Dataset Information

Proteome analysis of skeletal muscle of DMD, BMD and WT pigs

ABSTRACT: Duchenne muscular dystrophy is a fatal X-linked disease caused by mutations in the DMD gene, leading to the absence of dystrophin and progressive degeneration of skeletal and cardiac muscles. Pigs lacking DMD exon 52 (DMDΔ52) are a clinically severe model for DMD, mimicking molecular, functional, and pathological hallmarks of the human disease. Dystrophin expression can be restored by additionally deleting exon 51 (DMDΔ51-52), which reframes DMD transcripts and alleviates pathological alterations. DMDΔ51-52 pigs model Becker muscular dystrophy (BMD), a milder and slower-progressing form of muscle degeneration.

INSTRUMENT(S):

ORGANISM(S): Sus Scrofa Domesticus (domestic Pig)

TISSUE(S): Skeletal Muscle

SUBMITTER: Jan Bernd Stöckl

LAB HEAD: Thomas Fröhlich

PROVIDER: PXD064879 | Pride | 2026-03-12

REPOSITORIES: Pride

ACCESS DATA

Dataset's files

Source:

			Action	DRS
	EW_DMD2025_dia800ng.zip	Other
	checksum.txt	Txt
	report.parquet	Other

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Publications

Reduced Muscle Force in Dystrophic DMDΔ52 Pigs Is Incompletely Restored by Systemic Transcript Reframing (DMDΔ51-52).

Blasi Michaela M Hristov Hristiyan H Stöckl Jan B JB Kraetzl Martin M Fiedler Sonja S Kemter Elisabeth E Kurome Mayuko M Kessler Barbara B Cambra Josep M JM Zakhartchenko Valeri V Walter Maggie C MC Kupatt Christian C Klymiuk Nikolai N Fröhlich Thomas T Blutke Andreas A Stirm Michael M Jaudas Florian F Wolf Eckhard E

Journal of cachexia, sarcopenia and muscle 20250101 5

<h4>Background</h4>Duchenne muscular dystrophy (DMD) is a fatal X-linked disease caused by mutations in the DMD gene, leading to dystrophin deficiency and progressive degeneration of skeletal and cardiac muscles. Pigs lacking DMD exon 52 (DMDΔ52) are a clinically severe model for DMD, mimicking molecular, functional and pathological hallmarks of the human disease. Dystrophin expression can be restored by additionally deleting exon 51 (DMDΔ51-52), which reframes DMD transcripts and alleviates pat ...[more]

PMID: 41030228

Dataset Information

Proteome analysis of skeletal muscle of DMD, BMD and WT pigs

Dataset's files

Publications

Reduced Muscle Force in Dystrophic DMDΔ52 Pigs Is Incompletely Restored by Systemic Transcript Reframing (DMDΔ51-52).

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