Peroxisomal fatty acid beta-oxidation in relation to adrenoleukodystrophy.
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.
Wanders
R J
RJ
Department of Pediatrics, University Hospital Amsterdam, The Netherlands.
Tager
J M
JM
D000326:Adrenoleukodystrophy / Q000378:metabolism*; D000818:Animals; D001692:Biological Transport; D001921:Brain / Q000378:metabolism; D002451:Cell Compartmentation; D002648:Child; D005227:Fatty Acids / Q000378:metabolism*; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000378:metabolism; D008830:Microbodies / Q000201:enzymology / Q000378:metabolism*; D008928:Mitochondria / Q000378:metabolism; D010084:Oxidation-Reduction; D051381:Rats; D013379:Substrate Specificity; D014960:X Chromosome
D005227:Fatty Acids; D008565:Membrane Proteins