3.A.1.202.2
CFTR, an epithelial ion channel, plays a role in the regulation of epithelial ion and water transport
and fluid homeostasis (Bagnat et al. 2010; Navis et al. 2013; Navis and Bagnat 2015). It mediates the transport of chloride ions across the cell membrane. Channel activity is coupled to ATP hydrolysis. The ion
channel is also permeable to HCO3-; selectivity depends on
the extracellular chloride concentration. CFTR exerts its function in part by
modulating the activity of other ion channels and transporters, and it contributes to the regulation of the pH and the ion content of the
epithelial fluid layer. It is required for normal fluid
homeostasis in the gut (Bagnat et al. 2010) and for normal volume expansion of Kupffer's vesicle during
embryonic development as well as for normal establishment of left-right body
patterning (Navis et al. 2013; Roxo-Rosa et al. 2015). It is also required for normal resistance to infection by Pseudomonas aeruginosa (Phennicie et al. 2010).
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| Accession Number: | Q1LX78 |
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| Protein Name: | Cystic fibrosis transmembrane conductance regulator |
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| Length: | 1485 |
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| Molecular Weight: | 168400.00 |
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| Species: | Danio rerio (Zebrafish) [7955] |
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| Number of TMSs: | 12 |
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| Location1 / Topology2 / Orientation3: |
Apical cell membrane1 / Multi-pass membrane protein2 |
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| Substrate |
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1: MQRSPVEDAN CLSRYFFWWT NPIMRKGFKE KLRPSDVYQA PSQDAADILA ERLEKEWDRE
61: VASGKKKPSL LRAMARCYIK PFLLFGFLLY IGEATKTVQP QLLGRIIASF DPAHEPERAN
121: GYFLAFGLGL LFTARFLLLQ PAMFGLHHLG MQIRIALFSI IYKKTLKLSS RVLDKISTGQ
181: LVSLMSANLG KFDQSLGMAH FIWISPLQCI LCTGLIWELI DVNSFCALAA ISLLGVLQAF
241: LSHKMGPYKA QKVLLTNKRL ALTSEIMENL HSVKAYGWEE IMETLIKNIR QDEVKLTRKI
301: GSLRYFYSSA YFFSAIFVIV AAVVPHALSR GINLRRIFTT LSYCMVLRMT VTRQLPGSIQ
361: MWYDTMRLIW KIEEFLSKEE YKLMEYDLSI TELELQDVTA SWDEGPGELL ERIKQENKAN
421: GHHNGDAGLF FTNLYVAPVL KDISLKLKKG EMLAVTGSMG SGKSSLLMTI LGELVPSSGK
481: IRHSGRISYS SQTAWIMPGT IRDNILFGLT YDEYRYKSVV KACQLEEDLA ALPEKDKTPM
541: AEGGLNLSGG QKARVALARA VYRDADLYLL DAPFTHLDIA TEKEIFDKCL CKLMASKTRI
601: LVTNKIEHLK RADKILLLHN GESFFYGTFP ELQSERPDFS SLLLGLEAYD NISAERRCSI
661: LTETLHRVSV DESAGMQPER SAFRQVPPTK PMYIDERKAS VIVNPLGVAR KASFIQVPEE
721: EVRRTLPDRK FSLVPENELV DESFMGSDVY HNHGVHMAGQ RRQSVLAFMT NAQGQGRREH
781: LQSSFRRRLS VVPQSELASE LDIYTRRLSD STYDMTGILE EENIEACLTD EIDEIEETFE
841: TTKWNTYVRY VSNNKSLLYV LIFILFIAAI EIAGSVAGIF LITDELWREE HQRSEPNMTK
901: HSNASSSGQT YAITVTPTSS YYILYIYVAT SESLLAMGFF RGLPFVHTTI TISKKLHQKM
961: LHAVLSAPMS VLNTMKTGRI MNRFTKDMAT IDDMLPLLMF DFVQLTVVVV GCILVVSIVR
1021: PYIFLAATPL AIIFIVMRKY FLRTGQQLKQ LETEARSPIF SHLIMSLKGL WTIRAFERQA
1081: YFEALFHKTL NTHTATWFLY LSTLRWFLFR ADILFVFFFT LAAWIAVGTN QDKPGEIGII
1141: ICLAMLILGT FQWCVATSIA VDGMMRSVDR VFKFIDLPSE TPKPDKGKDS DLIIENVDAQ
1201: ADSSWPHRGQ IEVRNLTVKY TEAGHAVLKN LSFSAEGRQR VGILGRTGSG KSSLFNALLK
1261: LVYTDGEISI DGVNWNKMPL QKWRKAFGVV PQKVFIFTGP LRMNLDPYGC HSDEELWRVA
1321: EEVGLKTVIE QFPDKLDFQL EYGGYVLSNG HKQLICLARS ILSGARILLL DEPSAHLDPV
1381: TIKVLKKTLR QSFSTCTILL SEHKVEPLLE CQSFLMMDKG QVKTYDSIQK LLNETSHLKQ
1441: AISPAERLKL FPRRNSSMRT PQSKLSSVTQ TLQEEAEDNI QDTRL