The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
ATPase Activity of the Cystic Fibrosis Transmembrane Conductance Regulator
Open accessAbstract
The gene mutated in cystic fibrosis codes for the cystic fibrosis transmembrane conductance regulator (CFTR), a cyclic AMP-activated chloride channel thought to be critical for salt and water transport by epithelial cells. Plausible models exist to describe a role for ATP hydrolysis in CFTR channel activity; however, biochemical evidence that CFTR possesses intrinsic ATPase activity is lacking. In this study, we report the first measurements of the rate of ATP hydrolysis by purified, reconstituted CFTR. The mutation CFTRG551D resides within a motif conserved in many nucleotidases and is known to cause severe human disease. Following reconstitution the mutant protein exhibited both defective ATP hydrolysis and channel gating, providing direct evidence that CFTR utilizes ATP to gate its channel activity.
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Citations
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
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Cited by
Structure and Function of the CFTR Chloride Channel
SHEPPARD, D. N., & WELSH, M. J. (1999) · Physiological Reviews · doi:10.1152/physrev.1999.79.1.s23 · Cited by 1,018
Deeley, R. G., Westlake, C., & Cole, S. P. C. (2006) · Physiological Reviews · doi:10.1152/physrev.00035.2005 · Cited by 761
CFTR Function and Prospects for Therapy
Riordan, J. R. (2008) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.75.103004.142532 · Cited by 610
Physiological Substrates of cAMP-Dependent Protein Kinase
Shabb, J. B. (2001) · Chemical Reviews · doi:10.1021/cr000236l · Cited by 373
Structure, Gating, and Regulation of the CFTR Anion Channel
Csanády, L., Vergani, P., & Gadsby, D. C. (2019) · Physiological Reviews · doi:10.1152/physrev.00007.2018 · Cited by 262
Metadata
- Authors
- Li, C., Ramjeesingh, M., Wang, W., Garami, E., Hewryk, M., Lee, D., Rommens, J. M., Galley, K., & Bear, C. E.
- Venue
- Journal of Biological Chemistry
- Publisher
- Elsevier BV
- Published
- 1996-11
- Volume
- 271
- Issue
- 45
- Pages
- 28463-28468
- ISSN
- 0021-9258 (ISSN-L)
- OpenAlex
- W1982625622
- Cited by
- 267
- References
- 1
- FWCI
- 19.87
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Li, C., Ramjeesingh, M., Wang, W., Garami, E., Hewryk, M., Lee, D., Rommens, J. M., Galley, K., & Bear, C. E. (1996). ATPase Activity of the Cystic Fibrosis Transmembrane Conductance Regulator. Journal of Biological Chemistry. https://doi.org/10.1074/jbc.271.45.28463
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
