26 | PXD022432 | Pride
| S-EPMC3934366 | biostudies-literature
| S-EPMC4550816 | biostudies-literature
| S-EPMC8812793 | biostudies-literature
| S-EPMC8583295 | biostudies-literature
| S-EPMC2359814 | biostudies-other
| S-EPMC8675103 | biostudies-literature
| S-EPMC6502655 | biostudies-literature
| S-EPMC4478251 | biostudies-literature
| S-EPMC3724736 | biostudies-literature