Rat adrenoleukodystrophy related (ALDR) gene: full-length cDNA sequence and new insight in expression
作者： Sabrina Albet , Marc Bentejac , Stéphane Savary , Catherine Gondcaille , Angela Netik
DOI: 10.1016/S0167-4781(00)00291-8
关键词: Biology 、 Molecular biology 、 Adrenoleukodystrophy 、 Southern blot 、 Locus (genetics) 、 Polyadenylation 、 Gene expression 、 Gene 、 Genetics 、 Complementary DNA 、 Northern blot
摘要: 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), close homologous half-transporter, form functional heterodimer. For first time full-length cDNA (5.5 kb) was cloned, and 5' 3' RACE analysis revealed alternative usage of polyadenylation sites generates two transcripts 3.0 5.5 kb observed rat Northern blot analysis. Southern blotting chromosomal mapping demonstrated one ALDR locus genome. Characterisation flanking region ID sequence might be responsible for high expression transcript brain. gene found liver rats before weaning very low adult rats; reverse developmental regulation Fenofibrate, potent inducer rats, could not induce suckling rats. The exact significance this result regard development efficient pharmacological therapy X-ALD discussed.
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