PXD073710

PXD073710 is an original dataset announced via ProteomeXchange.

Title	Proteomic profiling of dysferlin microdeletion–induced skeletal muscle remodeling in a knock-in mouse model
Description	This project examines the proteomic impact of dysferlin deficiency using a genetically defined knock-in mouse model carrying a five-nucleotide microdeletion in the Dysf gene that corresponds to a pathogenic variant identified in limb-girdle muscular dystrophy type R2 (LGMD-R2) patients. Dysferlin is essential for sarcolemmal membrane repair in skeletal muscle, and its loss leads to progressive muscle degeneration associated with inflammation and tissue remodeling. Despite existing dysferlin-deficient mouse models, variant-specific proteomic characterization in vivo remains limited. Skeletal muscle tissues from wild-type and Dysf Δ5/Δ5 mice were analyzed by global, label-free proteomics to define molecular pathways associated with dysferlin loss. Proteins were extracted from muscle lysates, digested using an S-Trap–based workflow, and analyzed by high-resolution nanoLC–MS/MS. Data were acquired in data-independent acquisition mode using dia-PASEF on a trapped ion mobility mass spectrometer, enabling reproducible and comprehensive protein quantification.
HostingRepository	PRIDE
AnnounceDate	2026-04-09
AnnouncementXML	Submission_2026-04-09_06:55:53.149.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Wei-Chi Ku
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: NEWT:10090;
ModificationList	No PTMs are included in the dataset
Instrument	Bruker Daltonics timsTOF series

Revision	Datetime	Status	ChangeLog Entry
0	2026-01-28 05:30:49	ID requested
⏵ 1	2026-04-09 06:55:55	announced

10.1038/s41598-026-46635-2;

Chen YL, Lin WN, Pan PY, Ku WC, Wang P, Yip PK, Tsui KC, Wu YN, Lin YH, Generation of a novel Dysferlin microdeletion knock-in mouse model mimicking muscular dystrophy-like pathology. Sci Rep, 16(1):(2026) [pubmed]

submitter keyword: dysferlinopathy

limb-girdle muscular dystrophy

skeletal muscle proteomics

DIA-PASEF mass spectrometry

Wei-Chi Ku
contact affiliation	School of Medicine, College of Medicine, Fu Jen Catholic Univeristy, New Taipei City, Taiwan
contact email	089052@mail.fju.edu.tw
lab head
Wei-Chi Ku
contact affiliation	Fu Jen Catholic University
contact email	089052@mail.fju.edu.tw
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/2026/04/PXD073710

PRIDE project URI

• PRIDE
  1. PXD073710
     1. Label: PRIDE project
     2. Name: Proteomic profiling of dysferlin microdeletion–induced skeletal muscle remodeling in a knock-in mouse model