protein 蛋白质 cystic fibrosis transmembrane conductance regulator A protein that is a translation product of the human CFTR gene or a 1:1 ortholog thereof. Involved in the transport of chloride ions. The characteristic domain composition of the functional isoform consists of two modules of a membrane-spanning domain (ABC transporter transmembrane region) followed by a nucleotide-binding domain (ABC transporter domains), separated by an intracellular regulatory domain (R-domain). It also contains a C-terminal PDZ-binding region. The R-domain is an inhibitory domain of CFTR,and phosphorylation of the R-domain removes this inhibition, allowing activation of chloride flux. ABCC7 ATP-binding cassette transporter sub-family C member 7 CFTR Category=gene. PIRSF:PIRSF002777 PR:000001044 cAMP-dependent chloride channel channel conductance-controlling ATPase protein