Journal
TRENDS IN BIOCHEMICAL SCIENCES
Volume 31, Issue 9, Pages 533-540Publisher
ELSEVIER SCIENCE LONDON
DOI: 10.1016/j.tibs.2006.06.009
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Funding
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R01NS032765, P01NS016367, P50NS016367] Funding Source: NIH RePORTER
- NATIONAL LIBRARY OF MEDICINE [U54LM008748] Funding Source: NIH RePORTER
- NINDS NIH HHS [NS 32765, NS 16367] Funding Source: Medline
- NLM NIH HHS [LM 008748] Funding Source: Medline
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Thirteen years ago, the culmination of genetic rather than biochemical strategies resulted in the identification of the root cause of Huntington's disease: an expanded CAG trinucleotide repeat that leads to an elongated polyglutamine tract in the huntingtin protein. Since then, biochemical and cell biological attempts to elucidate pathogenesis have largely focused on N-terminal polyglutamine-containing huntingtin fragments. However, continued application of genetic strategies has suggested that the disease process is, in fact, triggered by the presence of expanded polyglutamine in intact huntingtin. An increased emphasis on the earliest presymptornatic stages of the disease, facilitated by incorporating genetic lessons from human patients into the search for biochemical targets, could provide a route to a rational treatment to prevent or slow the onset of this devastating neurodegenerative disorder.
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