PXD042141-1

PXD042141 is an original dataset announced via ProteomeXchange.

Title	Th17 and MAIT17 cells are associated with severe Recessive Dystrophic Epidermolysis Bullosa: Staphylococcus aureus involvement
Description	Recessive Dystrophic Epidermolysis bullosa (RDEB) is characterized by wounds colonized with Staphylococcus aureus associated with local and systemic inflammation. However, it remains unclear how the immune response and S. aureus interact. A novel proteomic pipeline was used to evaluate over 800 proteins in plasma, resulting in a unique proteomic signature that enabled the differentiation of RDEB patients (n=15); mild phenotype n=5; severe phenotype n=10; median 7 years) and controls (n=18; median 5 years). Immune cell (n= 30 subsets and cytokine-producing cells) and cytokine (n=38) profiling identified a severe inflammatory response and activation of conventional CD4+ T and innate-like T cells as Mucosal-Associated invariant T (MAIT) and gamma delta T cells in RDEB severe patients with increased frequency of IL-17A-producing CD4+ T and MAIT cells. Positive S. aureus cultures from skin of 12 out of 15 RDEB patients allowed whole-genome sequencing of patient's strains and assessment of primary keratinocyte immune response upon bacterial challenge. Conditioned media from keratinocytes challenged with S. aureus strains of severe RDEB patients induced a strong Th17 response. In conclusion, this study uncovers a huge systemic inflammatory response associated with a high level of circulating IL-17A-producing CD4+ T and MAIT cells in RDEB patients and supports the involvement of patient S. aureus strains in biasing the host immune response.
HostingRepository	PRIDE
AnnounceDate	2025-07-21
AnnouncementXML	Submission_2025-07-21_04:36:19.857.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Chiara guerrera
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	No PTMs are included in the dataset
Instrument	timsTOF Pro

Revision	Datetime	Status	ChangeLog Entry
0	2023-05-11 03:48:37	ID requested
⏵ 1	2025-07-21 04:36:20	announced

Dataset with its publication pending

submitter keyword: severe Recessive Dystrophic Epidermolysis Bullosa, DIA , Plasma,Staphylococcus aureus, Th17 and MAIT17 cells

Ida Chiara Guerrera
contact affiliation	Proteomics platform Necker, Université Paris Cité - Structure Fédérative de Recherche Necker, INSERM US24/CNRSUAR3633, Paris 75015, France.
contact email	chiara.guerrera@inserm.fr
lab head
Chiara guerrera
contact affiliation	Necker proteomics, INSERM
contact email	chiara.guerrera@inserm.fr
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/2025/07/PXD042141

PRIDE project URI

• PRIDE
  1. PXD042141
     1. Label: PRIDE project
     2. Name: Th17 and MAIT17 cells are associated with severe Recessive Dystrophic Epidermolysis Bullosa: Staphylococcus aureus involvement