PXD040527

PXD040527 is an original dataset announced via ProteomeXchange.

Title	Proteome profiling of mouse dystrophic diaphragm reveals decreased expression of H2S-generating enzyme
Description	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.
HostingRepository	PRIDE
AnnounceDate	2026-03-13
AnnouncementXML	Submission_2026-03-13_10:31:25.947.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Agata Malinowska
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: NEWT:10090;
ModificationList	monohydroxylated residue; iodoacetamide derivatized residue
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2023-03-01 06:53:04	ID requested
⏵ 1	2026-03-13 10:31:26	announced

10.3390/BIOM13111648;

submitter keyword: mdx diaphragm, MPST,Duchenne muscular dystrophy, CTH, hydrogen sulfide, CBS, proteome

Agnieszka Łoboda
contact affiliation	Department of Medical Biotechnology, Faculty of Biochemistry, Biophysics and Biotechnology, Jagiellonian University in Krakow, Kraków, 30-387, Poland
contact email	agnieszka.loboda@uj.edu.pl
lab head
Agata Malinowska
contact affiliation	IBB PAS
contact email	esme@ibb.waw.pl
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/PXD040527

PRIDE project URI

• PRIDE
  1. PXD040527
     1. Label: PRIDE project
     2. Name: Proteome profiling of mouse dystrophic diaphragm reveals decreased expression of H2S-generating enzyme