Effects of the testosterone metabolite dihydrotestosterone and 5α-androstan-3α,17β-diol on very long chain fatty acid metabolism in X-adrenoleukodystrophic fibroblasts

A. Petroni, M. Blasevich, G. Uziel

Research output: Contribution to journalArticlepeer-review

Abstract

X-Adrenoleukodystrophy (X-ALD) is a peroxisomal disorder associated with the abnormal accumulation of very long chain fatty acids (VLCFA) in plasma and tissues. We have demonstrated that the androgen dihydrotestosterone (DHT) and 5α-androstan-3α,17β-diol (3α-diol) have favorable effect on VLCFA metabolism. We have investigated the effect of androgens on peroxisomal β-oxidation, the incorporation of labelled lignoceric acid into cholesterol esters and VLCFA elongation, in cultured skin-fibroblasts from control and X-ALD patients. The androgens significantly increased peroxisomal β-oxidation in X-ALD fibroblasts although VLCFA levels were not normalized. The major effect was on the incorporation of labelled lignoceric acid into cholesterol esters, since the enhanced lignoceric acid incorporation into cholesterol ester fraction, which occurred in X-ALD fibroblasts, was reduced towards normal values. In contrast, the androgens had no effect on the elongation pathway.

Original languageEnglish
Pages (from-to)1567-1575
Number of pages9
JournalLife Sciences
Volume73
Issue number12
DOIs
Publication statusPublished - Aug 8 2003

Keywords

  • Cholesterol ester
  • Peroxisomal β-oxidation
  • Testosterone metabolites
  • Very long chain fatty acids
  • X-Adrenoleukodystrophy

ASJC Scopus subject areas

  • Pharmacology

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