The mitochondrial trifunctional protein: Centre of a β-oxidation metabolon?

Biochemical Society Transactions

Volumn 28, Issue 2, 2000, Pages 177-182

  Eaton, S ^a   Bursby, T ^b   Middleton, B ^c   Pourfarzam, M ^b   Mills, K ^a   Johnson, A W ^a   Bartlett, K ^b  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

^b ROYAL VICTORIA INFIRMARY   (United Kingdom)

^c UNIVERSITY OF NOTTINGHAM   (United Kingdom)

Author keywords

Acyl CoA esters; Channelling; Fatty acids; Mitochondria; NAD NADH

Indexed keywords

3 HYDROXYACYL COENZYME A DEHYDROGENASE; ACETYL COENZYME A ACYLTRANSFERASE; ENOYL COENZYME A HYDRATASE; LONG CHAIN FATTY ACID;

CONFERENCE PAPER; ENZYME DEFICIENCY; FATTY ACID OXIDATION; MITOCHONDRION; PRIORITY JOURNAL;

EID: 0033985020     PISSN: 03005127     EISSN: None     Source Type: Journal    
DOI: 10.1042/bst0280177     Document Type: Conference Paper

References (31)

1. Uchida, Y., Izai, K., Orii, T. and Hashimoto, T. (1992) J. Biol. Chem. 267, 1034-1041
2. Carpenter, K., Pollitt, K.J. and Middleton, B. (1992) Biochem. Biophys. Res. Commun. 183, 443-448
3. Jackson, S., Bartlett, K., Land, J., Moxon, E. R., Pollitt, R, J., Leonard, J. V. and Tumbull, D. M. (1991) Pediatr. Res. 29, 406-411
4. Jackson, S., Kler, R. S., Bartlett, K., Briggs, H., Bindoff, L. A., Pourfarzam, M., Gardner-Medwin, D. and Tumbull, D. M. (1992) J. Clin. Invest. 90, 1219-1225
5. Kamijo, T., Aoyama, T., Komiyama, A. and Hashimoto, T. (1994) Biochem. Biophys. Res. Commun. 199, 818-825
6. Kamijo, T., Aoyama, T., Miyazaki, J. and Hashimoto, T. (1993) J. Biol. Chem. 268, 26452-26460
7. Tyni, T. and Pihko, H. (1999) Acta Paediatr. 88, 237-245
8. Sims, H. F., Brackett, J. C., Powell, C. K., Treem, W. R., Hale, D. E., Bennett, M. J., Gibson, B., Shapiro, S. and Strauss, A. W. (1995) Proc. Natl. Acad. Sci. U. S.A. 92, 841-845
9. Orii, K. E., Aoyama, T., Wakui, K., Fukushima, Y., Miyajima, H. and Yamaguchi, S. (1997) Hum. Mol. Genet. 6, 1215-1224
10. Ushikubo, S., Aoyama, T., Kamijo, T., Wanders, R. J. A., Rinaldo, P. and Vockley, J. (1996) Am. J. Hum. Genet. 58, 979-988
11. Ijlst, L., Ruiter, J. P. N., Hoovers, J. M. N., Jakobs, M. E. and Wanders, R. J. A. (1996) J. Clin. Invest. 98, 1028-1033
12. Orii, K. E., Orii, K. O., Souri, M., Orii, T., Kondo, N., Hashimoto, T. and Aoyama, T. (1999) J. Biol. Chem. 274, 8077-8084
13. Ibdah, J. A., Bennett, M. J., Rinaldo, P., Zhao, Y. W., Gibson, B., Sims, H. F. and Strauss, A. W. (1999) N. Engl. J. Med. 340, 1723-1731
14. Wanders, R. J. A., Vreken, P., denBoer, M. E. J., Wijburg, F. A., vanGennip, A. H. and Ijlst, L. (1999) J. Inherit Metab. Dis. 22, 442-487
15. Tyni, T., Kivela, T., Lappi, M., Summanen, P., Nikoskelainen, E. and Pihko, H. (1998) Ophthalmology 105, 810-824
16. Tyni, T., Majander, A., Kalimo, H., Rapola, J. and Pihko, H. (1996) Neuromusc. Dis. 6, 327-337
17. Ventura, F. V., Ruiter, J. P., Ijlst, L., de Almeida, I. T. and Wanders, R. J.A. (1998) J. Inherit. Metab. Dis. 21, 645-654
18. Luo, M. J., He, X.-Y., Sprecher, H. and Schulz, H. (1993) Arch. Biochem. Biophys. 304, 266-271
19. Muller-Newen, G., Janssen, U. and Stoffel, W. (1995) Eur. J. Biochem. 228, 68-73
20. Palosaari, P. M., Vihinen, M., Mantsala, P. I., Alexson, S. E., Pihlajaniemi, T. and Hiltunen, J. K. (1991) J. Biol. Chem. 266, 10750-10753
21. Kiema, T. R., Engel, C. K., Schmitz, W., Filppula, S. A., Wierenga, R. K. and Hiltunen, J. K. (1999) Biochemistry 38, 2991-2999
22. Eaton, S., Middleton, B. and Bartlett, K. (1998) Biochim. Biophys. Acta 1429, 230-238
23. Eaton, S., Bhuiyan, A. K. M. J., Kler, R. S., Turnbull, D. M. and Bartlett, K. (1993) Biochem. J. 289, 161-168
24. Eaton, S., Bartlett, K. and Pourfarzam, M. (1999) Biochim. Biophys. Acta 1437, 402-408
25. Sleboda, J., Pourfarzam, M., Bartlett, K. and Osmundsen, H. (1995) Biochim. Biophys. Acta 1258, 309-318
26. Eaton, S., Bartlett, K. and Pourfarzam, M. (1996) Biochem. J. 320, 345-357
27. Pourfarzam, M., Schaefer, J., Tumbull, D. M. and Bartlett, K. (1994) Clin. Chem. 40, 2267-2275
28. Yao, K. and Schulz, H. (1996) J. Biol. Chem. 271 17816-17820
29. Izai, K., Uchida, Y., Orii, T., Yamamoto, S. and Hashimoto, T. (1992) J. Biol. Chem. 267, 1027-1033
30. Hofmann, K., Bucher, P., Falquet, L. and Bairoch, A. (1999) Nucleic Acids Res. 27, 215-219
31. Yang, S.-Y. and He, X.-Y. (1999) in Current Views of Fatty Acid Oxidation and Ketogenesis (Quant, P. A. and Eaton, S., eds.), pp. 133-143, Plenum Press, New York