PMID: 11342107May 9, 2001Paper

Rat adrenoleukodystrophy-related (ALDR) gene: full-length cDNA sequence and new insight in expression

Biochimica Et Biophysica Acta
S AlbetM Bugaut

Abstract

X-linked adrenoleukodystrophy (X-ALD) is an inherited demyelinating disorder due to mutations in the ALD gene, which encodes a peroxisomal ABC half-transporter (ALDP). It has been suggested that ALDP assembles with ALDRP (adrenoleukodystrophy-related protein), a close homologous half-transporter, to form a functional heterodimer. For the first time full-length ALDRP cDNA (5.5 kb) was cloned, and 5' and 3' RACE analysis revealed that alternative usage of polyadenylation sites generates the two transcripts of 3.0 and 5.5 kb observed in the rat in Northern blot analysis. Southern blotting and chromosomal mapping demonstrated one ALDR locus in the rat genome. Characterisation of the 3' flanking region suggested that an ID sequence might be responsible for high expression of the 5.5 kb ALDRP transcript in rat brain. ALDR gene expression was found to be high in the liver of rats before weaning and very low in adult rats; the reverse developmental regulation was observed in the brain. Fenofibrate, which is a potent inducer of the ALDR gene in the liver of adult rats, could not induce the ALDR gene in suckling rats. The exact significance of this result with regard to development of an efficient pharmacological gene therapy for X-ALD i...Continue Reading

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Citations

Oct 14, 2006·Pflügers Archiv : European journal of physiology·Ronald J A WandersHans R Waterham
Jul 16, 2010·Brain Pathology·Johannes BergerSonja Forss-Petter
Jan 18, 2006·Biochemical and Biophysical Research Communications·Fabien GueugnonStéphane Savary
Dec 18, 2013·Biochimica Et Biophysica Acta·Carlo W T van RoermundHans R Waterham
Feb 1, 2014·Biochemical and Biophysical Research Communications·Doriane TrompierStéphane Savary
Apr 6, 2005·The Journal of Cell Biology·Catherine GondcailleMaurice Bugaut
May 23, 2003·Molecular Pharmacology·Stéphane FourcadeMaurice Bugaut

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Adrenoleukodystrophy

Adrenoleukodystrophy (ALD), the most frequent peroxisomal disorder, is an X-linked disorder caused by a defect in the metabolism of long chain fatty acids leading to demyelination, neurodegeneration, and death. Here is the latest research.