Ivacaftor restores only 40 to 50% of ∆508 CFTR protein activity in individuals with cystic fibrosis. Why doesn’t Ivacaftor restore the ∆508 CFTR function to 100% of the native CFTR function?

Question

Accepted Answer

At any point in time, a portion of the ∆508 CFTR proteins will be bound to ivacaftor, whereas another portion won’t be

Answer

Ivacaftor's effectiveness is limited by the cell's ability to produce enough ATP to fuel the increased CFTR channel activity

Answer

Ivacaftor can only partially correct the misfolding of ∆508 CFTR

Answer

The cellular machinery can only process and transport a limited amount of CFTR to the cell surface, regardless of ivacaftor's presence

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Ivacaftor restores only 40 to 50% of ∆508 CFTR protein activity in individuals w...

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