Mouse models of Huntington's disease

LB Menalled, MF Chesselet - Trends in pharmacological sciences, 2002 - cell.com
LB Menalled, MF Chesselet
Trends in pharmacological sciences, 2002cell.com
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder. In 1993
the mutation that causes HD was identified as an unstable expansion of CAG repeats in the
IT15 gene. Since then one of the most important advances in HD research has been the
generation of various mouse models that enable the exploration of early pathological,
molecular and cellular abnormalities produced by the mutation. In addition, these models
have made it possible to test different pharmacological approaches to delay the onset or …
Abstract
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder. In 1993 the mutation that causes HD was identified as an unstable expansion of CAG repeats in the IT15 gene. Since then one of the most important advances in HD research has been the generation of various mouse models that enable the exploration of early pathological, molecular and cellular abnormalities produced by the mutation. In addition, these models have made it possible to test different pharmacological approaches to delay the onset or slow the progression of HD. In this article, insights gained from mouse models towards the understanding of HD and the design of new therapeutic strategies are discussed.
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