Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase

Journal of Biological Chemistry

Volumn 274, Issue 32, 1999, Pages 22763-22769

  Branda, Steven S ^a   Cavadini, Patrizia ^a   Adamec, Jiri ^a   Kalousek, Frantisek ^b   Taroni, Franco ^c   Isaya, Grazia ^a  

^a MAYO CLINIC   (United States)

^b YALE UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c Laboratory of Cellular Pathology   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

FRATAXIN; IRON; MITOCHONDRIAL PROCESSING PEPTIDASE; PEPTIDASE; PROTEIN PRECURSOR; UNCLASSIFIED DRUG;

ARTICLE; FRIEDREICH ATAXIA; HUMAN; IRON METABOLISM; MITOCHONDRION; NERVE DEGENERATION; NONHUMAN; PRIORITY JOURNAL; PROTEIN DEFICIENCY; PROTEIN DEGRADATION; PROTEIN PROCESSING; PROTEIN SYNTHESIS;

AMINO ACID SEQUENCE; BIOLOGICAL TRANSPORT; CELL COMPARTMENTATION; FREE RADICALS; FRIEDREICH ATAXIA; HOMEOSTASIS; HUMANS; IRON; IRON-BINDING PROTEINS; METALLOENDOPEPTIDASES; MOLECULAR SEQUENCE DATA; PHOSPHOTRANSFERASES (ALCOHOL GROUP ACCEPTOR); PROTEIN PROCESSING, POST-TRANSLATIONAL; SACCHAROMYCES CEREVISIAE; SPECIES SPECIFICITY;

ATAXIA;

EID: 0033529554     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.274.32.22763     Document Type: Article

References (32)

1. Babcock, M., De Silva, D., Oaks, R., Davis-Kaplan, S., Jiralerspong, S., Montermini, L., Pandolfo, M., and Kaplan, J. (1997) Science 276, 1709-1712
2. Foury, F., and Cazzalini, O. (1997) FEBS Lett. 411, 373-377
3. Wilson, R. B., and Roof, D. M. (1997) Nat. Genet. 16, 352-357
4. Koutnikova, H., Campuzano, V., Foury, F., Dollé, P., Cazzalini, O., and Koenig, M. (1997) Nat. Genet. 16, 345-351
5. Radisky, D. C., Babcock, M. C., and Kaplan, J (1999) J. Biol. Chem. 274, 4497-4499
6. Lamarche, J. B., Côté, M., and Lemieux, B. (1980) J. Can. Sci. Neurol. 7, 389-396
7. Rötig, A., De Lonlay, P., Chretien, D., Foury, F., Koenig, M., Sidi, D., Munnich, A., and Rustin, P. (1997) Nat. Genet. 17, 215-217
8. Wong, A., Yang, J., Cavadini, P., Gellera, C., Lonnerdal, B., Taroni, F., and Cortopassi, G. (1999) Hum. Mol. Genet. 8, 425-430
9. Campuzano, V., Montermini, L., Moltò, M. D., Pianese, L., Cossée, M., Cavalcanti, F., Monrós, E., Rodius, F., Duclos, F., Monticelli, A., Zara, F., Cañizares, J., Koutnikova, H., Bidichandani, S. I., Gellera, C., Brice, A., Trouillas, P., De Michele, G., Filla, A., De Frutos, R., Palau, F., Patel, P. I., Di Donato, S., Mandel, J.-L., Cocozza, S., Keenig, M., and Pandolfo, M. (1996) Science 271, 1423-1427
10. Campuzano, V., Montermini, L., Lutz, Y., Cova, L., Hindelang, C., Jiralerspong, S., Trottier, Y., Kish, S. J., Faucheux, B., Trouillas, P., Authier, F. J., Dürr, A., Mandel, J.-L., Vescovi, A., Pandolfo, M., and Koenig, M. (1997) Hum. Mol. Genet. 6, 1771-1780
11. Pandolfo, M. (1998) Neuromusc. Dis. 8, 409-415
12. Knight, S. A. B., Sepuri, N. B. V., Pain, D., and Dancis, A. (1998) J. Biol. Chem. 273, 18389-18393
13. Koutnikova, H., Campuzano, V., and Koenig, M. (1998) Hum. Mol. Gen. 7, 1485-1489
14. Neupert, W. (1997) Annu. Rev. Biochem. 66, 863-917
15. Isaya, G., Miklos, D., and Rollins, R. A. (1994) Mol. Cell. Biol. 14, 5603-5616
16. Branda, S. S., Yang, Z., Chew, A., and Isaya, G. (1999) Hum. Mol. Gen. 8, 1099-1110
17. Rowley, N., Prip-Buus, C., Westermann, B., Brown, C., Schwarz, E., Barrell, B., and Neupert, W. (1994) Cell 77, 249-259
18. Layne, E. (1957) Methods Enzymol. 3, 447-454
19. Daum, G., Bohni, P. C., and Schatz, G. (1982) J. Biol. Chem. 257, 13028-13033
20. Kalousek, F., Isaya, G., and L. E. Rosenberg. (1992) EMBO J. 11, 2803-2809
21. Geli, V. (1993) Proc. Natl. Acad. Sci. U. S. A. 90, 6247-6251
22. Schneider, A., Behrens, M., Scherer, P., Pratje, E., Michaelis, G., and Schatz, G. (1991) EMBO J. 10, 247-254
23. Cheng, M. Y., Hartl, F. U., and Horwich, A. L. (1990) Nature 348, 455-458
24. Glick, B. S., Brandt, A., Cunningham, K., Muller, S., Hallberg, R. L., and Schatz, G. (1992) Cell 69, 809-822
25. Graham, L. A., Brandt, U., Sargent, J. S., and Trumpower, B. L. (1993) J. Bioenerg. Biomembr. 25, 245-257
26. Branda, S. S., and Isaya, G. (1995) J. Biol. Chem. 270, 27366-27373
27. Hendrick, J. P., Hodges, P. E., and Rosenberg, L. E. (1989) Proc. Natl. Acad. Sci. U. S. A. 86, 4056-4060
28. Gavel, Y., and Von Heijne, G. (1990) Protein Eng. 4, 33-37
29. Isaya, G., Kalousek, F., Fenton, W. A., and Rosenberg, L. E. (1991) J. Cell Biol. 113, 65-76
30. Yaffe, M. P. (1991) Methods Enzymol. 194, 627-643
31. Hawlitschek, G., Schneider, H., Schmidt, B., Tropschug, M., Hartl, F. U., and Neupert, W. (1988) Cell 53, 785-806
32. Cavadini, P., DiBlasi, C., Princivalle, A., Baratta, S., DiDonato, S., Battaglia, G., Mora, M., and Taroni, F. (1997) Am. J. Hum. Genet. 61, 42 (abstr.)