The DOI matches its Crossref record; 2 of the 2 references it cites were found in a registry or an index.
COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code
Open accessAbstract
Cystic fibrosis (CF) is a childhood hereditary disease in which the most common mutant form of the CF transmembrane conductance regulator (CFTR) ΔF508 fails to exit the endoplasmic reticulum (ER). Export of wild-type CFTR from the ER requires the coat complex II (COPII) machinery, as it is sensitive to Sar1 mutants that disrupt normal coat assembly and disassembly. In contrast, COPII is not used to deliver CFTR to ER-associated degradation. We find that exit of wild-type CFTR from the ER is blocked by mutation of a consensus di-acidic ER exit motif present in the first nucleotide binding domain. Mutation of the code disrupts interaction with the COPII coat selection complex Sec23/Sec24. We propose that the di-acidic exit code plays a key role in linking CFTR to the COPII coat machinery and is the primary defect responsible for CF in ΔF508-expressing patients.
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Chardin, P., & McCormick, F. (1999) · Cell · doi:10.1016/s0092-8674(00)80724-2 · Cited by 407
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Meacham, G. C., Lu, Z., King, S., Sorscher, E., Tousson, A., & Cyr, D. M. (1999) · The EMBO Journal · doi:10.1093/emboj/18.6.1492 · Cited by 335
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Wang, X., Venable, J., LaPointe, P., Hutt, D. M., Koulov, A. V., Coppinger, J., Gurkan, C., Kellner, W., Matteson, J., Plutner, H., et al. (2006) · Cell · doi:10.1016/j.cell.2006.09.043 · Cited by 601
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Regulation of G protein-coupled receptor export trafficking
Dong, C., Filipeanu, C. M., Duvernay, M. T., & Wu, G. (2007) · Biochimica et Biophysica Acta (BBA) - Biomembranes · doi:10.1016/j.bbamem.2006.09.008 · Cited by 278
Metadata
- Authors
- Wang, X., Matteson, J., An, Y., Moyer, B., Yoo, J.-S., Bannykh, S., Wilson, I. A., Riordan, J. R., & Balch, W. E.
- Venue
- The Journal of Cell Biology
- Publisher
- Rockefeller University Press
- Published
- 2004-10-11
- Volume
- 167
- Issue
- 1
- Pages
- 65-74
- ISSN
- 0021-9525 (ISSN-L)
- 1540-8140
- OpenAlex
- W2141857608
- Cited by
- 222
- References
- 2
- FWCI
- 5.34
- Type
- journal article
- Language
- en
- Open access
- green
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- Wang, X., Matteson, J., An, Y., Moyer, B., Yoo, J.-S., Bannykh, S., Wilson, I. A., Riordan, J. R., & Balch, W. E. (2004). COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code. The Journal of Cell Biology. https://doi.org/10.1083/jcb.200401035
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