Lecture 1

Question 1

What is the contribution of ASL abnormalities to cystic fibrosis lung disease?

Answer 1

Affected absorption, secretion, pH and mucus viscosity
Reduced ASL volume and impaired mucociliary clearance in cystic fibrosis

Airway dehydration due to hyperactive Na+ absorption
Low volume due to the failure of submucosal glands to secrete and release CF
Low antimicrobial activity due to abnormal ASL pH which reduces antimicrobial activities

Increased risk of infection and inflammation

Question 2

The paper by Yuan et al., 2023 concludes that ionocytes are the main site of ASL formation and most responsible for CF lung disease pathogenesis. Is that conclusion supported by the evidence?, please justify your response. Can you design a better experiment to test the role of ionocytes on ASL formation?

Answer 2

That conclusion is supported by the evidence as it was shown in the experiments that impairing ionocyte development reuslted in ASL formation impairment. However, this cannot be conclusively determined as cells often work in conjunction with other in order to take effect. Therefore, while Ionocytes may play a role in ASL formation it is probably not entirely responsible for ASL impairment