PXD061751

PXD061751 is an original dataset announced via ProteomeXchange.

Title	Changes in Sputum Viscoelastic Properties and Airway Inflammation in Primary Ciliary Dyskinesia are Comparable to Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor Therapy
Description	ivacaftor (ETI) therapy. Methods: We measured and compared sputum rheology, inflammation markers and the proteome in 42 clinically stable adolescent and adult patients with PCD, 40 patients with CF with at least one F508del allele before and 3 months after initiation of ETI and 15 age-matched healthy controls. Results: Patients with PCD showed increased viscoelastic properties (G’ and G’’ P<0.001) of airway mucus and elevated levels of airway inflammation markers, including free neutrophil serine protease activity (free NE, CatG, PR3 P<0.001), IL-1β and IL-8 (P<0.001), compared to healthy controls, but lower than observed in CF patients at baseline (free proteases, IL-1β, G’ and G’’ P<0.001, IL-8 P<0.05). Significant differences in sputum viscoelastic properties, airway inflammation and the sputum proteome between PCD patients and CF patients on ETI therapy were no longer observed. Conclusions: Clinically stable patients with PCD exhibit abnormalities in sputum viscoelastic properties, inflammation markers and the proteome that are less severe than in patients with CF without ETI, but similar to observed under ETI therapy.
HostingRepository	PRIDE
AnnounceDate	2026-01-12
AnnouncementXML	Submission_2026-01-12_10:10:37.152.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Marieluise Kirchner
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: NEWT:9606;
ModificationList	acetylated residue; monohydroxylated residue; deamidated residue; iodoacetamide derivatized residue
Instrument	Q Exactive HF-X

Revision	Datetime	Status	ChangeLog Entry
0	2025-03-12 03:28:07	ID requested
⏵ 1	2026-01-12 10:10:37	announced

Dataset with its publication pending

submitter keyword: sputum, cystic fibrosis, CF,Primary ciliary dyskinesia, mucus, elexacaftor/tezacaftor/ivacaftor, HF-X, PCD, DDA, airway inflammation

Philipp Mertins
contact affiliation	Max Delbrueck Center for Molecular Medicine in the Helmholtz Association, Berlin, Germany. Berlin Institute of Health (BIH), Berlin, Germany.
contact email	Philipp.mertins@mdc-berlin.de
lab head
Marieluise Kirchner
contact affiliation	Proteomics Platform, BIH@Charite
contact email	marieluise.kirchner@mdc-berlin.de
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD061751
     1. Label: PRIDE project
     2. Name: Changes in Sputum Viscoelastic Properties and Airway Inflammation in Primary Ciliary Dyskinesia are Comparable to Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor Therapy