[HTML][HTML] Differential sensitivity of the cystic fibrosis (CF)-associated mutants G551D and G1349D to potentiators of the cystic fibrosis transmembrane conductance …
Z Cai, A Taddei, DN Sheppard - Journal of Biological Chemistry, 2006 - ASBMB
The genetic disease cystic fibrosis (CF) is caused by loss of function of the cystic fibrosis
transmembrane conductance regulator (CFTR) Cl–channel. Two CF mutants, G551D and
G1349D, affect equivalent residues in the highly conserved LSGGQ motifs that are essential
components of the ATP-binding sites of CFTR. Both mutants severely disrupt CFTR channel
gating by decreasing mean burst duration (MBD) and prolonging greatly the interburst
interval (IBI). To identify small molecules that rescue the gating defects of G551D-and …
transmembrane conductance regulator (CFTR) Cl–channel. Two CF mutants, G551D and
G1349D, affect equivalent residues in the highly conserved LSGGQ motifs that are essential
components of the ATP-binding sites of CFTR. Both mutants severely disrupt CFTR channel
gating by decreasing mean burst duration (MBD) and prolonging greatly the interburst
interval (IBI). To identify small molecules that rescue the gating defects of G551D-and …