Nasal Potential Difference in Cystic Fibrosis considering Severe CFTR Mutations

Figure 1

(a) CFTR mutations distributed by exon/intron localization and class. (b) CFTR gene with exon and intron descriptions. (c) CFTR protein domains. (d) For all the data, consider the following: (A) cystic fibrosis patients with two class I, II, or III CFTR mutations; (B) cystic fibrosis patients with two class IV, V, or VI CFTR mutations; and (C) healthy subjects. The comparison was made using the Mann-Whitney test considering the following associations: A versus B, A versus C, and B versus C. α considered was 0.05. For the finger (mV), ^∗; ^# = 0.021; ^¥ = 0.003. For the PDMax (mV), ; ^# = 0.040; ^¥. For the Δamiloride (mV), ^∗; ^#; ^¥. For the Δchloride-free + isoproterenol, ^∗; ^# = 0.007; ^¥. For the Wilschanski index, ^∗ = 0.050; ^# = 0.002; ^¥. The maximum difference was observed by finger (B versus C) and for the Wilschanski index (A versus C). CFTR = cystic fibrosis transmembrane regulator, MSD = membrane-spanning domains, NBD = nucleotide-binding domains, R = regulator, and mV = millivolts.