Defects of the cystic fibrosis (CF) transmembrane conduc-tance regulator (CFTR) protein affect the homeostasis of chloride, bicarbonate, sodium, and water in the airway surface liquid, influencing the mucus composition and viscosity, which induces a severe condition of infection and inflammation along the whole life of CF patients. The introduction of CFTR mod-ulators, novel drugs directly intervening to rescue the function of CFTR protein, opens a new era of experimental research. The review summarizes the most recent advancements to understand the characteristics of the infective and inflamma -tory pathology of CF lungs.

Overview of CF lung pathophysiology

Cabrini, Giulio
Primo
;
Rimessi, Alessandro
Secondo
;
Borgatti, Monica;Pinton, Paolo
Penultimo
;
Gambari, Roberto
Ultimo
2022

Abstract

Defects of the cystic fibrosis (CF) transmembrane conduc-tance regulator (CFTR) protein affect the homeostasis of chloride, bicarbonate, sodium, and water in the airway surface liquid, influencing the mucus composition and viscosity, which induces a severe condition of infection and inflammation along the whole life of CF patients. The introduction of CFTR mod-ulators, novel drugs directly intervening to rescue the function of CFTR protein, opens a new era of experimental research. The review summarizes the most recent advancements to understand the characteristics of the infective and inflamma -tory pathology of CF lungs.
2022
Cabrini, Giulio; Rimessi, Alessandro; Borgatti, Monica; Pinton, Paolo; Gambari, Roberto
File in questo prodotto:
File Dimensione Formato  
1-s2.0-S1471489222000418-main (3).pdf

solo gestori archivio

Descrizione: versione editoriale
Tipologia: Full text (versione editoriale)
Licenza: NON PUBBLICO - Accesso privato/ristretto
Dimensione 1.05 MB
Formato Adobe PDF
1.05 MB Adobe PDF   Visualizza/Apri   Richiedi una copia

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11392/2497652
Citazioni
  • ???jsp.display-item.citation.pmc??? 4
  • Scopus 7
  • ???jsp.display-item.citation.isi??? 7
social impact