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首页> 外文期刊>Life sciences >Effects of the testosterone metabolite dihydrotestosterone and 5alpha-androstan-3alpha,17beta-diol on very long chain fatty acid metabolism in X-adrenoleukodystrophic fibroblasts.
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Effects of the testosterone metabolite dihydrotestosterone and 5alpha-androstan-3alpha,17beta-diol on very long chain fatty acid metabolism in X-adrenoleukodystrophic fibroblasts.

机译：睾丸激素代谢产物二氢睾丸激素和5α-雄烷3α，17β-二醇对X肾上腺皮质营养型成纤维细胞中非常长链脂肪酸代谢的影响。

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摘要

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 5alpha-androstan-3alpha,17beta-diol (3alpha-diol) have favorable effect on VLCFA metabolism. We have investigated the effect of androgens on peroxisomal beta-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 beta-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.
机译：X肾上腺皮质营养不良（X-ALD）是一种过氧化物酶体紊乱，与血浆和组织中超长链脂肪酸（VLCFA）的异常蓄积有关。我们已经证明，雄激素二氢睾丸激素（DHT）和5alpha-雄烷3alpha，17beta-二醇（3alpha-二醇）对VLCFA代谢具有有利作用。我们已经研究了雄性激素对过氧化物酶体β-氧化，将标记的木质酸掺入胆固醇酯和VLCFA延长中的作用，来自对照组和X-ALD患者的培养皮肤成纤维细胞中。尽管VLCFA水平未标准化，但雄激素显着增加了X-ALD成纤维细胞的过氧化物酶体β-氧化。主要作用是将标记的木质酸掺入胆固醇酯中，因为X-ALD成纤维细胞中增加的木质酸掺入胆固醇酯级分已降低至正常值。相反，雄激素对延伸途径没有影响。

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