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Proteome profiling of mouse dystrophic diaphragm reveals decreased expression of H2S-generating enzyme

ABSTRACT: Mdx mice with a spontaneous mutation in exon 23 of the Dmd gene represent the most common model to investigate the pathophysiology of Duchenne muscular dystrophy (DMD). The disease is characterized by irreversible impairment of muscle functions, with the diaphragm affected earlier and more severely than other skeletal muscles, primarily due to a lack of functional dystrophin. The preliminary, label-free (LF) and more thorough tandem mass tag (TMT)-based method revealed 88 commonly changed proteins, in the diaphragm of relatively young 6-week-old mdx mice. A more in-depth analysis of the TMT-based method revealed 953 significantly changed proteins with 867 upregulated and 86 downregulated in dystrophic animals (q-value < 0.05, fold change threshold: 1.5). Consequently, several dysregulated processes were demonstrated, including the immune response, fibrosis, translation, and programmed cell death. Interestingly, in the dystrophic diaphragm, we found a significant decrease in the expression of enzymes generating hydrogen sulfide (H2S): cystathionine γ-lyase level (CTH), 3-mercaptopyruvate sulfurtransferase (MPST) and cystathionine β-synthase (CBS). In summary, dysregulation of different processes was revealed in the diaphragm of mdx mice at the proteome level. Alterations in the mRNA and protein levels of H2S-generating enzymes suggest that this gaseous mediator might be an important regulator of DMD progression.

INSTRUMENT(S):

ORGANISM(S): Mus Musculus (mouse)

SUBMITTER: Agata Malinowska

LAB HEAD: Agnieszka Łoboda

PROVIDER: PXD040527 | Pride | 2026-03-13

REPOSITORIES: Pride

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			Action	DRS
	201909201895loboda_WT1.dat	Other
	201909201895loboda_WT1.mgf	Mgf
	201909201895loboda_WT1.raw	Raw
	201909201896loboda_WT2.dat	Other
	201909201896loboda_WT2.mgf	Mgf

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