PXD049374-1

PXD049374 is an original dataset announced via ProteomeXchange.

Title	IL-17A-producing MAIT cells and epidermolyis bullosa inflammation
Description	Background: Recessive Dystrophic Epidermolysis Bullosa (RDEB) is characterized by wounds chronically colonized with Staphylococcus aureus associated with local and systemic inflammation. This study aimed to clarify how the immune response is shaped by S. aureus in RDEB patients with focus on Mucosal-Associated invariant T (MAIT) cells. Methods: Fifteen RDEB children (moderate phenotype n=5; severe phenotype n=10) and controls (n=18) were enrolled. A novel proteomic pipeline was used to evaluate over 800 proteins in plasma. Patient-specific cytokine, immune T cell and S. aureus signatures were evaluated. The effect of the patient's S. aureus strain on T-cell function was analyzed in vitro and correlated with the bacterial secretome. Findings: We detected a unique plasma proteomic signature that enabled the differentiation of RDEB children and controls. Profiling of immune cells (n= 30 subsets and cytokine-producing cells) and cytokines (n=38) identified a severe inflammatory response and activation of CD4+ T and MAIT cells in severe RDEB patients with increased frequency of IL-17A-producing CD4+ T and MAIT cells. Positive S. aureus cultures from the skin of 12 of the 15 RDEB patients allowed whole-genome sequencing of patient strains, assessment of the primary keratinocyte response to bacterial challenge, and identification of potential immunomodulatory bacterial determinants. Conditioned media from keratinocytes challenged with S. aureus strains of severe RDEB patients induced a strong Th17 response. Interpretation: 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_02:55:59.186.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Chiara guerrera
SpeciesList	scientific name: Staphylococcus aureus; NCBI TaxID: 1280;
ModificationList	acetylated residue; monohydroxylated residue; iodoacetamide derivatized residue
Instrument	timsTOF Pro

Revision	Datetime	Status	ChangeLog Entry
0	2024-02-14 06:40:38	ID requested
⏵ 1	2025-07-21 02:55:59	announced

Dataset with its publication pending

submitter keyword: Mucosal-Associated invariant T (MAIT) cells, IL-17, inflammation,Recessive Dystrophic Epidermolysis Bullosa (RDEB), skin, Staphylococcus aureus, cytokines, patients, proteomic

Ida Chiara Guerrera
contact affiliation	Proteomics platform Necker, Université Paris Cité - Structure Fédérative de Recherche Necker, INSERM US24/CNRS UAR3633, 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/PXD049374

PRIDE project URI

• PRIDE
  1. PXD049374
     1. Label: PRIDE project
     2. Name: IL-17A-producing MAIT cells and epidermolyis bullosa inflammation