Expression and characterization of two pathogenic mutations in human electron transfer flavoprotein

Journal of Biological Chemistry

Volumn 272, Issue 42, 1997, Pages 26425-26433

  Salazar, Denise ^a   Zhang, Lening ^a   DeGala, Gregory D ^a   Frerman, Frank E ^a,b  

^a Children's Hospital Colorado   (United States)

^b UNIVERSITY OF COLORADO SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHAPERONIN; FLAVOPROTEIN; MUTANT PROTEIN;

ARTICLE; CIRCULAR DICHROISM; CRYSTAL STRUCTURE; ELECTRON TRANSPORT; ENZYME DEFECT; ESCHERICHIA COLI; GENE EXPRESSION; HUMAN; NONHUMAN; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN CONFORMATION; PROTEIN EXPRESSION; PROTEIN FOLDING; PROTEIN PURIFICATION; PROTEIN STABILITY; PROTEIN STRUCTURE; THERMODYNAMICS;

CLONING, MOLECULAR; ELECTRON-TRANSFERRING FLAVOPROTEINS; FLAVINS; FLAVOPROTEINS; HUMANS; MUTAGENESIS, SITE-DIRECTED; MUTATION; OXIDATION-REDUCTION; PROTEIN BINDING;

EID: 0030698002     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.272.42.26425     Document Type: Article

References (37)

1. Thorpe, C. (1991) in Chemistry and Biochemistry of Flavoenzymes (Müller, F., ed) Vol. II, pp. 471-486, CRC Press, Boca Raton, FL
2. Frerman, F. E., and Goodman, S. I. (1985) Proc. Natl. Acad. Sci. U. S.A. 82, 4517-4520
3. Frerman, F. E., and Goodman, S. I. (1985) Biochem. Med. 33, 38-43
4. Gorelick, R. J., Schopfer, L. M., Ballou, D. P., Massey, V., and Thorpe, C. (1985) Biochemistry 24, 6830-6839
5. Herrick, K. R., Salazar, D., Goodman, S. I., Finocchiaro, G., Bedzyk, L. A., and Frerman, F. E. (1994) J. Biol. Chem. 269, 32239-32245
6. Ramsay, R. R., Steenkamp, D. J., and Husain, M. (1987) Biochemistry J. 241, 883-892
7. Roberts, D. L., Frerman, F. E., and Kim, J-J. P. (1996) Proc. Natl. Acad. Sci. U. S. A. 90, 14355-14360
8. Sato, K., Nishina, Y., and Shiga, K. (1993) J. Biochem. 114, 215-222
9. Freneaux, E., Sheffield, V. C., Molin, L., Shires, A., and Rhead, W. J. (1992) J. Clin. Invest. 90, 1670-1686
10. Loehr, J. P., Goodman, S. I., and Frerman, F. E. (1990) Pediatr. Res. 27, 311-315
11. Ghisla, S., and Massey, V. (1989) Eur. J. Biochem. 18, 1-17
12. McKean, M. C., Beckmann, J. D., and Frerman, F. E. (1983) J. Biol. Chem. 258, 1866-1870
13. Beckmann, J. D., and Frerman, F. E. (1985) Biochemistry 24, 3913-3924
14. Massey, V., and Swoboda, B. E. P. (1963) Biochem. Z. 338, 474-484
15. Clark, W. M. (1960) Oxidation-Reduction Potentials of Organic Systems, pp. 184-203, The Williams and Wilkins Co., Baltimore, MD
16. McIlwain, H. (1937) J. Chem. Soc. 2, 1704-1711
17. Higuchi, R., Kummel, B., and Saiki, R. K. (1988) Nucleic Acids Res. 16, 7351-7367
18. Sanger, F., Nicklen, S., and Coulson, A. R. (1977) Proc. Natl. Acad. Sci. U. S. A. 74, 5463-5467
19. Husain, M., and Steenkamp, D. J. (1983) Biochem. J. 209, 541-545
20. Gatenby, A. A. (1992) Plant Molec. Biol. 19, 677-687
21. Griffin, K. J., Dwyer, T. M., Manning, M. C., Meyer, J. D., Carpenter, J. F., and Frerman, F. E. (1997) Biochemistry 36, 4194-4202
22. Massay, V. (1990) in Flavins and Flavoproteins (Curti, B., Ronchi, S., and Zanetti, G., eds) pp. 59-66, Walter de Gruyter, Berlin
23. Minnaert, K. (1965) Biochim. Biophys. Acta 110, 42-56
24. Madden, M., Lau, S-M., and Thorpe, C. (1984) Biochem. J. 224, 577-580
25. Eweg, J. D., Müller, F., van Dam, H., Terpstra, A., and Oskam, A. (1980) J. Am. Chem. Soc. 102, 51-61
26. Mayhew, S. G., Whitfield, C. D., Ghisla, S., and Jorns, M. (1974) Eur. J. Biochem. 44, 579-591
27. Whitfield, C. D., and Mayhew, S. G. (1974) J. Biol. Chem. 249, 2801-2810
28. Hsu, M. C., and Woody, R. W. (1971) J. Am. Chem. Soc. 93, 3515-3525
29. Blauer, G., Srreerama, N., and Woody, R. W. (1993) Biochemistry 32, 6674-6679
30. Edmondson, D. E., and Tollin, G. (1971) Biochemistry 10, 113-123
31. Koziol, J., and Knobloch, E. (1965) Biochim. Biophys. Acta 102, 289-300
32. Husain, M., Stankovich, M. T., and Fox, P. G. (1984) Biochem. J. 219, 1043-1047
33. Gustafson, W. G., Feinberg, B. A., and McFarland, J. T. (1986) J. Biol. Chem. 26, 7733-7741
34. Dao-pin, S., Anderson, D. E., Baase, W. A., Dahlquist, F. W., and Matthews, B. W. (1991) Biochemistry 30, 11521-11529
35. Swenson, R. P., and Krey, G. D. (1994) Biochemistry 33, 8505-8514
36. Tatwawadi, S. V., Santhanam, K. S., and Bard, A. J. (1968) J. Electroanal. Chem. 17, 411-416
37. Bedzyk, L. A., Escudero, K. W., Gill, R. E., Griffin, K. J., and Frerman, F. E. (1993) J. Biol. Chem. 268, 20211-20217