Abstract
Cystic fibrosis (CF) is an autosomal recessive disease affecting over 100,000 patients globally. Caused by a large number of mutations affecting the CFTR gene (Cystic Fibrosis Transmembrane Conductance Regulator).
This defect leads to abnormal ion transport across lung cells surface, decreased water level resulting in mucus obstruction, inflammation, and bacterial infection. Figure(1).
Several studies have shown that Cathepsin S (CatS) a cysteine protease found mainly in antigen - presenting cells, to be involved in CF. A study at Queen’s University Belfast, showed it to be elevated in 100% of CF patients. Furthermore, studies using CatS knockout showed improved bacterial clearance and reduced inflammation.
This defect leads to abnormal ion transport across lung cells surface, decreased water level resulting in mucus obstruction, inflammation, and bacterial infection. Figure(1).
Several studies have shown that Cathepsin S (CatS) a cysteine protease found mainly in antigen - presenting cells, to be involved in CF. A study at Queen’s University Belfast, showed it to be elevated in 100% of CF patients. Furthermore, studies using CatS knockout showed improved bacterial clearance and reduced inflammation.
| Original language | English |
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| Title of host publication | Winter School (Medicine, Dentistry and Biomedical Sciences) Meeting 2021: Proteinases and their Inhibitors |
| Publication status | Published - 25 Feb 2021 |