Effects of the testosterone metabolite dihydrotestosterone and 5 alpha-androstan-3 alpha,17 beta-diol on very long chain fatty acid metabolism in X-adrenoleukodystrophic fibroblasts
Articolo
Data di Pubblicazione:
2003
Citazione:
Effects of the testosterone metabolite dihydrotestosterone and 5 alpha-androstan-3 alpha,17 beta-diol on very long chain fatty acid metabolism in X-adrenoleukodystrophic fibroblasts / A. Petroni, M. Blasevich, G. Uziel. - In: LIFE SCIENCES. - ISSN 0024-3205. - 73:12(2003), pp. 1567-1575.
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.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
Cholesterol ester; Peroxisomal β-oxidation; Testosterone metabolites; Very long chain fatty acids; X-Adrenoleukodystrophy
Elenco autori:
A. Petroni, M. Blasevich, G. Uziel
Link alla scheda completa: