[Term]
id: PR:Q1LX78
name: cystic fibrosis transmembrane conductance regulator (zebrafish)
def: "A cystic fibrosis transmembrane conductance regulator that is encoded in the genome of zebrafish." [OMA:Q1LX78, PRO:DNx]
comment: Category=organism-gene.
synonym: "cftr" RELATED Gene-based [UniProtKB:Q1LX78]
synonym: "z-CFTR" EXACT PRO-short-label [PRO:DNx]
synonym: "cAMP-dependent chloride channel (zebrafish)" EXACT [UniProtKB:Q1LX78]
synonym: "channel conductance-controlling ATPase (zebrafish)" EXACT [UniProtKB:Q1LX78]
synonym: "ATP-binding cassette sub-family C member 7 (zebrafish)" EXACT [UniProtKB:Q1LX78]
xref: UniProtKB:Q1LX78
intersection_of: only_in_taxon NCBITaxon:7955 ! Danio rerio
intersection_of: PR:000001044 ! cystic fibrosis transmembrane conductance regulator
relationship: has_gene_template ZFIN:ZDB-GENE-050517-20 ! cftr (zebrafish)

[Term]
id: PR:000001044
name: cystic fibrosis transmembrane conductance regulator
def: "A chloride channel 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." [PMID:9922375, PRO:CNA]
comment: Category=gene. Note: The open conformation of the CFTR channel is stabilized via interaction of its N-terminal region with other proteins, most notably filamin. [PRO:DAN, PMID:29779042]
synonym: "CFTR" EXACT PRO-short-label [PRO:DNx]
synonym: "ABCC7" RELATED Gene-based []
synonym: "cAMP-dependent chloride channel" EXACT []
synonym: "channel conductance-controlling ATPase" EXACT []
synonym: "ATP-binding cassette sub-family C member 7" EXACT []
synonym: "ATP-binding cassette transporter sub-family C member 7" EXACT []
xref: IUPHARobj:707
xref: PIRSF:PIRSF002777
is_a: PR:000044678 ! chloride channel protein