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Nucleic Acids Research, 2002, Vol. 30, No. 23 5110-5119
© 2002 Oxford University Press

An upstream open reading frame impedes translation of the huntingtin gene

Joseph Lee1, Eun Hee Park1, Graeme Couture1, Isabelle Harvey1, Philippe Garneau1 and Jerry Pelletier*,1,2

1 Department of Biochemistry and 2 McGill Cancer Center, McIntyre Medical Sciences Building, McGill University, Montreal, Quebec H3G 1Y6, Canada

*To whom correspondence should be addressed at McIntyre Medical Sciences Building, Room 810, 3655 Promenade Sir William Osler, McGill University, Montreal, Quebec H3G 1Y6, Canada. Tel: +1 514 398 2323; Fax: +1 514 398 7384; Email: jerry.pelletier{at}mcgill.ca

Expansion of a CAG tract within the huntingtin gene, leading to the production of a protein with an expanded polyglutamine tract, is responsible for Huntington’s disease. We show here that the 5' untranslated region (UTR) of the huntingtin gene plays an important role in controlling the synthesis of huntingtin. In particular, the 5' UTR contains an upstream open reading frame (uORF) encoding a 21 amino acid peptide. We demonstrate that the presence of this uORF negatively influences expression from the huntingtin mRNA. Our results suggest a role for the uORF in limiting ribosomal access to downstream initiation sites. Mechanisms involving the post-transcriptional regulation of huntingtin are not well understood, and this may be an important way of regulating huntingtin protein levels.


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