Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation

Journal of Biological Chemistry

Volumn 271, Issue 2, 1996, Pages 635-638

  Sato, Sachiko ^a   Ward, Cristina L ^a   Krouse, Mauri E ^a   Wine, Jeffrey J ^a   Kopito, Ron R ^a  

^a STANFORD UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE CHANNEL; CHLORIDE ION; COMPLEMENTARY DNA; GLYCEROL;

ANIMAL CELL; ARTICLE; CELL MEMBRANE CONDUCTANCE; CHLORIDE CONDUCTANCE; CONTROLLED STUDY; CYSTIC FIBROSIS; ENDOPLASMIC RETICULUM; GENE MUTATION; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; PROTEIN FOLDING; TEMPERATURE SENSITIVITY;

CELLS, CULTURED; CHLORIDES; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; GLYCEROL; HUMANS; MUTATION; PROTEIN FOLDING; SOLVENTS;

EID: 0030042386     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.271.2.635     Document Type: Article

References (22)

1. Welsh, M. J., and Smith, A. E. (1993) Cell 73, 1251-1254
2. Kerem, B., Zielenski, J., Markiewicz, D., Bozon, D., Gazit, E., Yahav, J., Kennedy, D., Riordan, J. R., Collins, F. S., Rommens, J. M., and Tsui, L. (1990) Proc. Natl. Acad. Sci. U. S. A. 87, 8447-8451
3. Tsui, L.-C. (1992) Trends Genet. 8, 392-398
4. Cheng, S. H., Gregory, R. J., Marshall, J., Paul, S., Souza, D. W., White, G. A., O'Riordan, C. R., and Smith, A. E. (1990) Cell 63, 827-834
5. Ward, C. L., and Kopito, R. R. (1994) J. Biol. Chem. 269, 25710-25718
6. Lukacs, G. L., Mohamed, A., Kartner, N., Chang, X.-B., Riordan, J. R., and Grinstein, S. (1994) EMBO J. 13, 6076-6086
7. Ward, C. L., and Kopito, R. R. (1995) Cell 83, 121-127
8. Cheng, S. H., Fang, S. L., Zabner, J., Marshall, J., Piraino, S., Schiavi, S. C., Jefferson, D. M., Welsh, M. J., and Smith, A. E. (1995) Am. J. Physiol. 268, L615-L624
9. Denning, G. M., Anderson, M. P., Amara, J. F., Marshall, J., Smith, A. E., and Welsh, M. J. (1992) Nature 358, 761-764
10. Gekko, K., and Timasheff, S. N. (1981) Biochemistry 20, 4667-4676
11. Sawano, H., Koumoto, Y., Ohta, K., Sasaki, Y., Segawa, S., and Tachibana, H. (1992) FEBS Lett. 303, 11-14
12. Shelanski, M. L., Gaskin, P., and Cantor, C. R. (1973) Proc. Natl. Acad. Sci. U. S. A. 70, 765-768
13. Gunderson, K. L., and Kopito, R. R. (1995) Cell 82, 231-239
14. Restrepo, D., Kozody, D. J., Spinelli, L. J., and Knauf, P. A. (1989) Am. J. Physiol. 257, C520-C527
15. Fukuda, M., Guan, J. L., and Rose, J. K (1988) J. Biol. Chem. 263, 5314-5318
16. Marshall, J., Fang, S., Ostedgaard, L. S., O'Riordan, C. R., Ferrara, D., Amara, J. F., Hoppe, H., IV. Scheule, R. K., Welsh, M. J., Smith, A. E., and Cheng, S. H. (1994) J. Biol. Chem. 269, 2978-2995
17. Ishibashi, K., Sasaki, S., Fushimi, K., Uchida, S., Kuwahara, M., Saito, H., Furukawa, T., Nakajima, K., Yamaguchi, Y., and Gojobori, T. (1994) Proc. Natl. Acad. Sci. U. S. A. 91, 6269-6273
18. Ma, T., Frigeri, A., Hasegawa, H., and Verkman, A. S. (1994) J. Biol. Chem. 269, 21845-21849
19. Dalemans, W., Barbry, P., Champigny, G., Jallat, S., Dott, K., Dreyer, D., Crystal, R. G., Pavirani, A., Lecocq, J. P., and Lazdunski, M. (1991) Nature 354, 526-528
20. Hawthorne, D. C., and Friis, J. (1964) Genetics 50, 829-839
21. Russell, R. R. (1972) J. Bacterial. 112, 661-665
22. Yancey, P. H., Clark, M. E., Hand, S. C., Bowlus, R. D., and Somero, G. N. (1982) Science 217, 1214-1222