Mechanisms of Ataxin-3 Misfolding and Fibril Formation: Kinetic Analysis of a Disease-associated Polyglutamine Protein

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

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

Montserrat Arrasate, Siddhartha Mitra, Erik S. Schweitzer et al. · Nature · 2004 · 2K citations

Life Sciences, Neurobiology, Neuroscience +15

CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1

Yoshiya Kawaguchi, Toshihiro Okamoto, Masafumi Taniwaki et al. · Nature Genetics · 1994 · 1.8K citations

Life Sciences, Genetics, Molecular Genetics +11

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

Huntingtin-Encoded Polyglutamine Expansions Form Amyloid-like Protein Aggregates In Vitro and In Vivo

Eberhard Scherzinger, Rudi Lurz, Mark Turmaine et al. · Cell · 1997 · 1.3K citations · Full text

Life Sciences, Molecular Biology, Neuroscience +10