PXD047400

PXD047400 is an original dataset announced via ProteomeXchange.

Title	Mass-spectrometry based screening revealing proteins bound to FMR1 mRNA
Description	Short nucleotide repeats are robustly distributed in human genome and contribute to pathogenesis of multiple neurodegenerative disorders such as Fragile X-associated Tremor/Ataxia Syndrome (FXTAS). Pathogenesis of FXTAS is driven by premutation status of CGG repeats expansion (CGGexp) in the 5’UTR region of fragile X messenger ribonucleoprotein 1 (FMR1) gene, when CGG triplet varies between 55-200. One of the proposed molecular mechanism involved in disease progression is repeats associated non-AUG (RAN) translation, which results in the production of toxic aggregation-prone protein called FMRpolyG. Mechanistic insights of RAN translation remain elusive, therefore we aimed to identify novel RAN translation modifiers. In order to identify factors involved in FMRpolyG synthesis we applied RNA-tagging system combined with mass spectrometry (MS) based protein identification to elucidate pool of proteins natively bound to FMR1 transcript harboring CGGexp mimicking FXTAS premutation status. As a control RNAm we used construct enriched with G and C nucleotides (named GCrich RNA) to mimic GC content in investigated FMR1 mRNA. Overall, performed MS-based screening revealed novel proteins which bind specifically to 5’UTR of FMR1 with CGGexp in cellulo.
HostingRepository	PRIDE
AnnounceDate	2025-05-26
AnnouncementXML	Submission_2025-05-25_16:08:08.466.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Anna Baud
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	monohydroxylated residue; iodoacetamide derivatized residue
Instrument	Q Exactive HF

Revision	Datetime	Status	ChangeLog Entry
0	2023-11-30 05:28:42	ID requested
⏵ 1	2025-05-25 16:08:09	announced

10.7554/elife.98631;

Tutak K, Broniarek I, Zielezinski A, Niewiadomska D, Skrzypczak T, Baud A, Sobczak K, Insufficiency of 40S ribosomal proteins, RPS26 and RPS25, negatively affects biosynthesis of polyglycine-containing proteins in fragile-X associated conditions. Elife, 13():(2025) [pubmed]

submitter keyword: HEK,FMR1, human, mRNA, RPS26, RNA binding proteins

Krzysztof Sobczak
contact affiliation	Department of Gene Expression Institute of Molecular Biology and Biotechnology Faculty of Biology Adam Mickiewicz University Uniwersytetu Poznanskiego 6 61-614 Poznan
contact email	ksobczak@amu.edu.pl
lab head
Anna Baud
contact affiliation	Adam Mickiewicz University in Poznan
contact email	annbau1@amu.edu.pl
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/PXD047400

PRIDE project URI

• PRIDE
  1. PXD047400
     1. Label: PRIDE project
     2. Name: Mass-spectrometry based screening revealing proteins bound to FMR1 mRNA