Short Communication — SSIEM Award
Phytanic acid oxidation in man: identification of a new enzyme catalysing the formation of 2-ketophytanic acid from 2-hydroxyphytanic acid and its deficiency in the Zellweger syndrome
R. J. A. Wanders,
R. J. A. Wanders
Departments of Pediatrics and Clinical Chemistry, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
Search for more papers by this author C. W. T. van Roermund,
C. W. T. van Roermund
Departments of Pediatrics and Clinical Chemistry, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
Search for more papers by this author D. S. M. Schor,
D. S. M. Schor
Department of Pediatrics, Free University of Amsterdam, Amsterdam, The Netherlands
Search for more papers by this author H. J. ten Brink,
H. J. ten Brink
Department of Pediatrics, Free University of Amsterdam, Amsterdam, The Netherlands
Search for more papers by this author C. Jakobs,
C. Jakobs
Department of Pediatrics, Free University of Amsterdam, Amsterdam, The Netherlands
Search for more papers by this author
R. J. A. Wanders,
R. J. A. Wanders
Departments of Pediatrics and Clinical Chemistry, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
Search for more papers by this author C. W. T. van Roermund,
C. W. T. van Roermund
Departments of Pediatrics and Clinical Chemistry, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
Search for more papers by this author D. S. M. Schor,
D. S. M. Schor
Department of Pediatrics, Free University of Amsterdam, Amsterdam, The Netherlands
Search for more papers by this author H. J. ten Brink,
H. J. ten Brink
Department of Pediatrics, Free University of Amsterdam, Amsterdam, The Netherlands
Search for more papers by this author C. Jakobs,
C. Jakobs
Department of Pediatrics, Free University of Amsterdam, Amsterdam, The Netherlands
Search for more papers by this author
First published: 01 March 1995
Department of Pediatrics, Academic Medical Center, University Hospital Amsterdam, Meibergdreef 9, Amsterdam, 1105 AZ, The Netherlands
No abstract is available for this article.
References
- 1Huang S, Van Veldhoven PP, Vanhoutte F, Eyssen HJ, Mannaerts GP (1992) α-Oxidation of 3-methyl-substituted fatty acids in rat liver. Arch Biochem Biophys, 296: 214–223.
- 2Singh H, Usher S, Johnson D, Poulos AA (1990) 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.
- 3Singh I, Pahan K, Dhaunsi GS, Lazo O, Ozand P (1993) Phytanic acid α-oxidation: differential subcellular localization in rat and human tissues and its inhibition by Nycodenz. J Biol Chem, 268: 9972–9979.
- 4Ten Brink HJ, Schor DSM, Kok RM, Poll-The BT, Wanders RJA, Jakobs C (1992) Phytanic acid α-oxidation: accumulation of 2-hydroxyphytanic acid and absence of 2-oxophytanic acid in plasma from patients with peroxisomal disorders. J Lipid Res, 33: 1449–1457.
- 5Tranchant C, Aubourg P, Mohr M, Rocchiccioli F, Zaenker Ch, Warter JM (1993) A new peroxisomal disease with impaired phytanic and pipecolic acid oxidation. Neurology, 43: 2044–2048.
- 6Wanders RJA, Van Roermund CWT (1993) Studies on phytanic acid α-oxidation in rat liver and cultured skin fibroblasts. Biochim Biophys Acta, 1167: 345–350.
- 7Wanders RJA, Schutgens RBH, Barth PG, Tager JM, Van der Bosch H (1993) Postnatal diagnosis of peroxisomal disorders: a biochemical approach. Biochimie, 75: 269–279.