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Journal of Inherited Metabolic Disease

, Volume 14, Issue 3, pp 349–352 | Cite as

Identification of pristanoyl-CoA oxidase and phytanic acid decarboxylation in peroxisomes and mitochondria from human liver: Implications for Zellweger syndrome

  • R. J. A. Wanders
  • C. W. T. van Roermund
  • C. Jakobs
  • H. J. ten Brink
Short Communication

Keywords

Public Health Internal Medicine Metabolic Disease Human Liver Phytanic Acid 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. Lazarow, P. D. and Moser, H. W. Disorders of peroxisome biogenesis. In: Scriver, C. A., Beaudet, A. L., Sly, W. S. and Valle, D. (eds.),The Metabolic Basis of Inherited Disease, McGraw-Hill, New York, 1989, pp. 1501–1532Google Scholar
  2. Poulos, A., Sharp, P., Fellenberg, A. J. and Johnson, D. W. Accumulation of pristanic acid in the plasma of patients with generalized peroxisomal disorders.Eur. J. Pediatr. 147 (1988) 143–147PubMedGoogle Scholar
  3. Singh, H., Usher, S., Johnson, D. and Poulos, A. A comparative study of straight chain and branched chain fatty acid oxidation in fibroblasts from patients with peroxisomal disorders.J. Lipid Res. 31 (1990) 217–255PubMedGoogle Scholar
  4. Skjeldal, O. and Stokke, O. The subcellular localization of phytanic acid oxidase in rat liver.Biochim. Biophys. Acta 921 (1987) 38–42PubMedGoogle Scholar
  5. Steinberg, D. Refsum disease. In: Scriver, C. R., Beaudet, A. L., Sly, W. S. and Valle, D. (eds.),The Metabolic Basis of Inherited Disease, McGraw-Hill, New York, 1989, pp. 1533–1550Google Scholar
  6. Tsai, S. C., Avigan, J. and Steinberg, D. Studies on the alpha-oxidation of phytanic acid by rat liver mitochondria.J. Biol. Chem. 244 (1969) 2682–2687PubMedGoogle Scholar
  7. Wanders, R. J. A., Heymans, H. S. A., Schutgens, R. B. H., Barth, P. G., van den Bosch, H. and Tager, J. M. Peroxisomal disorders in neurology.J. Neurol. Sci. 88 (1988) 1–39PubMedGoogle Scholar
  8. Wanders, R. J. A., Romeyn, G. J., Schutgens, R. B. H. and Tager, J. M.L-Pipecolate oxidase: a distinct peroxisomal enzyme in man.Biochem. Biophys. Res. Commun. 164 (1989) 550–555PubMedGoogle Scholar

Copyright information

© SSIEM and Kluwer Academic Publishers 1991

Authors and Affiliations

  • R. J. A. Wanders
    • 1
  • C. W. T. van Roermund
    • 1
  • C. Jakobs
    • 2
  • H. J. ten Brink
    • 2
  1. 1.Department of PaediatricsUniversity Hospital AmsterdamAmsterdamThe Netherlands
  2. 2.Department of PaediatricsFree University of AmsterdamAmsterdamThe Netherlands

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