Characterisation of cystic fibrosis airway-associated proteases in the era of CFTR modulation

  • Zhixuan Yang

Student thesis: Doctoral Thesis › Doctor of Philosophy

Abstract

Cystic fibrosis (CF) is a hereditary disease caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The lung disease is, however, also associated with dysregulation of the epithelial sodium channel (ENaC) which leads to dehydrated airways predisposing the individual to chronic cycles of infection and inflammation. ENaC is activated by trypsin-like channel activating proteases (TLPs), inhibition of which increases airway surface liquid volume and improves mucociliary function of primary human airway epithelial cells (hAECs) grown at air-liquid interface (ALI).

Thesis is embargoed until 31 December 2027.
Date of AwardDec 2022
Original languageEnglish
Awarding Institution
  • Queen's University Belfast
SupervisorLorraine Martin (Supervisor), Damian Downey (Supervisor) & James Reihill (Supervisor)

Keywords

  • cystic fibrosis
  • CFTR
  • epithelial sodium channel
  • inflammation
  • infection

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