مورد إلكتروني
Rat adrenoleukodystrophy related (ALDR) gene: full-length cDNA sequence and new insight in expression
| العنوان: | Rat adrenoleukodystrophy related (ALDR) gene: full-length cDNA sequence and new insight in expression |
|---|---|
| المصدر: | Biochimica et Biophysica Acta, 1517 |
| بيانات النشر: | 2001 |
| تفاصيل مُضافة: | Albet, S. Bentejac, M. Savary, S. Gondcaille, C. Netik, A. Berger, Johannes Szpirer, Claude Troffer-Charlier, N. Bugaut, M. |
| نوع الوثيقة: | Electronic Resource |
| مستخلص: | 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 is discussed. Comparative Study Journal Article Research Support, Non-U.S. Gov't info:eu-repo/semantics/published |
| مصطلحات الفهرس: | Biologie moléculaire, 3' Untranslated Regions -- chemistry, 5' Untranslated Regions -- chemistry, ATP-Binding Cassette Transporters, Adrenoleukodystrophy -- genetics, Amino Acid Sequence, Animals, Base Sequence, Brain -- metabolism, Chromosome Mapping, Cloning, Molecular, DNA, Complementary -- biosynthesis, DNA, Complementary -- chemistry, Gene Expression Regulation, Developmental, Gene Library, Mice, Molecular Sequence Data, Procetofen, Protein Biosynthesis, Proteins -- chemistry, Proteins -- genetics, Rats, Rats, Wistar, info:eu-repo/semantics/article, info:ulb-repo/semantics/articlePeerReview, info:ulb-repo/semantics/openurl/article |
| URL: | |
| الإتاحة: | Open access content. Open access content |
| ملاحظة: | No full-text files English |
| أرقام أخرى: | EQY oai:dipot.ulb.ac.be:2013/17889 uri/info:pii/S0167-4781(00)00291-8 uri/info:pmid/11342107 local/cs-0019 1363760504 |
| المصدر المساهم: | UNIVERSITE LIBRE DE BRUXELLES From OAIster®, provided by the OCLC Cooperative. |
| رقم الأكسشن: | edsoai.on1363760504 |
| قاعدة البيانات: | OAIster |
| الوصف غير متاح. |