Genetic and environmental factors in the pathogenesis of Huntington’s disease

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes

1993 • 7,941 citations

Running increases cell proliferation and neurogenesis in the adult mouse dentate gyrus

1999 • 3,805 citations

More hippocampal neurons in adult mice living in an enriched environment

1997 • 3,476 citations

The Neuroendocrinology of Stress: A Never Ending Story

2008 • 3,415 citations

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group.

1993 • 3,080 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

Neural consequences of enviromental enrichment

2000 • 2,376 citations

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

1997 • 2,184 citations

Loss of Huntingtin-Mediated BDNF Gene Transcription in Huntington's Disease

2001 • 1,293 citations

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

2001 • 1,218 citations

Enhanced long-term potentiation and impaired learning in mice with mutant postsynaptic density-95 protein

1998 • 1,147 citations

Trinucleotide repeat length instability and age of onset in Huntington's disease

1993 • 1,062 citations

Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes

2003 • 900 citations

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

2003 • 833 citations

A YAC Mouse Model for Huntington’s Disease with Full-Length Mutant Huntingtin, Cytoplasmic Toxicity, and Selective Striatal Neurodegeneration

1999 • 831 citations

Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

1995 • 774 citations

Environmental enrichment inhibits spontaneous apoptosis, prevents seizures and is neuroprotective

1999 • 733 citations

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

2000 • 722 citations

Increased Sensitivity to N-Methyl-D-Aspartate Receptor-Mediated Excitotoxicity in a Mouse Model of Huntington's Disease

2002 • 603 citations

Effects of environmental enrichment on gene expression in the brain

2000 • 602 citations

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

1995 • 582 citations

Transcriptional abnormalities in Huntington disease

2003 • 525 citations

Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human Huntington disease gene

1998 • 509 citations

Rearing Complexity Affects Branching of Dendrites in the Visual Cortex of the Rat

1972 • 508 citations

Delaying the onset of Huntington's in mice

2000 • 507 citations

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

1998 • 490 citations

Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease

2000 • 433 citations

Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice

2003 • 417 citations

Abnormal Synaptic Plasticity and Impaired Spatial Cognition in Mice Transgenic for Exon 1 of the Human Huntington's Disease Mutation

2000 • 406 citations

Transcriptional dysregulation in Huntington’s disease

2000 • 401 citations

Ectopically Expressed CAG Repeats Cause Intranuclear Inclusions and a Progressive Late Onset Neurological Phenotype in the Mouse

1997 • 374 citations

Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain

2002 • 365 citations

Transient and Progressive Electrophysiological Alterations in the Corticostriatal Pathway in a Mouse Model of Huntington's Disease

2003 • 353 citations

Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease

1999 • 348 citations

Huntingtin Aggregate-Associated Axonal Degeneration is an Early Pathological Event in Huntington's Disease Mice

2001 • 347 citations

Environmental enrichment slows disease progression in R6/2 Huntington's disease mice

2002 • 333 citations

Brain-derived neurotrophic factor in Huntington disease

2000 • 326 citations

Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation

1997 • 319 citations

Dynamic mutations: A new class of mutations causing human disease

1992 • 315 citations

Mouse models of Huntington's disease

2002 • 304 citations

Mutant Huntingtin Expression in Clonal Striatal Cells: Dissociation of Inclusion Formation and Neuronal Survival by Caspase Inhibition

1999 • 301 citations

Changes in Cortical and Striatal Neurons Predict Behavioral and Electrophysiological Abnormalities in a Transgenic Murine Model of Huntington's Disease

2001 • 294 citations

Brain-derived neurotrophic factor rapidly enhances phosphorylation of the postsynaptic N -methyl- <scp>d</scp> -aspartate receptor subunit 1

1997 • 286 citations

Severe deficiencies in dopamine signaling in presymptomatic Huntington's disease mice

2000 • 284 citations

Changes in brain nerve growth factor levels and nerve growth factor receptors in rats exposed to environmental enrichment for one year

1999 • 257 citations

Genotypes at the GluR6 kainate receptor locus are associated with variation in the age of onset of Huntington disease

1997 • 245 citations

Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease

1999 • 241 citations

Impaired Synaptic Plasticity in Mice Carrying the Huntington's Disease Mutation

1999 • 231 citations

SH3GL3 Associates with the Huntingtin Exon 1 Protein and Promotes the Formation of Polygln-Containing Protein Aggregates

1998 • 219 citations

Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease

2000 • 215 citations

Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects

2002 • 213 citations

Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses

2002 • 196 citations

Apoptosis in Huntington's disease

2003 • 189 citations

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

1999 • 181 citations

Crystalline Fraction I Protein: Preparation in Large Yield

1972 • 170 citations

Polyglutamine-Expanded Ataxin-7 Promotes Non-Cell-Autonomous Purkinje Cell Degeneration and Displays Proteolytic Cleavage in Ataxic Transgenic Mice

2002 • 161 citations

Enhancement of hippocampal field potentials in rats exposed to a novel, complex environment

1985 • 145 citations

Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease

2002 • 142 citations

Evidence for the GluR6 gene associated with younger onset age of Huntington’s disease

1999 • 137 citations

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

2002 • 134 citations

Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice

2001 • 133 citations

Mechanism of altered synaptic strength due to experience: relation to long-term potentiation

1996 • 127 citations

Overexpression of heat shock protein 70 in R6/2 Huntington’s disease mice has only modest effects on disease progression

2003 • 121 citations

N-Acetylaspartate and DARPP-32 levels decrease in the corpus striatum of Huntingtonʼs disease mice

2000 • 120 citations

Essential fatty acids given from conception prevent topographies of motor deficit in a transgenic model of Huntington’s disease

2002 • 120 citations

Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis

2000 • 120 citations

Neurodegeneration: a failure of neuroregeneration?

2001 • 115 citations

Pyramidal Cell Loss in Motor Cortices in Huntington's Disease

2002 • 112 citations

Analysis of germline mutation spectra at the Huntington's disease locus supports a mitotic [correction of amitotic] mutation mechanism [published erratum appears in Hum Mol Genet 1999 Apr;8(4):717]

1999 • 100 citations

Trinucleotide (CAG) repeat length is positively correlated with the degree of DNA fragmentation in Huntington's disease striatum

1998 • 95 citations

Differential clinical and motor control function in a pair of monozygotic twins with Huntington's disease

1999 • 90 citations

Huntington's Disease (3rd edn)

2003 • 84 citations

A mouse model of spinal and bulbar muscular atrophy

2002 • 79 citations

Huntingtin Protein Colocalizes with Lesions of Neurodegenerative Diseases: An Investigation in Huntington's, Alzheimer's, and Pick's Diseases

1998 • 78 citations

Huntington's disease gene product, huntingtin, associates with microtubules in vitro

1997 • 66 citations

Anterior cingulate cortical transplantation in transgenic Huntington’s disease mice

2001 • 62 citations

Mice Overexpressing 70-kDa Heat Shock Protein Show Increased Resistance to Malonate and 3-Nitropropionic Acid

2002 • 50 citations

Aggregation of truncated GST--HD exon 1 fusion proteins containing normal range and expanded glutamine repeats

1999 • 45 citations

Neurofilament and neural cell adhesion molecule immunocytochemistry of Huntington's disease striatum

1992 • 24 citations