Fig. 1

Schematic illustration of Δ(G970-T1122)-CFTR. a The gene mutation identified most frequently in Japanese CF patients. A massive deletion of 459 base pairs, which results in a deletion of 153 amino acids from glycine at position 970 (G970) to threonine at 1122 (T1122). b Topological location of the deleted region G970-T1122 in the membrane. The amino acid deletion is over three transmembrane helices TM9–TM11. Asn894 and Asn900 on the fourth extracellular loop are consensus sites for N-glycosylation. NBD nucleotide binding domain, R regulatory domain, TMD transmembrane domain