CFTR Protein (Cystic Fibrosis)

Question 1

What is cystic fibrosis?

Answer 1

• Genetic disorder that impairs the lungs and gastrointestinal tract

Question 2

What is cystic fibrosis caused by?

Answer 2

• Mutations to a single gene that codes for the CFTR protein

Question 3

What does CFTR stand for?

Answer 3

cystic fibrosis transmembrane conductance regulator

Question 4

In properly functioning cells what happens?

Answer 4

• CFTR acts as a membrane transport protein
• It helps move Cl- ions out of the cells that line the lungs and intestinal tract and into surrounding mucous

Question 5

What is the outcome of CFTR functioning properly?

Answer 5

• Results in an electrical gradient across the membrane
• Leads to the movement of Na+ ions in the same direction as the chloride
• Na+ and Cl- concentrations cause water to move into the mucous lining

Question 6

In individuals with cystic fibrosis what happens?

Answer 6

• The Cl- channel of CFTR malfunctions and water is retained within the cells
• A lack of moisture in the mucous, makes it very thick

Question 7

Negative effect of having cystic fibrosis

Answer 7

• Breathing becomes difficult because mucous blocks the airways
• More susceptible to bacterial infections
• Mucous clogs pancreatic ducts, blocking enzymes that would normally enter the intestine
• Destroys pancreas and ability to make necessary digestive enzymes

Question 8

Treatments for cystic fibrosis patients

Answer 8

• Take dietary supplements to survive
• No cure
• Lung transplants is an option
• Gene therapy