Fig. 6.

Quantitative gating activity of CFTR. #1 is a healthy control subject (its activity is considered 100%); #2 and #3 are two CF patients compound heterozygous for two severe mutations each (i.e., F508del/F508del for case #2 and G542X/4016insT for case #3): they show an activity of 9.9% and 10.4% compared to the control, respectively; case #4 is a CF patient with a severe and a mild CF mutation (i.e., W1282X/D1152H) with 20.3% of activity. Finally, case #5 is a heterozygous carrier of the severe G542X mutation with an activity of 76.8%.