Cystic Fibrosis Airway Mucus Hyperconcentration Produces a Vicious Cycle of Mucin, Pathogen, and Inflammatory Interactions that Promotes Disease Persistence.


Journal

American journal of respiratory cell and molecular biology
ISSN: 1535-4989
Titre abrégé: Am J Respir Cell Mol Biol
Pays: United States
ID NLM: 8917225

Informations de publication

Date de publication:
08 2022
Historique:
pubmed: 30 4 2022
medline: 4 8 2022
entrez: 29 4 2022
Statut: ppublish

Résumé

The dynamics describing the vicious cycle characteristic of cystic fibrosis (CF) lung disease, initiated by stagnant mucus and perpetuated by infection and inflammation, remain unclear. Here we determine the effect of the CF airway milieu, with persistent mucoobstruction, resident pathogens, and inflammation, on the mucin quantity and quality that govern lung disease pathogenesis and progression. The concentrations of MUC5AC and MUC5B were measured and characterized in sputum samples from subjects with CF (

Identifiants

pubmed: 35486871
doi: 10.1165/rcmb.2021-0359OC
pmc: PMC9348562
doi:

Substances chimiques

Mucin 5AC 0
Mucin-5B 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't Research Support, N.I.H., Extramural

Langues

eng

Sous-ensembles de citation

IM

Pagination

253-265

Commentaires et corrections

Type : CommentIn

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Auteurs

Bethany D Batson (BD)

Marsico Lung Institute/Cystic Fibrosis Research Center.
Department of Pathology and Laboratory Medicine.

Bryan T Zorn (BT)

Marsico Lung Institute/Cystic Fibrosis Research Center.
Department of Microbiology and Immunology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.

Giorgia Radicioni (G)

Marsico Lung Institute/Cystic Fibrosis Research Center.
Department of Pathology and Laboratory Medicine.

Stephanie S Livengood (SS)

Marsico Lung Institute/Cystic Fibrosis Research Center.
Department of Pathology and Laboratory Medicine.

Tadahiro Kumagai (T)

Complex Carbohydrate Research Center, University of Georgia, Athens, Georgia.

Hong Dang (H)

Marsico Lung Institute/Cystic Fibrosis Research Center.

Agathe Ceppe (A)

Marsico Lung Institute/Cystic Fibrosis Research Center.

Phillip W Clapp (PW)

Marsico Lung Institute/Cystic Fibrosis Research Center.

Michael Tunney (M)

Queen's University, Belfast, Northern Ireland, United Kingdom; and.

J Stuart Elborn (JS)

Queen's University, Belfast, Northern Ireland, United Kingdom; and.

Noel G McElvaney (NG)

Irish Centre for Genetic Lung Disease, Royal College of Surgeons in Ireland Education and Research Centre, Beaumont Hospital, Dublin, Ireland.

Marianne S Muhlebach (MS)

Marsico Lung Institute/Cystic Fibrosis Research Center.

Richard C Boucher (RC)

Marsico Lung Institute/Cystic Fibrosis Research Center.

Michael Tiemeyer (M)

Complex Carbohydrate Research Center, University of Georgia, Athens, Georgia.

Matthew C Wolfgang (MC)

Marsico Lung Institute/Cystic Fibrosis Research Center.
Department of Microbiology and Immunology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.

Mehmet Kesimer (M)

Marsico Lung Institute/Cystic Fibrosis Research Center.
Department of Pathology and Laboratory Medicine.

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Classifications MeSH