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8 Cards in this Set

  • Front
  • Back
Most common manifestation of CF?
Pancreatic insufficiency
Most common pathogen found in CF?
Pseudomonas
90% of people with CF have this pulmonary function disease?
COPD
CFTR gene?
7q31-32
What does the mutation do?
The chloride ion channel protein is messed up. You get increased intracellular sodium in the lungs and an increase in sodium and chloride in sweat.
What is wrong with the CFTR protein that causes its degredation in a class II mutation?
-it is not glycosylated and causes a defect in the protein, undergoes accelerated degredation in the ER
-this is from abnormal trafficking that leads to its degredation before it reaches the membrane
Why does it affect males more?
it is a obstructive disease of ducts, the vas deferens is a smaller duct
What does the normal CFTR protein have to do with pseudomonas?
-it has some sort of magical power to internalize the bug
-it binds the LPS and internalizes it