α-Oxidatian of 3-methyl-substituted fatty acids in rat liver. Production of formic acid instead of CO2, cofactor requirements, subcellular localization and formation of a 2-hydroxy-3-methylacyl-CoA intermediate

European Journal of Biochemistry

Volumn 240, Issue 3, 1996, Pages 674-683

  Croes, Kathleen ^a   Casteels, Minne ^a   De Hoffmann, Edmond ^b   Mannaerts, Guy P ^a   Van Veldhoven, Paul P ^a  

^a UNIVERSITY OF LEUVEN   (Belgium)

^b UNIVERSITÉ CATHOLIQUE DE LOUVAIN   (Belgium)

Author keywords

Efsum's disease; Hydroxylase; Peroxisomes; Phytanic acid; Oxidation

Indexed keywords

CARBON DIOXIDE; FORMIC ACID; ACYL COENZYME A; FATTY ACID; FORMIC ACID DERIVATIVE; PHYTANIC ACID;

ANIMAL CELL; ANIMAL TISSUE; ARTICLE; CELLULAR DISTRIBUTION; CONTROLLED STUDY; CYTOSOL; FATTY ACID OXIDATION; IN VITRO STUDY; LIVER CELL; LIVER HOMOGENATE; NONHUMAN; PEROXISOME; PRIORITY JOURNAL; RAT; REACTION ANALYSIS; ANIMAL; CELL FRACTIONATION; CHEMICAL MODEL; CHEMISTRY; HYDROXYLATION; LIVER; MALE; MASS SPECTROMETRY; METABOLISM; METHYLATION; OXIDATION REDUCTION REACTION; WISTAR RAT;

ANIMALIA;

ACYL COENZYME A; ANIMALS; CARBON DIOXIDE; FATTY ACIDS; FORMIC ACIDS; HYDROXYLATION; LIVER; MALE; MASS SPECTROMETRY; METHYLATION; MODELS, CHEMICAL; OXIDATION-REDUCTION; PHYTANIC ACID; RATS; RATS, WISTAR; SUBCELLULAR FRACTIONS;

EID: 0029662167     PISSN: 00142956     EISSN: None     Source Type: Journal    
DOI: 10.1111/j.1432-1033.1996.0674h.x     Document Type: Article

References (44)

