PXD049234

PXD049234 is an original dataset announced via ProteomeXchange.

Title	Stress Granules FUS WT e FUS P525L
Description	Stress Granules (SG) formation is a cellular protection mechanism, constituting a storage for untranslated mRNAs and RNA-binding proteins (RBPs); however, these condensates can turn into pathological aggregates, related to the onset of neurodegenerative diseases like Amyotrophic Lateral Sclerosis (ALS). This transition towards cytotoxic inclusions is triggered by ALS-causative mutations in the RBP FUS, which lead to its cytoplasmic mis-localization and accumulation in SG. Here, we describe the SG transcriptome in a neural context and describe several features for RNA recruitment in SG. We demonstrate that SG dynamics and RNA content are strongly modified by the incorporation of mutant FUS, switching to a more unstructured, AU-rich SG transcriptome. Moreover, we show that mutant FUS, together with its protein interactors and their target RNAs, are responsible for the reshaping of the mutant SG transcriptome with alterations that can be linked to neurodegeneration. Therefore, our data give a comprehensive view of the molecular differences between physiological and pathological SG in ALS conditions, showing how FUS mutations impact the RNA and protein population of these condensates.
HostingRepository	PRIDE
AnnounceDate	2025-05-06
AnnouncementXML	Submission_2025-05-06_12:54:12.775.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Nara Liessi
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	No PTMs are included in the dataset
Instrument	Orbitrap Exploris 480

Revision	Datetime	Status	ChangeLog Entry
0	2024-02-07 02:56:23	ID requested
⏵ 1	2025-05-06 12:54:13	announced

10.1093/nar/gkae942;

Mariani D, Setti A, Castagnetti F, Vitiello E, Stufera Mecarelli L, Di Timoteo G, Giuliani A, D'Angelo A, Santini T, Perego E, Zappone S, Liessi N, Armirotti A, Vicidomini G, Bozzoni I, ALS-associated FUS mutation reshapes the RNA and protein composition of stress granules. Nucleic Acids Res, 52(21):13269-13289(2024) [pubmed]

submitter keyword: Stress Granules, ALS, RNA-binding proteins, neurodegeneration, aggregation

Andrea Armirotti
contact affiliation	Istituto Italiano di Tecnologia
contact email	andrea.armirotti@iit.it
lab head
Nara Liessi
contact affiliation	Istituto Italiano di Tecnologia
contact email	nara.liessi@iit.it
dataset submitter

Dataset FTP location NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.pride.ebi.ac.uk/pride/data/archive/2025/05/PXD049234

PRIDE project URI

• PRIDE
  1. PXD049234
     1. Label: PRIDE project
     2. Name: Stress Granules FUS WT e FUS P525L