X-linked adrenoleukodystrophy: defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters.

@article{Wanders1987XlinkedAD,
  title={X-linked adrenoleukodystrophy: defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters.},
  author={Ronald Wanders and Carlo W T van Roermund and Michel J A Van Wijland and A. A. Nijenhuis and A M Tromp and Ruud B. H. Schutgens and E M Brouwer-Kelder and Andr{\'e} W. Schram and Joseph M. Tager and Han van den Bosch},
  journal={Clinica chimica acta; international journal of clinical chemistry},
  year={1987},
  volume={165 2-3},
  pages={321-9}
}
We investigated the peroxisomal fatty acid beta-oxidation system in liver and cultured skin fibroblasts from patients with X-linked adrenoleukodystrophy known to accumulate very long chain fatty acids. In order to examine whether the deficient peroxisomal oxidation of very long chain fatty acids in these patients results from a deficiency in one of the peroxisomal beta-oxidation enzyme proteins (acyl-CoA oxidase, bifunctional protein with enoyl-CoA hydratase and 3-hydroxyacyl-CoA dehydrogenase… CONTINUE READING