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Table 1. Enzymatic activity of b -oxidative enzymes in the liver
|
|
WT |
PPARg hyp/hyp |
|
Medium chain acyl-CoA dehydrogenase* |
2.13 ± 0.08 |
2.23 ± 0.12 |
|
Short chain 3-hydroxyacyl-CoA dehydrogenase† |
720.37 ± 16.12 |
708.31 ± 23.42 |
|
Long chain 3-hydroxyacyl-CoA dehydrogenase‡ |
359.61 ± 13.44 |
337.77 ± 19.41 |
|
Long chain 3-hydroxyacyl-CoA thiolase‡ |
33.54 ± 2.09 |
31.14 ± 1.62 |
|
Carnitine acyltransferase-2§ |
91.23 ± 2.77 |
92.95 ± 3.22 |
Activity is expressed as nmol/min per mg.
*See ref. 1 for methodology for enzyme measurements.
†See ref. 2 for methodology for enzyme measurements.
‡See ref. 3 for methodology for enzyme measurements.
§See ref. 4 for methodology for enzyme measurements.
1. Zschocke, J., Schulze, A., Lindner, M., Fiesel, S., Olgemoller, K., Hoffmann, G. F., Penzien, J., Ruiter, J. P., Wanders, R. J., Mayatepek, E., et al. (2001) Hum. Genet. 108, 404–408.
2. Bennett, M. J., Spotswood, S. D., Ross, K. F., Comfort, S., Koonce, R., Boriack, R. L., IJlst, L. & Wanders, R. J. (1999) Pediatr. Dev. Pathol. 2, 337–345.
3. Wanders, R. J., IJlst, L., Poggi, F., Bonnefont, J. P., Munnich, A., Brivet, M., Rabier, D. & Saudubray, J. M. (1992) Biochem. Biophys. Res. Commun. 188, 1139–1145.
4. IJlst, L., Mandel, H., Oostheim, W., Ruiter, J. P., Gutman, A. & Wanders, R. J. (1998) J. Clin. Invest. 102, 527–531.