Protein: Cystic fibrosis transmembrane conductance regulator (EC 5.6.1.6) (ATP-binding cassette sub-family C member 7) (Channel conductance-controlling ATPase) (cAMP-dependent chloride channel) (Q1LX78)
Organism: Danio rerio (Zebrafish) (Brachydanio rerio)
Function: Epithelial ion channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis. Mediates the transport of chloride ions across the cell membrane (By similarity). Channel activity is coupled to ATP hydrolysis. The ion channel is also permeable to HCO(3)(-); selectivity depends on the extracellular chloride concentration. Exerts its function also by modulating the activity of other ion channels and transporters. Contributes to the regulation of the pH and the ion content of the epithelial fluid layer (By similarity). Required for normal fluid homeostasis in the gut. Required for normal volume expansion of Kupffer's vesicle during embryonic development and for normal establishment of left-right body patterning. Required for normal resistance to infection by P.aeruginosa strain PA14 and strain SMC573.
Subcellular location: Apical cell membrane {ECO:0000269|PubMed:20933420, ECO:0000269|PubMed:23487313, ECO:0000269|PubMed:25592226}; Multi-pass membrane protein {ECO:0000269|PubMed:27912062}. Early endosome membrane {ECO:0000250|UniProtKB:P13569}; Multi-pass membrane protein {ECO:0000269|PubMed:27912062}. Cell membrane {ECO:0000269|PubMed:23487313, ECO:0000305|PubMed:27912062}; Multi-pass membrane protein {ECO:0000269|PubMed:27912062}. Recycling endosome membrane {ECO:0000250|UniProtKB:P13569}; Multi-pass membrane protein {ECO:0000269|PubMed:27912062}. Endoplasmic reticulum membrane {ECO:0000250|UniProtKB:P13569}; Multi-pass membrane protein {ECO:0000269|PubMed:27912062}. Note=The channel is internalized from the cell surface into an endosomal recycling compartment, from where it is recycled to the cell membrane. {ECO:0000250|UniProtKB:P13569}.
Interaction partners:
- Na
- Tight junction protein 1a
- Golgi-associated PDZ and coiled-coil motif-containing protein
- E3 ubiquitin-protein ligase CHIP
- Ubiquitin carboxyl-terminal hydrolase.
- Transitional endoplasmic reticulum ATPase
- Derlin
- E3 ubiquitin-protein ligase RNF185
- Ring finger protein 5.
- Na
Sequence:
MQRSPVEDANCLSRYFFWWTNPIMRKGFKEKLRPSDVYQAPSQDAADILAERLEKEWDREVASGKKKPSLLRAMARCYIKPFLLFGFLLYIGEATKTVQPQLLGRIIASFDPAHEPERANGYFLAFGLGLLFTARFLLLQPAMFGLHHLGMQIRIALFSIIYKKTLKLSSRVLDKISTGQLVSLMSANLGKFDQSLGMAHFIWISPLQCILCTGLIWELIDVNSFCALAAISLLGVLQAFLSHKMGPYKAQKVLLTNKRLALTSEIMENLHSVKAYGWEEIMETLIKNIRQDEVKLTRKIGSLRYFYSSAYFFSAIFVIVAAVVPHALSRGINLRRIFTTLSYCMVLRMTVTRQLPGSIQMWYDTMRLIWKIEEFLSKEEYKLMEYDLSITELELQDVTASWDEGPGELLERIKQENKANGHHNGDAGLFFTNLYVAPVLKDISLKLKKGEMLAVTGSMGSGKSSLLMTILGELVPSSGKIRHSGRISYSSQTAWIMPGTIRDNILFGLTYDEYRYKSVVKACQLEEDLAALPEKDKTPMAEGGLNLSGGQKARVALARAVYRDADLYLLDAPFTHLDIATEKEIFDKCLCKLMASKTRILVTNKIEHLKRADKILLLHNGESFFYGTFPELQSERPDFSSLLLGLEAYDNISAERRCSILTETLHRVSVDESAGMQPERSAFRQVPPTKPMYIDERKASVIVNPLGVARKASFIQVPEEEVRRTLPDRKFSLVPENELVDESFMGSDVYHNHGVHMAGQRRQSVLAFMTNAQGQGRREHLQSSFRRRLSVVPQSELASELDIYTRRLSDSTYDMTGILEEENIEACLTDEIDEIEETFETTKWNTYVRYVSNNKSLLYVLIFILFIAAIEIAGSVAGIFLITDELWREEHQRSEPNMTKHSNASSSGQTYAITVTPTSSYYILYIYVATSESLLAMGFFRGLPFVHTTITISKKLHQKMLHAVLSAPMSVLNTMKTGRIMNRFTKDMATIDDMLPLLMFDFVQLTVVVVGCILVVSIVRPYIFLAATPLAIIFIVMRKYFLRTGQQLKQLETEARSPIFSHLIMSLKGLWTIRAFERQAYFEALFHKTLNTHTATWFLYLSTLRWFLFRADILFVFFFTLAAWIAVGTNQDKPGEIGIIICLAMLILGTFQWCVATSIAVDGMMRSVDRVFKFIDLPSETPKPDKGKDSDLIIENVDAQADSSWPHRGQIEVRNLTVKYTEAGHAVLKNLSFSAEGRQRVGILGRTGSGKSSLFNALLKLVYTDGEISIDGVNWNKMPLQKWRKAFGVVPQKVFIFTGPLRMNLDPYGCHSDEELWRVAEEVGLKTVIEQFPDKLDFQLEYGGYVLSNGHKQLICLARSILSGARILLLDEPSAHLDPVTIKVLKKTLRQSFSTCTILLSEHKVEPLLECQSFLMMDKGQVKTYDSIQKLLNETSHLKQAISPAERLKLFPRRNSSMRTPQSKLSSVTQTLQEEAEDNIQDTRL