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PXD059914

PXD059914 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleLaminin-a2 is required for the maintenance of the myotendinous junction in vivo
DescriptionThe myotendinous junction (MTJ) is a critical interface between muscle fibers and tendons, essential for force transmission between muscle and bone. Laminin-a2, a key extracellular matrix (ECM) component, is strongly enriched at this interface. Mutations in the LAMA2 gene cause LAMA2-related muscular dystrophy (LAMA2 MD), an early-onset severe congenital muscular dystrophy. Here, we examined the MTJ in dyW/dyW mice, a mouse model for LAMA2 MD. We find a strong disruption of MTJ morphology, including altered muscle fiber tips, collagen XXII mislocalization, and reduced muscle tendon interface. As MTJ loading is altered in dyW/dyW mice and MTJ maintenance requires loading and unloading, we also examined MTJ structures upon denervation-induced unloading. While muscle fiber tip morphology resembled that of dyW/dyW mice, collagen XXII distribution was not affected and the muscle–tendon interface was preserved. Finally, proteomic profiling via laser capture microdissection and mass spectrometry revealed significant regional and global shifts in MTJ protein composition in dyW/dyW and denervated mice. Across both models, we identified integrin-associated remodeling as a shared response linked to the perturbed muscle fiber tip morphology. These findings demonstrate that laminin-a2 is required for MTJ stability, and that mechanical unloading contributes to the observed phenotype. Importantly, our results suggest that disruptions in MTJ structure and protein composition may contribute to the pathology observed in LAMA2 MD.
HostingRepositoryMassIVE
AnnounceDate2026-03-26
AnnouncementXMLSubmission_2026-03-26_02:09:48.197.xml
DigitalObjectIdentifier
ReviewLevelNon peer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterThomas Bock
SpeciesList scientific name: Mus musculus; common name: house mouse; NCBI TaxID: 10090;
ModificationListNo PTMs are included in the dataset
InstrumentOrbitrap Eclipse; timsTOF Ultra
Dataset History
RevisionDatetimeStatusChangeLog Entry
02025-01-17 02:54:34ID requested
12026-03-26 02:09:48announced
Publication List
no publication
Keyword List
submitter keyword: Laminin, Myotendinous Junction, DatasetType:Proteomics
Contact List
Alexander Schmidt
contact affiliationBiozentrum, Universtiy of Basel, 4056 Basel, Switzerland
contact emailalex.schmidt@unibas.ch
lab head
Thomas Bock
contact affiliationUni Basel
contact emailthomas.bock@unibas.ch
dataset submitter
Full Dataset Link List
MassIVE dataset URI
Dataset FTP location
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