5uar

Structural highlights

Function

CFTR_DANRE Epithelial ion channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis (PubMed:20933420, PubMed:23487313, PubMed:25592226). 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 (PubMed:20933420). Required for normal volume expansion of Kupffer's vesicle during embryonic development and for normal establishment of left-right body patterning (PubMed:23487313, PubMed:26432887). Required for normal resistance to infection by P.aeruginosa strain PA14 and strain SMC573 (PubMed:20732993).[UniProtKB:P13569]^{[1] [2] [3] [4] [5]}

See Also

• ABC transporter 3D structures

References

1. ↑ Phennicie RT, Sullivan MJ, Singer JT, Yoder JA, Kim CH. Specific resistance to Pseudomonas aeruginosa infection in zebrafish is mediated by the cystic fibrosis transmembrane conductance regulator. Infect Immun. 2010 Nov;78(11):4542-50. doi: 10.1128/IAI.00302-10. Epub 2010 Aug, 23. PMID:20732993 doi:http://dx.doi.org/10.1128/IAI.00302-10
2. ↑ Bagnat M, Navis A, Herbstreith S, Brand-Arzamendi K, Curado S, Gabriel S, Mostov K, Huisken J, Stainier DY. Cse1l is a negative regulator of CFTR-dependent fluid secretion. Curr Biol. 2010 Oct 26;20(20):1840-5. doi: 10.1016/j.cub.2010.09.012. Epub 2010, Oct 7. PMID:20933420 doi:http://dx.doi.org/10.1016/j.cub.2010.09.012
3. ↑ Navis A, Marjoram L, Bagnat M. Cftr controls lumen expansion and function of Kupffer's vesicle in zebrafish. Development. 2013 Apr;140(8):1703-12. doi: 10.1242/dev.091819. Epub 2013 Mar 13. PMID:23487313 doi:http://dx.doi.org/10.1242/dev.091819
4. ↑ Roxo-Rosa M, Jacinto R, Sampaio P, Lopes SS. The zebrafish Kupffer's vesicle as a model system for the molecular mechanisms by which the lack of Polycystin-2 leads to stimulation of CFTR. Biol Open. 2015 Oct 2;4(11):1356-66. doi: 10.1242/bio.014076. PMID:26432887 doi:http://dx.doi.org/10.1242/bio.014076
5. ↑ Navis A, Bagnat M. Loss of cftr function leads to pancreatic destruction in larval zebrafish. Dev Biol. 2015 Mar 15;399(2):237-48. doi: 10.1016/j.ydbio.2014.12.034. Epub 2015 , Jan 13. PMID:25592226 doi:http://dx.doi.org/10.1016/j.ydbio.2014.12.034