Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease

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

1993 • 7,941 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

Neuropathological Classification of Huntingtonʼs Disease

1985 • 2,517 citations

NeuN, a neuronal specific nuclear protein in vertebratesxs

1992 • 2,268 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

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

2001 • 1,293 citations

Lesion of striatal neurons with kainic acid provides a model for Huntington's chorea

1976 • 1,247 citations

Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines

2002 • 1,009 citations

Characterization of Progressive Motor Deficits in Mice Transgenic for the Human Huntington’s Disease Mutation

1999 • 933 citations

Nuclear and Neuropil Aggregates in Huntington’s Disease: Relationship to Neuropathology

1999 • 879 citations

Duplication of biochemical changes of Huntington's chorea by intrastriatal injections of glutamic and kainic acids

1976 • 832 citations

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

1999 • 831 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

Huntington's Disease

1979 • 607 citations

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

2002 • 603 citations

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

2001 • 576 citations

Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

2000 • 553 citations

Neuroprotective Effects of Creatine in a Transgenic Mouse Model of Huntington's Disease

2000 • 526 citations

Genetic determinants of susceptibility to excitotoxic cell death: Implications for gene targeting approaches

1997 • 523 citations

Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice

2000 • 456 citations

Proteases Acting on Mutant Huntingtin Generate Cleaved Products that Differentially Build Up Cytoplasmic and Nuclear Inclusions

2002 • 408 citations

Caspase Cleavage of Mutant Huntingtin Precedes Neurodegeneration in Huntington's Disease

2002 • 386 citations

Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis

2001 • 382 citations

The likelihood of being affected with Huntington disease by a particular age, for a specific CAG size.

1997 • 365 citations

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

1999 • 348 citations

Calpain Activation in Huntington's Disease

2002 • 321 citations

The Influence of Huntingtin Protein Size on Nuclear Localization and Cellular Toxicity

1998 • 313 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

Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease

2002 • 293 citations

NMDA receptor function in mouse models of Huntington disease

2001 • 277 citations

A Huntington's Disease CAG Expansion at the Murine Hdh Locus Is Unstable and Associated with Behavioural Abnormalities in Mice

1999 • 265 citations

Wild-Type Huntingtin Reduces the Cellular Toxicity of Mutant Huntingtin In Vivo

2001 • 237 citations

‘Tissue’ transglutaminase ablation reduces neuronal death and prolongs survival in a mouse model of Huntington's disease

2002 • 227 citations

Early Motor Dysfunction and Striosomal Distribution of Huntingtin Microaggregates in Huntington's Disease Knock-In Mice

2002 • 220 citations

Huntington's chorea.

1967 • 211 citations

Quantitative immunocytochemistry of DARPP-32-expressing neurons in the rat caudatoputamen

1998 • 197 citations

Mutant Huntingtin Enhances Excitotoxic Cell Death

2001 • 191 citations

Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice

2002 • 177 citations

The Cellular and Subcellular Localization of Huntingtin-Associated Protein 1 (HAP1): Comparison with Huntingtin in Rat and Human

1998 • 174 citations

Striatal volume loss in HD as measured by MRI and the influence of CAG repeat

2001 • 155 citations

A simple, single-step technique for selecting and cloning hybridomas for the production of monoclonal antibodies

1982 • 148 citations

Evaluation of R6/2 HD transgenic mice for therapeutic studies in Huntington's disease: behavioral testing and impact of diabetes mellitus

2001 • 135 citations

Human huntingtin derived from YAC transgenes compensates for loss of murine huntingtin by rescue of the embryonic lethal phenotype

1996 • 100 citations

Modulation of Cell Death by Mouse Genotype: Differential Vulnerability to Excitatory Amino Acid-Induced Lesions

2002 • 68 citations

Insertion of a pathogenic mutation into a yeast artificial chromosome containing the human amyloid precursor protein gene.

1994 • 33 citations

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

1999 • 29 citations