The metabotropic glutamate receptor 5 antagonist MPEP and the mGluR2 agonist LY379268 modify disease progression in a transgenic mouse model of Huntington's disease

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes

Marcy E. MacDonald · Cell · 1993 · 8.4K citations · Full text

Life Sciences, Genetics, Molecular Genetics +13

Exon 1 of the HD Gene with an Expanded CAG Repeat Is Sufficient to Cause a Progressive Neurological Phenotype in Transgenic Mice

Laura Mangiarini, Kirupa Sathasivam, Mary J. Seller et al. · Cell · 1996 · 3.1K citations · Full text

Life Sciences, Genetics, Molecular Genetics +17

Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain

Marian DiFiglia, Ellen Sapp, Kathryn Chase et al. · Science · 1997 · 2.9K citations

Life Sciences, Neurobiology, Neuroscience +16

Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation

Stephen W. Davies, Mark Turmaine, Barbara A. Cozens et al. · Cell · 1997 · 2.2K citations · Full text

Life Sciences, Genetics, Neurology +17

Huntingtin Acts in the Nucleus to Induce Apoptosis but Death Does Not Correlate with the Formation of Intranuclear Inclusions

Frédéric Saudou, Steven Finkbeiner, Didier Devys et al. · Cell · 1998 · 1.6K citations · Full text

Life Sciences, Cell Biology, Molecular Biology +12