Cholesterol regulates ABCD2 expression: implications for the therapy of X-linked adrenoleukodystrophy

X-linked adrenoleukodystrophy (X-ALD) is a severe neurodegenerative disorder with impaired very long-chain fatty acid (VLCFA) metabolism. The disease-associated ABCD1 (ALD) gene encodes a peroxisomal membrane protein, which belongs to the superfamily of ATP-binding cassette transporters. Several tre...

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Veröffentlicht in:	Human molecular genetics 2002-10, Vol.11 (22), p.2701-2708
Hauptverfasser:	Weinhofer, Isabelle, Forss-Petter, Sonja, Žigman, Mihaela, Berger, Johannes
Format:	Artikel
Sprache:	eng
Schlagworte:	Adrenals. Adrenal axis. Renin-angiotensin system (diseases) Adrenoleukodystrophy - drug therapy Adrenoleukodystrophy - genetics Adrenoleukodystrophy - metabolism Animals Anticholesteremic Agents - therapeutic use ATP Binding Cassette Transporter, Subfamily D ATP Binding Cassette Transporter, Subfamily D, Member 1 ATP-Binding Cassette Transporters - genetics ATP-Binding Cassette Transporters - metabolism Base Sequence Biological and medical sciences CCAAT-Enhancer-Binding Proteins - metabolism Cell Line Cells, Cultured Cholesterol - metabolism Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases DNA - genetics DNA-Binding Proteins - metabolism Endocrinopathies Errors of metabolism Fatty Acids - metabolism Gene Expression - drug effects Humans Lovastatin - therapeutic use Malignant tumors Medical sciences Metabolic diseases Mice Miscellaneous hereditary metabolic disorders Mutagenesis, Site-Directed Neurology Promoter Regions, Genetic Sterol Regulatory Element Binding Protein 1 Transcription Factors
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