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8 Cards in this Set
- Front
- Back
Most common manifestation of CF?
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Pancreatic insufficiency
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Most common pathogen found in CF?
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Pseudomonas
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90% of people with CF have this pulmonary function disease?
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COPD
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CFTR gene?
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7q31-32
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What does the mutation do?
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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.
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What is wrong with the CFTR protein that causes its degredation in a class II mutation?
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-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 |
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Why does it affect males more?
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it is a obstructive disease of ducts, the vas deferens is a smaller duct
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What does the normal CFTR protein have to do with pseudomonas?
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-it has some sort of magical power to internalize the bug
-it binds the LPS and internalizes it |