1. Steinberg, D. (1989) Refsum disease, in The metabolic basis of inherited disease (Scriver, C. R., Beaudet, A. L., Sly, W. S. & Valle, D., eds) 6th edn, pp. 1533-1550, McGraw-Hill Books, New York.
2. Singh, H., Usher, S., Johnson, D. & Poulos, A. (1990) A comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders. J. Lipid Res. 31, 217-225.
3. Vanhove, G., Van Veldhoven, P. P., Vanhoutte, F., Parmentier, G., Eyssen, H. J. & Mannaerts, G. P. (1991) Mitochondrial and peroxisomal α-oxidation of the branched chain fatty acid 2-methylpalmitate in rat liver, J. Biol. Chem. 266, 24670-24675.
4. Van Veldhoven, P. P., Huang, S., Eyssen, H. J. & Mannaerts, G. P. (1993) The deficient degradation of synthetic 2- and 3-methyl-branched fatty acids in fibroblasts from patients with peroxisomal disorders. J. Inher. Metab. Dis. 16, 381-391.
5. Jakobs, B. S., van den Bogert, C., Dacremont, G. & Wanders, R. J. A. (1994) β-Oxidation of fatty acids in cultured human skin fibroblasts devoid of the capacity for oxidative phosphorylation, Biochim. Biophys. Acta 1211, 37-43.
6. Singh, H., Beckman, K. & Poulos, A. (1994) Peroxisomal β-oxidation of branched chain fatty acids in rat liver. Evidence that carnitine palmitoyltransferase I prevents transport of branched chain fatty acids into mitochondria. J. Biol. Chem. 269, 9514-9520.
7. Poulos, A., Sharp, P., Fellenberg, A. J. & Johnson, D. W. (1988) Accumulation of pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) in the plasma of patients with generalised peroxisomal dysfunction, Eur. J. Pediatr. 147, 143-147.
8. Tsai, S.-C., Avigan, J. & Steinberg, D. (1969) Studies on the α-oxidation of phytanic acid by rat liver mitochondria, J. Biol. Chem. 244, 2682-2692.
9. Watkins, P. A. & Mihalik, S. J. (1990) Mitochondrial oxidation of phytanic acid in human and monkey liver: implication that Refsum's disease is not a peroxisomal disorder, Biochem. Biophys. Res. Commun. 167, 580-586.
10. Skjeldal, O. H. & Stokke, O. (1987) The subcellular localization of phytanic acid oxidase in rat liver. Biochim. Biochim. Acta 921, 38-42.
11. Huang, S., Van Veldhoven, P. P., Vanhoutte, F., Parmentier, G., Eyssen, H. J. & Mannaerts, G. P. (1992) α-Oxidation of 3-methyl-subslituted fatty acids in rat liver. Arch. Biochem. Biophys. 296, 214-223.
12. Singh, I., Pahan, K., Dhaunsi, G. S., Lazo, O. & Ozand, P. (1993) Phytanic acid α-oxidation. Differential localization in rat and human tissues and its inhibition by Nycodenz, J. Biol. Chem. 268, 9972-9979.
13. Mihalik, S. J., Rainville, A. M. & Watkins, P. A. (1995) Phytanic acid α-oxidation in rat liver peroxisomes. Production of α-hydroxyphytanoyl-CoA and formate is enhanced by dioxygenase cofactors, Eur. J. Biochem. 232, 545-551.
14. Fingerhut, R., Schmitz, W. & Conzelmann, E. (1993) Accumulation of phytanic acid α-oxidation intermediates in Zellweger fibroblasts, J. Inher. Metab. Dis. 16, 591-594.
15. Wanders, R. J. A. & van Roermund, C. W. T. (1993) Studies on phytanic acid α-oxidation in rat liver and cultured human skin fibroblasts. Biochim. Biophys. Acta 1167, 345-350.
16. Muralidharan, V. B. & Kishimoto, Y. (1984) Phytanic acid α-oxidation in rat liver. Requirement of cytosolic factor, J. Biol. Chem. 259, 13021-13026.
17. Croes, K., Casteels, M., Van Veldhoven, P. P. & Mannaerts, G. P. (1995) Evidence for the importance of iron in the α-oxidation of 3-methyl-substituted fatty acids in the intact cell. Biochim. Biophys. Acta 1255, 63-67.
18. Poulos, A., Sharp, P., Singh, H., Johnson, D. W., Carey, W. F. & Easton, C. (1993) Formic acid is a product of the alpha-oxidation of fatty acids by human skin fibroblasts: deficiency of formic acid production in peroxisome-deficient fibroblasts, Biochem. J. 292, 457-461.
19. Casteels, M. Croes, K., Van Veldhoven, P. P. & Mannaerts, G. P. (1994) Aminotriazole is a potent inhibitor of α-oxidation of 3-methyl-substituted fatty acids in rat liver, Biochem. Pharmacol. 48, 1973-1975.
20. Tsai, S.-C., Herndon, J. H., Uhlendorf, B. W., Fales, H. M. & Mize, C. E. (1967) The formation of α-hydroxy phytanic acid from phytanic acid in mammalian tissues. Biochem. Biophys. Res. Commun. 28, 571-577.
21. ten Brink, H. J., Schor, D. S. M., Kok, R. M., Poll-Thé, B. T., Wanders, R. J. A. & Jakobs, C. (1992) Phytanic acid α-oxidation: accumulation of 2-hydroxyphytanic acid and absence of 2-oxo-phytanic acid in plasma from patients with peroxisomal disorders, J. Lipid Res. 33, 1449-1457.
