CFTR: New insights into structure and function and implications for modulation by small molecules

Mutations in the Cystic Fibrosis (CF) gene alter the structure and function of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) channel, impairing the flow of chloride and bicarbonate ions across epithelia in many organs of the body [1]. CFTR shares an overall architecture that is conserved in ATP-binding cassette (ABC) transporters and consists of two transmembrane domains (TMD1 and TMD2), two nucleotide-binding domains (NBD1 and NBD2), and a unique regulatory (R)-region (Fig. 1A) [2].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Original Article Source Type: research