Polyglutamine Expansions

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

1996 • 3,012 citations

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

1997 • 2,184 citations

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

1998 • 1,592 citations

Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases.

1994 • 1,065 citations

Reversal of Neuropathology and Motor Dysfunction in a Conditional Model of Huntington's Disease

2000 • 1,063 citations

Ataxin-1 Nuclear Localization and Aggregation

1998 • 959 citations

Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

1999 • 641 citations

Genetic Suppression of Polyglutamine Toxicity in Drosophila

2000 • 562 citations

Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins

2000 • 549 citations

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

1999 • 507 citations

Analysis of the Role of Heat Shock Protein (Hsp) Molecular Chaperones in Polyglutamine Disease

1999 • 419 citations

Caspase-8 Is Required for Cell Death Induced by Expanded Polyglutamine Repeats

1999 • 418 citations

Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1

2000 • 375 citations

Recruitment and the Role of Nuclear Localization in Polyglutamine-mediated Aggregation

1998 • 317 citations

Aberrant Interactions of Transcriptional Repressor Proteins with the Huntington's Disease Gene Product, Huntingtin

1999 • 299 citations

Characterization and localization of the Huntington disease gene product

1993 • 202 citations

A cellular model that recapitulates major pathogenic steps of Huntington's disease

1998 • 192 citations

Cellular Defects and Altered Gene Expression in PC12 Cells Stably Expressing Mutant Huntingtin

1999 • 117 citations

A homologue of the bacterial heat-shock gene DnaJ that alters protein sorting in yeast

1991 • 117 citations

Regulation of expanded polyglutamine protein aggregation and nuclear localization by the glucocorticoid receptor

2000 • 67 citations