The DOI matches its Crossref record; 2 of the 2 references it cites were found in a registry or an index.
Structure and Function of the CFTR Chloride Channel
Open accessAbstract
Sheppard, David N., and Michael J. Welsh. Structure and Function of the CFTR Chloride Channel. Physiol. Rev. 79 , Suppl.: S23–S45, 1999. — The cystic fibrosis transmembrane conductance regulator (CFTR) is a unique member of the ABC transporter family that forms a novel Cl−channel. It is located predominantly in the apical membrane of epithelia where it mediates transepithelial salt and liquid movement. Dysfunction of CFTR causes the genetic disease cystic fibrosis. The CFTR is composed of five domains: two membrane-spanning domains (MSDs), two nucleotide-binding domains (NBDs), and a regulatory (R) domain. Here we review the structure and function of this unique channel, with a focus on how the various domains contribute to channel function. The MSDs form the channel pore, phosphorylation of the R domain determines channel activity, and ATP hydrolysis by the NBDs controls channel gating. Current knowledge of CFTR structure and function may help us understand better its mechanism of action, its role in electrolyte transport, its dysfunction in cystic fibrosis, and its relationship to other ABC transporters.
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Cites
Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
Riordan, J. R., Rommens, J. M., Kerem, B.-S., Alon, N., Rozmahel, R., Grzelczak, Z., Zielenski, J., Lok, S., Plavsic, N., Chou, J.-L., et al. (1989) · Science · doi:10.1126/science.2475911 · Cited by 7,418
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
Welsh, M. J., & Smith, A. E. (1993) · Cell · doi:10.1016/0092-8674(93)90353-r · Cited by 1,485
Self-citation
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Torres, V. E., Harris, P. C., & Pirson, Y. (2007) · The Lancet · doi:10.1016/s0140-6736(07)60601-1 · Cited by 1,550
Molecular Structure and Physiological Function of Chloride Channels
Jentsch, T. J., Stein, V., Weinreich, F., & Zdebik, A. A. (2002) · Physiological Reviews · doi:10.1152/physrev.00029.2001 · Cited by 1,256
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Du, K., Sharma, M., & Lukacs, G. L. (2005) · Nature Structural & Molecular Biology · doi:10.1038/nsmb882 · Cited by 356
Cooperative Assembly and Misfolding of CFTR Domains In Vivo
Du, K., & Lukacs, G. L. (2009) · Molecular Biology of the Cell · doi:10.1091/mbc.e08-09-0950 · Cited by 147
Metadata
- Authors
- SHEPPARD, D. N., & WELSH, M. J.
- Venue
- Physiological Reviews
- Publisher
- American Physiological Society
- Published
- 1999-01-01
- Volume
- 79
- Issue
- 1
- Pages
- S23-S45
- ISSN
- 0031-9333 (ISSN-L)
- 1522-1210
- OpenAlex
- W1745204207
- Cited by
- 1,018
- References
- 2
- FWCI
- 6.69
- Type
- journal article
- Language
- en
- Open access
- bronze
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