PXD067080

PXD067080 is an original dataset announced via ProteomeXchange.

Title	The Perijunctional Zone Is a Molecularly Distinct Muscle Subdomain Altered in Duchenne Muscular Dystrophy
Description	The neuromuscular junction (NMJ) is a well-established model for synapse development, structure, and function. Surrounding the NMJ is a narrow perijunctional zone (PJZ), enriched in muscle-specific voltage-gated sodium channels that prevent synaptic fatigue. Despite this role, the PJZ remains poorly characterized. To determine its molecular composition, we engineered mice to express the biotin ligase TurboID fused to the cell adhesion molecule neurofascin (Nfasc), and that localizes to the PJZ through ankyrin scaffolding proteins. Using proximity proteomics, we identified numerous PJZ-associated proteins, including Perilipin 4 (Plin4), that were highly enriched and clustered at the PJZ. We also performed proximity proteomics on the PJZ of mdx mice, a model of Duchenne muscular dystrophy. We found broad changes in PJZ composition, including significantly reduced PJZ Plin4. These findings establish the PJZ as a molecularly distinct subdomain of skeletal muscle and provide insight into its potential roles in neuromuscular function and disease.
HostingRepository	PRIDE
AnnounceDate	2026-03-27
AnnouncementXML	Submission_2026-03-26_17:24:13.453.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Juan Oses-Prieto
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: NEWT:10090;
ModificationList	No PTMs are included in the dataset
Instrument	Orbitrap Fusion Lumos; Orbitrap Exploris 480

Revision	Datetime	Status	ChangeLog Entry
0	2025-08-07 10:54:48	ID requested
⏵ 1	2026-03-26 17:24:13	announced

Dataset with its publication pending

submitter keyword: muscle proteomics, perijunctional zone, Perilipin 4, TurboID,Proximity labelling, Duchenne Muscular Dystrophy, Neuromuscular junction

Matthew Rasband
contact affiliation	Professor and Vivian L. Smith Endowed Chair in Neuroscience Department of Neuroscience. Baylor College of Medicine Houston, Texas United States
contact email	rasband@bcm.edu
lab head
Juan Oses-Prieto
contact affiliation	UCSF
contact email	joses@cgl.ucsf.edu
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/2026/03/PXD067080

PRIDE project URI

• PRIDE
  1. PXD067080
     1. Label: PRIDE project
     2. Name: The Perijunctional Zone Is a Molecularly Distinct Muscle Subdomain Altered in Duchenne Muscular Dystrophy