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

Site-directed mutagenesis by overlap extension using the polymerase chain reaction

1989 • 7,496 citations

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

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

1997 • 2,762 citations

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

1997 • 2,184 citations

Phosphorylated CREB binds specifically to the nuclear protein CBP

1993 • 2,039 citations

Cell Survival Promoted by the Ras-MAPK Signaling Pathway by Transcription-Dependent and -Independent Mechanisms

1999 • 1,897 citations

Ca2+ Influx Regulates BDNF Transcription by a CREB Family Transcription Factor-Dependent Mechanism

1998 • 1,504 citations

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

1997 • 1,283 citations

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

1994 • 1,065 citations

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

2000 • 1,033 citations

Ataxin-1 Nuclear Localization and Aggregation

1998 • 959 citations

Intranuclear Inclusions of Expanded Polyglutamine Protein in Spinocerebellar Ataxia Type 3

1997 • 821 citations

Mediation by a CREB Family Transcription Factor of NGF-Dependent Survival of Sympathetic Neurons

1999 • 794 citations

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin [published erratum appears in Hum Mol Genet 1999 May;8(5):943]

1999 • 742 citations

Decreased expression of striatal signaling genes in a mouse model of Huntington's disease

2000 • 722 citations

CREB-binding protein sequestration by expanded polyglutamine

2000 • 540 citations

Stimulation of cAMP response element (CRE)-mediated transcription during contextual learning

1998 • 532 citations

Is CREB a key to neuronal survival?

2000 • 516 citations

Regulation of Dendritic Growth and Remodeling by Rho, Rac, and Cdc42

1997 • 493 citations

Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates

1998 • 490 citations

Distinct roles for bFGF and NT-3 in the regulation of cortical neurogenesis

1995 • 476 citations

Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells

1999 • 451 citations

Intranuclear Neuronal Inclusions in Huntington's Disease and Dentatorubral and Pallidoluysian Atrophy: Correlation between the Density of Inclusions andIT15CAG Triplet Repeat Length

1998 • 421 citations

CBP: A Signal-Regulated Transcriptional Coactivator Controlled by Nuclear Calcium and CaM Kinase IV

1998 • 420 citations

Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

2000 • 400 citations

Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture

1998 • 376 citations

Glutamine Repeats and Neurodegenerative Diseases: Molecular Aspects

2002 • 354 citations

Brain-derived neurotrophic factor in Huntington disease

2000 • 326 citations

Trinucleotide Repeats in Neurogenetic Disorders

1996 • 323 citations

Control of Recruitment and Transcription-Activating Function of CBP Determines Gene Regulation by NMDA Receptors and L-Type Calcium Channels

1999 • 278 citations

Amyloid Formation by Mutant Huntingtin: Threshold, Progressivity and Recruitment of Normal Polyglutamine Proteins

1998 • 277 citations

Manganese Superoxide Dismutase Protects nNOS Neurons from NMDA and Nitric Oxide-Mediated Neurotoxicity

1998 • 266 citations

When more is less: Pathogenesis of glutamine repeat neurodegenerative diseases

1995 • 263 citations

Regulation of CBP-Mediated Transcription by Neuronal Calcium Signaling

1999 • 220 citations

Nuclear Targeting of Mutant Huntingtin Increases Toxicity

1999 • 199 citations

Nuclear Accumulation of Truncated Atrophin-1 Fragments in a Transgenic Mouse Model of DRPLA

1999 • 181 citations

Huntington Disease and the Related Disorder, Dentatorubral-Pallidoluysian Atrophy (DRPLA)

1997 • 99 citations

Evidence for a recruitment and sequestration mechanism in Huntington'sdisease

1999 • 95 citations

Reduced expression of preproenkephalin in striatal neurons from huntington's disease patients

1995 • 82 citations

TRINUCLEOTIDE INSTABILITY: A Repeating Theme in Human Inherited Disorders

1996 • 70 citations

Mutant Huntingtin Forms in Vivo Complexes with Distinct Context-Dependent Conformations of the Polyglutamine Segment

1999 • 62 citations

Preparation of human cDNAs encoding expanded polyglutamine repeats

1999 • 17 citations