tbl 4

Question 1

when does CF occur?

Answer 1

when the CFTR protein is not synthesized and folded correctly

Question 2

what is the function of the CFTR protein?

Answer 2

it helps to maintain the balance of salt and water on many body surfaces, like the lung surface

when the protein is not working correctly, chloride becomes trapped in cells, then water cannot hydrate the cellular surface and mucus covering the cells becomes thick and sticky

Question 3

what does a defective CFTR produce?

Answer 3

it appears to produce a redox imbalance in epi cells and extracell fluids and an abnormal generation of ROS

massive defective CFTR gives rise to extracell GSH level DECREASE and ELEVATED glucose concentrations in the airway surface liquid –> encouraging lung infection by pathogens in CF advancement

Question 4

what cell can alleviate CF?

Answer 4

mitochondria is said to have a leading role in oppsoing the high ASL glucose level and oxidative stress in CF