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Peroxisomal Fatty Acid Alpha-and Beta-Oxidation in Health and Disease: New insights

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Peroxisomal Disorders and Regulation of Genes

Abstract

In humans, peroxisomes play a number of essential metabolic functions, of which most have to do with lipid metabolism including fatty acid alpha-and beta-oxidation. The importance of the peroxisomal alpha-and alpha-and systems in humans is stressed by the existence of a number of peroxisomal disorders, in which one or both of these pathways are disturbed. X-linked adrenoleukodystrophy is the most well known among the disorders of peroxisomal beta-oxidation whereas Refsum disease is the prototype of the group of alpha-oxidation defects. In this paper we will describe the basic aspects of the peroxisomal alpha-and beta-oxidation systems with particular emphasis on recently acquired knowledge.

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Wanders, R.J. et al. (2003). Peroxisomal Fatty Acid Alpha-and Beta-Oxidation in Health and Disease: New insights. In: Roels, F., Baes, M., De Bie, S. (eds) Peroxisomal Disorders and Regulation of Genes. Advances in Experimental Medicine and Biology, vol 544. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-9072-3_37

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  • DOI: https://doi.org/10.1007/978-1-4419-9072-3_37

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-4782-8

  • Online ISBN: 978-1-4419-9072-3

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