3.4 Cystic Fibrosis

Question 1

What is the function of ERAD?

Answer 1

ER assisted protein degradation - takes the mutated CFTR protein and tags it for degradation via proteasomes

Question 2

How does CFTR affect cellular Na+ concentrations?

Answer 2

It inhibits ENaC thus decreasing the amount of Na+ that enters the cell

Question 3

What about the mucus in the lung is most dangerous for CF pts?

Answer 3

The cilia are prevented from functioning to clear the mucus and this results in build up and possibly death

Question 4

In which organ do CF patients form fibrous cysts?

Answer 4

pancreas

Question 5

What effect does CF have on the heart?

Answer 5

Right ventricular disease as a result of hypoxia and progressive lung destruction that leads to increased PA pressure.

Question 6

What protein is responsible for the increased concentration of Cl via sweat?

Answer 6

NKCC transporter

Question 7

How is insulin resistance affected in CF pts?

Answer 7

They have increased resistance with infection

Question 8

What’s the function of Calnexin?

Answer 8

Calnexin aids in the degradation of CFTR via ERAD or the UPR pathway

Question 9

What is a normal concentration of Cl in sweat? In CF patients?

Answer 9

Normal (40 or less); CF (60 or greater)