PXD074360

PXD074360 is an original dataset announced via ProteomeXchange.

Title	EMILIN1 emerges as a SETDB1-dependent modulator of the secretome in Duchenne Muscular Dystrophy
Description	Duchenne Muscular Dystrophy (DMD) is an incurable muscle-wasting disorder characterized by chronic membrane damage, inflammation, and progressive fibrosis. Fibrosis in DMD is driven by sustained TGF signaling, which promotes extracellular matrix (ECM) accumulation. We previously showed that SETDB1 sustains the TGF-induced fibrotic response in DMD myotubes. Here, we further show that SETDB1 modulates the TGF-induced secretome, particularly by regulating ECM-related proteins. Comparison of the basal secretome from DMD patient-derived myotubes and healthy controls revealed a distinct disease-specific profile. Integrating both secretome analyses, we identified EMILIN1, an ECM glycoprotein not previously studied in skeletal muscle, as a robust shared candidate; EMILIN1 is enriched in the DMD secretome, further upregulated by TGF and downregulated upon SETDB1 depletion. We confirmed EMILIN1 overexpression in DMD patient muscle biopsies, validating its pathological relevance. Functionally, EMILIN1 depletion modulated myogenic differentiation and reduced expression of the fibrotic marker SERPINE1. These findings establish EMILIN1 as a novel secreted regulator of myogenesis and fibrosis, and implicate SETDB1 in shaping the TGF-dependent secretome in DMD. Our integrative proteomic approach provides new insights into the molecular drivers of impaired regeneration in DMD and highlights potential therapeutic targets.
HostingRepository	PRIDE
AnnounceDate	2026-08-24
AnnouncementXML	Submission_2026-08-23_16:10:26.869.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Guilhem Clary
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: NEWT:9606;
ModificationList	acetylated residue; monohydroxylated residue; iodoacetamide derivatized residue
Instrument	timsTOF Pro 2

Revision	Datetime	Status	ChangeLog Entry
0	2026-02-11 14:48:58	ID requested
⏵ 1	2026-08-23 16:10:27	announced

10.1038/s41419-026-08825-8;

Zamperoni M, Muraine L, Tran MY, Granados A, Bigot A, Petit V, Bensalah M, Ohana J, Legros V, Boyarchuk E, Bruce J, Chevreux G, Joliot V, Negroni E, Moulin M, Trollet C, Ait-Si-Ali S, /SETDB1-regulated secreted biomarker in Duchenne muscular dystrophy. Cell Death Dis, 17(1):(2026) [pubmed]

submitter keyword: TGFbeta, muscle fibrosis,human muscular dystrophy, muscle secretome, duchenne muscular dystrophy

Capucine Trollet
contact affiliation	Sorbonne Université, Inserm, Institut de Myologie, Centre de Recherche en Myologie, Paris, France.
contact email	capucine.trollet@upmc.fr
lab head
Guilhem Clary
contact affiliation	Istitut Cochin
contact email	guilhem.clary@inserm.fr
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD074360
     1. Label: PRIDE project
     2. Name: EMILIN1 emerges as a SETDB1-dependent modulator of the secretome in Duchenne Muscular Dystrophy