Developmental Neuroscience
Clinical and Biological Aspects of Leukodystrophies
Peroxisomal Fatty Acid β-Oxidation in Relation to AdrenoleukodystrophyWanders R.J.A.a · Tager J.M.ba Department of Pediatrics, University Hospital Amsterdam, and b E.C. Slater Institute for Biochemical Research, University of Amsterdam, The Netherlands
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Article / Publication Details
Published online: December 16, 1991
Issue release date: 1991
Number of Print Pages: 5
Number of Figures: 0
Number of Tables: 0
ISSN: 0378-5866 (Print)
eISSN: 1421-9859 (Online)
For additional information: https://www.karger.com/DNE
Abstract
X-linked adrenoleukodystrophy is a neurological disease characterized by progressive demyelination with destruction of the white matter, and adrenal insufficiency. Biochemically there is accumulation of very-long-chain fatty acids resulting from an impairment in the peroxisomal oxidation of these fatty acids. In this paper we describe the present state of our knowledge with regard to the organization of the peroxisomal fatty acid oxidation system, with particular emphasis on X-linked adrenoleukodystrophy.
© 1991 S. Karger AG, Basel
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Article / Publication Details
Published online: December 16, 1991
Issue release date: 1991
Number of Print Pages: 5
Number of Figures: 0
Number of Tables: 0
ISSN: 0378-5866 (Print)
eISSN: 1421-9859 (Online)
For additional information: https://www.karger.com/DNE
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