⮝ Full datasets listing

PXD040331

PXD040331 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleAltered striatal actin - dynamics drives behavioral inflexibility in a mouse model of Fragile X syndrome
DescriptionThe proteome of glutamatergic synapses is diverse across the mammalian brain and involved in neurodevelopmental disorders (NDDs). Among those is Fragile X syndrome (FXS), an NDD caused by the dysfunctional RNA binding protein FMRP. Here we demonstrate how the brain region-specific composition of post-synaptic density (PSD) contributes to FXS. The FXS mouse model shows, in the striatum, an altered association of the PSD with the actin cytoskeleton, reflecting immature dendritic spine morphology and reduced synaptic actin dynamics. Enhancing actin turnover with constitutively active RAC1 ameliorates these deficits. At the behavioral level, the FXS model displays striatal-driven inflexibility, a typical feature of FXS individuals, that is rescued by exogenous RAC1. Striatal ablation of Fmr1 is sufficient to recapitulate behavioral impairments observed in the FXS model. These results indicate that dysregulation of synaptic actin dynamics in the striatum, a region largely unexplored in FXS, contributes to the manifestation of FXS behavioral phenotypes.
HostingRepositoryPRIDE
AnnounceDate2024-10-22
AnnouncementXMLSubmission_2024-10-22_05:47:24.877.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterTilmann Achsel
SpeciesList scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationListNo PTMs are included in the dataset
InstrumentQ Exactive
Dataset History
RevisionDatetimeStatusChangeLog Entry
02023-02-22 10:17:06ID requested
12023-06-08 09:21:29announced
22023-11-14 08:48:36announced2023-11-14: Updated project metadata.
32024-10-22 05:47:25announced2024-10-22: Updated project metadata.
Publication List
Mercaldo V, Vidimova B, Gastaldo D, Fern, á, ndez E, Lo AC, Cencelli G, Pedini G, De Rubeis S, Longo F, Klann E, Smit AB, Grant SGN, Achsel T, Bagni C, Altered striatal actin dynamics drives behavioral inflexibility in a mouse model of fragile X syndrome. Neuron, 111(11):1760-1775.e8(2023) [pubmed]
10.1016/j.neuron.2023.03.008;
Keyword List
submitter keyword: Fmr1(y/ -), hippocampus, striatum, actin, cortex,post-synaptic density
Contact List
Claudia Bagni
contact affiliationDepartment of Fundamental Neurosciences, Université de Lausanne, 1005 Lausanne, Switzerland & Department of Biomedicine and Prevention, Università degli studi di Roma "Tor Vergata", 00133 Rome, Italy
contact emailClaudia.Bagni@unil.ch
lab head
Tilmann Achsel
contact affiliationDept. of Fundamental Neurosciences University of Lausanne
contact emailTilmann.Achsel@unil.ch
dataset submitter
Full Dataset Link List
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/2023/06/PXD040331
PRIDE project URI
Repository Record List
[ + ]