Rapid degradation of short-chain acyl-CoA dehydrogenase variants with temperature-sensitive folding defects occurs after import into mitochondria

Journal of Biological Chemistry

Volumn 273, Issue 21, 1998, Pages 13065-13071

  Corydon, Thomas J ^a   Bross, Peter ^b   Jensen, Thomas G ^a   Corydon, Morten J ^b   Lund, Tommy B ^a   Jensen, Uffe B ^a   Kim, Jung Ja P ^c   Gregersen, Niels ^b   Bolund, Lars ^a  

^a AARHUS UNIVERSITY   (Denmark)

^b AARHUS UNIVERSITY HOSPITAL   (Denmark)

^c MEDICAL COLLEGE OF WISCONSIN   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ACYL COENZYME A DEHYDROGENASE; ENZYME VARIANT;

ARTICLE; ENZYME DEGRADATION; GENETIC TRANSFECTION; MISSENSE MUTATION; MITOCHONDRION; MUTAGENESIS; NONHUMAN; PRIORITY JOURNAL; PROTEIN FOLDING; PROTEIN SYNTHESIS; PROTEIN TRANSPORT; THERMOSTABILITY;

ACYL-COA DEHYDROGENASE; ACYL-COA DEHYDROGENASES; ANIMALS; BIOLOGICAL TRANSPORT; COS CELLS; HUMANS; HYDROLYSIS; MICROSCOPY, CONFOCAL; MITOCHONDRIA; MUTAGENESIS; PROTEIN FOLDING; TEMPERATURE;

EID: 0032557512     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.273.21.13065     Document Type: Article

References (40)

