The ubiquitin-conjugating enzyme UbcH6 regulates the transcriptional repression activity of the SCA1 gene product ataxin-1

Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1

Harry T. Orr, Ming‐Yi Chung, Sandro Banfi et al. · Nature Genetics · 1993 · 1.6K citations

Molecular Neuroscience, Genetics, Molecular Biology +6

Ataxin-1 Nuclear Localization and Aggregation

Ivan A. Klement, Pamela J. Skinner, Michael D. Kaytor et al. · Cell · 1998 · 959 citations · Full text

Nuclear Organization, Nuclear Engineering, Molecular Biology +1

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1

Christopher J. Cummings, Michael A. Mancini, Barbara Antalffy et al. · Nature Genetics · 1998 · 742 citations

Protein Function, Protein Assembly, Protein Folding +9

Mutation of the E6-AP Ubiquitin Ligase Reduces Nuclear Inclusion Frequency While Accelerating Polyglutamine-Induced Pathology in SCA1 Mice

Christopher J. Cummings, Eyal Reinstein, Yaling Sun et al. · Neuron · 1999 · 504 citations · Full text

Chromatin, Genome Instability, Cell Regulation +5

Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice

Christopher J. Cummings · Human Molecular Genetics · 2001 · 486 citations