Revisiting Cystic Fibrosis Transmembrane Conductance Regulator Structure and Function

摘要: The cystic fibrosis transmembrane conductance regulator (CFTR) is a channel/enzyme which mediates passive diffusion of chloride and bicarbonate through epithelial cell membranes. It expressed in many types throughout the body, but airways it found mainly secretory serous cells submucosal glands. CFTR belongs to large super-family ATP binding cassette transporters that have two nucleotide domains with characteristic sequences or “motifs.” Although most other consume actively transport various substrates, interactions control opening closing channel pore (i.e., gating). Recent high resolution structures bacterial combined new biochemical electrophysiological studies itself led major advances our understanding gating. For example, now clear ATPase activity not strictly...