1. Naito, E., Indo, Y., and Tanaka, K. (1990) J. Clin. Invest. 85, 1575-1582
2. Kristensen, M. J., Kmoch, S., Bross, P., Andresen, B. S., and Gregersen N. (1994) Hum. Mol. Genet. 3, 1711
3. Brackett, J. C., Sims, H. F., Steiner, R. D., Nunge, M., Zimmerman, E. M., deMartinville, B., Rinaldo, P., Slaugh, R., and Strauss, A. W. (1994) J. Clin. Invest. 94, 1477-1483
4. Tanaka, K., Yokota, I., Coates, P. M., Strauss, A. W., Kelly, D. P., Zhang, Z., Gregersen, N., Andresen, B. S., Matsubara, Y., Curtis, D., and Chen, Y.-T. (1992) Hum. Mutat. 1, 271-279
5. Andresen, B. S., Bross, P., Udvari, S., Kirk, J., Gray, G., Kmoch, S., Chamoles, N., Knudsen, I., Winter, V., Wilcken, B., Yokota, I., Hart, K., Packman, S., Harpey, J. P., Saudubray, J. M., Hale, D. E., Bolund, L., Kølvraa, S., and Gregersen, N. (1997) Hum. Mol. Genet. 6, 695-707
6. Beinert, H. (1963) Enzymes 7, 447-476
7. Kunau, W. H., Dommes, V., and Schulz, H. (1995) Prog. Lipid Res. 34, 267-342
8. Eaton, S., Bartlett, K., and Pourfarzam, M. (1996) Biochem. J. 320, 345-357
9. Ikeda, Y., Keese, S. M., Fenton, W. A., and Tanaka, K. (1987) Arch. Biochem. Biophys. 252, 662-674
10. Naito, E., Indo, Y., and Tanaka, K. (1989) J. Clin. Invest. 84, 1671-1674
11. Coates, P. M., Indo, Y., Young, D., Hale, D. E., and Tanaka, K. (1992) Pediatr. Res. 31, 34-38
12. Bross, P., Jespersen, C., Jensen, T. G., Andresen, B. S., Kristensen, M. J., Winter, V., Nandy, A., Kräutle, F., Ghisla, S., Bolundi, L., Kim, J.-J. P., and Gregersen, N. (1995) J. Biol. Chem. 270, 10284-10290
13. Saijo, T., Welch, W. J., and Tanaka, K. (1994) J. Biol. Chem. 269, 4401-4408
14. Andresen, B. S., Bross, P., Jensen, T. G., Winter, V., Knudsen, I., Kølvraa, S., Jensen, U. B., Bolund, L., Duran, M., Kim, J. J., Curtis, D., Divry, P., Vianey-Saban, C., and Gregersen, N. (1993) Am. J. Hum. Genet. 53, 730-739
15. Jensen, T. G., Bross, P., Andresen, B. S., Lund, T. B., Kristensen, T. J., Jensen, U. B., Winther, V., Kølvraa, S., Gregersen, N., and Bolund, L. (1995) Hum. Mutat. 6, 226-231
16. Naito, E., Ozasa, H., Ikeda, Y., and Tanaka, K. (1989) J. Clin. Invest. 83, 1605-1613
17. Kim, J. J. P., Wang, M., Djordjevic, S., Paschke, R., and Bennett, D. W. (1993) in Flavins and Flavoproteins: Proceedings of the 11th International Symposium, Nagoya, Japan (Yagi, K., ed) pp. 273-281, Walter de Gruyter, Berlin
18. Djordjevic, S., Pace, C. P., Stankovich, M. T., and Kim, J. J. (1995) Biochemistry 34, 2163-2171
19. Thorpe, C., and Kim, J. J. (1995) FASEB J. 9, 718-725
20. Langer, T., and Neupert, W. (1996) Experientia 52, 1069-1076
21. Gottesman, S., Wickner, S., and Maurizi, M. R. (1997) Genes Dev. 11, 815-823
22. Sarkar, G., and Sommer, S. S. (1990) BioTechniques 8, 404-407
23. Corydon, M. J., Gregersen, N., Lehnert, W., Ribes, A., Rinaldo, P., Kmoch, S., Christensen, E., Kristensen, T. J., Andresen, B. S., Bross, P., Winter, V., Martinez, G., Neve, S., Jensen, T. G., Bolund, L., and Kølvraa, S. (1996) Pediatr. Res. 39, 1059-1066
24. Cooper, G. M. (1982) Science 217, 801-806
25. Graham, F. L., and Eb, A. J. v. d. (1973) Virology 52, 456-467
26. Jensen, T. G., Andresen, B. S., Bross, P., Jensen, U. B., Holme, E., Kølvraa, S., Gregersen, N., and Bolund, L. (1992) Biochim. Biophys. Acta 1180, 65-72
27. Bross, P., Andresen, B. S., Winter, V., Kräutle, F., Jensen, T. G., Nandy, A., Kølvraa, S., Ghisla, S., Bolund, L., and Gregersen, N. (1993) Biochim. Biophys. Acta 1182, 264-274
28. Ikeda, Y., Okamura-Ikeda, K., and Tanaka, K. (1985) J. Biol. Chem. 260, 1311-1325
29. Lehman, T. C., Hale, D. E., Bhala, A., and Thorpe, C. (1990) Anal. Biochem. 186, 280-284
30. Corydon, T. J., Bross, P., Holst, H. U., Neve, S., Kristiansen, K., Gregersen, N., and Bolund, L. (1998) Biochem. J. 331, 309-316
31. Ikeda, Y., Keese, S. M., and Tanaka, K. (1985) Proc. Natl. Acad. Sci. U. S. A. 82, 7081-7085
32. Fenton, W. A., Hack, A. M., Kraus, J. P., and Rosenberg, L. E. (1987) Proc. Natl. Acad. Sci. U. S. A. 84, 1421-1424
33. Andresen, B. S., Vianey-Saban, C., Bross, P., Divry, P., Roe, C. R., Nada, M. A., Knudsen, I. ,and Gregersen, N. (1996) J. Inherited Metab. Dis. 19, 169-172
34. Corydon, M. J., Andresen, B. S., Bross, P., Kjeldsen, M., Andreasen, P. H., Eiberg, H., Kølvraa, S., and Gregersen, N. (1997) Mamm. Genome 8, 922-926
35. Mitraki, A., Danner, M., King, J., and Seckler, R. (1993) J. Biol. Chem. 268, 20071-20075
36. Gordon, C. L., and King, J. (1994) Genetics 136, 427-438
37. Fenton, W. A., and Horwich, A. L. (1997) Protein Sci. 6, 743-760
38. Hartl, F. U. (1996) Nature 381, 571-579
39. Gregersen, N., Winter, V., Corydon, M. J., Corydon, T. J., Rinaldo, P., Ribes, A., Martinez, G., Bennett, M. J., Vianey-Saban, C., Bhala, A., Hale, D. E., Lehnert, W., Kmoch, S., Roig, M., Riudor, E., Eiberg, H., Andresen, B. S., Bross, P., Bolund, L., and Kølvraa, S. (1998) Hum. Mol. Genet. 7, 619-627
40. Kristensen, T. J., Jensen, T. G., Corydon, M. J., Bross, P., Lund, T. B., Jensen, U. B., Kim, J-J. P., Gregersen, N., and Bolund, L. (1996) Molecular Chaperones and the Heat Shock Response, May 1-5, 1996, Cold Spring Harbor Laboratory, Cold Spring Harbor, New York