A Long CAG Repeat in the Mouse Sca1 Locus Replicates SCA1 Features and Reveals the Impact of Protein Solubility on Selective Neurodegeneration

Naturally secreted oligomers of amyloid β protein potently inhibit hippocampal long-term potentiation in vivo

2002 • 4,135 citations

Soluble pool of A? amyloid as a determinant of severity of neurodegeneration in Alzheimer's disease

1999 • 1,787 citations

Glutamine Repeats and Neurodegeneration

2000 • 1,309 citations

A thin slice preparation for patch clamp recordings from neurones of the mammalian central nervous system

1989 • 1,283 citations

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

2001 • 1,218 citations

Interference by Huntingtin and Atrophin-1 with CBP-Mediated Transcription Leading to Cellular Toxicity

2001 • 1,085 citations

Ubiquitination of a New Form of α-Synuclein by Parkin from Human Brain: Implications for Parkinson's Disease

2001 • 1,036 citations

Ataxin-1 Nuclear Localization and Aggregation

1998 • 959 citations

Deficient cerebellar long-term depression and impaired motor learning in mGluR1 mutant mice

1994 • 884 citations

Mutation of the Angelman Ubiquitin Ligase in Mice Causes Increased Cytoplasmic p53 and Deficits of Contextual Learning and Long-Term Potentiation

1998 • 820 citations

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

1998 • 730 citations

Identification of genes that modify ataxin-1-induced neurodegeneration

2000 • 659 citations

SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein

2001 • 624 citations

SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat

1995 • 582 citations

Neurological abnormalities in a knock-in mouse model of Huntington's disease

2001 • 576 citations

CREB-binding protein sequestration by expanded polyglutamine

2000 • 540 citations

Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures

1997 • 528 citations

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

1999 • 507 citations

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

2001 • 489 citations

Synaptic‐ and agonist‐induced excitatory currents of Purkinje cells in rat cerebellar slices.

1991 • 475 citations

The dorsal hippocampus is essential for context discrimination but not for contextual conditioning.

1998 • 455 citations

The dorsal hippocampus is essential for context discrimination but not for contextual conditioning.

1998 • 421 citations

Molecular genetics: Unmasking polyglutamine triggers in neurodegenerative disease

2000 • 397 citations

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

2000 • 375 citations

Huntington aggregates may not predict neuronal death in Huntington's disease.

1999 • 365 citations

Hippocampal lesions cause learning deficits in inbred mice in the Morris water maze and conditioned-fear task.

1997 • 362 citations

Hippocampal lesions cause learning deficits in inbred mice in the Morris water maze and conditioned-fear task.

1997 • 304 citations

Persistent Multiple Climbing Fiber Innervationof Cerebellar Purkinje Cellsin Mice Lacking mGluR1

1997 • 300 citations

Huntingtin aggregates may not predict neuronal death in Huntington's disease

1999 • 294 citations

Expression analysis of the ataxin–1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals

1995 • 294 citations

Purkinje Cell Expression of a Mutant Allele ofSCA1in Transgenic Mice Leads to Disparate Effects on Motor Behaviors, Followed by a Progressive Cerebellar Dysfunction and Histological Alterations

1997 • 270 citations

DBA/2 and C57BL/6 mice differ in contextual fear but not auditory fear conditioning.

1994 • 238 citations

DBA/2 and C57BL/6 mice differ in contextual fear but not auditory fear conditioning.

1994 • 204 citations

Mice Lacking Ataxin-1 Display Learning Deficits and Decreased Hippocampal Paired-Pulse Facilitation

1998 • 204 citations

Molecular analysis of juvenile Huntington disease: the major influence on (CAG)_n repeat length is the sex of the affected parent

1993 • 195 citations

Spinocerebellar ataxia type 1

1995 • 190 citations

Transient Activation of Cyclic AMP-dependent Protein Kinase during Hippocampal Long-term Potentiation

1996 • 163 citations

Cognitive deficits in olivopontocerebellar atrophy: Implications for the cholinergic hypothesis of Alzheimer's dementia

1988 • 137 citations

Clinical, neuropathologic, and genetic studies of a large spinocerebellar ataxia type 1 (SCA1) kindred

1995 • 136 citations

Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment

2002 • 134 citations

Repeat instability and motor incoordination in mice with a targeted expanded CAG repeat in the Sca1 locus

2000 • 96 citations

Expanding Our Understanding of Polyglutamine Diseases through Mouse Models

1999 • 81 citations

Executive Dysfunction in Spinocerebellar Ataxia Type 1

2001 • 69 citations