| Literature DB >> 24480443 |
Doriane Trompier1, Catherine Gondcaille1, Gérard Lizard1, Stéphane Savary2.
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.Entities:
Keywords: ABC transporters; Adrenoleukodystrophy; LXR antagonists; Neurodegenerative disorders; Oxysterols; Peroxisome
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Year: 2014 PMID： 24480443 DOI： 10.1016/j.bbrc.2014.01.025
Source DB: PubMed Journal: Biochem Biophys Res Commun ISSN： 0006-291X Impact factor: 3.575