22. Skjeldal, O. H. & Stokke, O. (1988) Evidence against α-hydroxyphytanic acid as an intermediate in the metabolism of phytanic acid, Scand. J. Clinn. Lab. Invest. 48, 97-102.
23. Harshman, S., Sugg, N. & Cassidy, P. (1988) Preparation and purification of Staphylococcal alpha toxin. Methods Enzymol. 165, 3-7.
24. Casteels, M. Schepers, L., Van Eldere, J., Eyssen, H. J. & Mannaerts, G. P. (1988) Inhibition of 3α,7α,12α-trihydroxy-5β-cholestanoic acid oxidation and of bile acid secretion in rat liver by fatty acids, J. Biol. Chem. 263, 4654-4661.
25. Schmitz, W., Fingerhut, R. & Conzelman, E. (1994) Purification and properties of an α-methylacyl-CoA racemase from rat liver. Eur. J. Biochem. 222, 313-323.
26. Zahlten, R. N. & Stratman, F. W. (1974) The isolation of hormone-sensitive rat hepatocytes by a modified enzymatic technique, Arch. Biochem. Biophys. 163, 600-608.
27. Declercq, P. K., Haagsman, H. P., Van Veldhoven, P. P., Debeer, L. J., Van Golde, L. M. G. & Mannaerts, G. P. (1984) Rat liver dihydroxyacetone-phosphate acyltransferases and their contribution to glycerolipid synthesis, J. Biol. Chem. 259, 9064-9075.
28. Novikov, D. K., Vanhove, G. F., Carchon, H., Asselberghs, S., Eyssen, H. J., Van Veldhoven, P. P., & Mannaerts, G. P. (1994) Peroxisomal β-oxidation. Purification of four novel 3-HydroxyacylCoA dehydrogenases from rat liver peroxisomes, J. Biol. Chem. 269, 27125-27135.
29. Van Veldhoven, P. P., Just, W. W. J. & Mannaerts, G. P. (1987) Permeability of the peroxisomal membrane to cofactors of β-oxidation. Evidence for the presence of a pore-forming protein. J. Biol. Chem. 262, 4310-4318.
30. 14C]hexadecanoate oxidation by intact rat liver mitochondria. Biochem. J. 262, 261-269.
31. Durst, H. D., Milano, M., Kikta Jr., E. J., Connelly, S. A. & Grushka, E. (1975) Phenacyl esters of fatty acids via crown ether catalysts for enhanced ultraviolet detection in liquid chromatography, Anal. Chem. 47, 1797-1801.
32. 3+ and phosphate, Lipids 29, 671-678.
33. McEwen, B. F. & Arion W. J. (1985) Permeabilization of rat hepatocytes with Staphylococcus aureus a-toxin. J. Cell Biol. 100, 1922-1929.
34. Mannaerts, G. P., Debeer, L. J., Thomas, J. & De Schepper, P. J. (1979) Mitochondrial and peroxisomal fatty acid oxidation in liver homogenates and isolated hepatocytes from control and clofibrate-treated rats, J. Biol. Chem. 254, 4585-4595.
35. Pedersen, J. I. & Gustafsson, J. (1980) Conversion of 3α,7α,12α-trihydroxy-5β-cholestanoic acid into cholic acid by rat liver peroxisomes, FEBS Lett. 121, 345-348.
36. Paul, H. S., Sekas, G. & Adibi, S. A. (1992) Carnitine biosynthesis in hepatic peroxisomes. Demonstration of γ-butyrobetaine hydroxylase activity, Eur. J. Biochem. 203, 599-605.
37. Hayaishi, O., Nozaki, M. & Abbott, M. T. (1975) Oxygenases: dioxygenases, in The enzymes (Boyer, P. D., ed.) vol. 12, pp. 119-189, Academic Press, New York.
38. Murphy, R. C. (1993) Fatty acids, in Handbook of Lipid research (Snyder, F., ed.) vol. 7, pp. 71-130, Plenum Press, New York.
39. Lageweg, W. & Wanders, R. J. A. (1993) Studies on the effect of fenoprofen on the activation and oxidation of long chain and very long chain fatty acids in hepatocytes and subcellular fractions from rat liver, Biochem. Pharmacol. 46, 79-85.
40. Skorin, C., Necochea, C., Johow, V., Soto, U., Grau, A. M., Bremer, J. & Leighton, F. (1992) Peroxisomal fatty acid oxidation and inhibitors of the mitochondrial carnitine palmitoyltransferase I in isolated rat hepatocytes, Biochem. J. 281, 561-567.
41. Pahan, K., Cofer, J., Baliga, P. & Singh, I. (1993) Identification of phytanoyl-CoA ligase as a distinct acyl-CoA ligase in peroxisomes from cultured human skin fibroblasts, FEBS Lett. 322, 101-104.
42. Vanhooren, J. C. T., Asselberghs, S., Eyssen, H. J., Mannaerts, G. P. & Van Veldhoven, P. P. (1994) Activation of 3-methyl-branched fatty acids in rat liver. Int. J. Biochem. 26, 1095-1101.
43. Santos, M. J., Imanaka, T., Shio, H., Small, G. M. & Lazarow, P. B. (1988) Peroxisomal membrane ghosts in Zellweger Syndrome - Aberrant organelle assembly, Science 239, 1536-1538.
44. Declercq, P. E., Debeer, L. J. & Mannaerts, G. P. (1982) Role of glycerol 3-phosphate and glycerophosphate acyltransferase in the nutritional control of hepatic triacylglycerol synthesis, Biochem. J. 204, 247-256.