Regulation of the adrenoleukodystrophy-related gene (ABCD2): Focus on oxysterols and LXR antagonists |
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Authors: | Doriane Trompier,Catherine GondcailleGé rard Lizard,Sté phane Savary |
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Affiliation: | Laboratoire Bio-PeroxIL, EA7270 University of Bourgogne, 6 Bd Gabriel, Dijon F-21000, France |
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Abstract: | The regulation of the ABCD2 gene is recognized as a possible therapeutic target for X-linked adrenoleukodystrophy, a rare neurodegenerative disease caused by mutations in the ABCD1 gene. Up-regulation of ABCD2 expression has indeed been demonstrated to compensate for ABCD1 deficiency, restoring peroxisomal β-oxidation of very-long-chain fatty acids. Besides the known inducers of the ABCD2 gene (phenylbutyrate and histone deacetylase inhibitors, fibrates, dehydroepiandrosterone, thyroid hormone and thyromimetics), this review will focus on LXR antagonists and 22S-hydroxycholesterol, recently described as inducers of ABCD2 expression. Several LXR antagonists have been identified and their possible indication for neurodegenerative disorders will be discussed. |
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Keywords: | LXR antagonists Oxysterols Adrenoleukodystrophy Peroxisome ABC transporters Neurodegenerative disorders |
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