(Q45306743)

11 December 2017

R6/2 neurons with intranuclear inclusions survive for prolonged periods in the brains of chimeric mice (English)

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11 December 2017

Daniel Goldowitz

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author name string

Anton Reiner

1

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11 December 2017

Nobel Del Mar

2

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11 December 2017

Yun-Ping Deng

3

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11 December 2017

Christopher A Meade

4

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11 December 2017

Zhiqiang Sun

5

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11 December 2017

language of work or name

English

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publication date

1 December 2007

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The Journal of Comparative Neurology

11 December 2017

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11 December 2017

505

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11 December 2017

6

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11 December 2017

603-629

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Regional and progressive thinning of the cortical ribbon in Huntington's disease

11 December 2017

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Evidence for more widespread cerebral pathology in early HD: an MRI-based morphometric analysis

21 January 2018

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Neuronal intranuclear inclusions and neuropil aggregates in HdhCAG(150) knockin mice

21 January 2018

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Genetics and molecular biology of Huntington's disease

21 January 2018

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7 January 2021

Striatal and nigral neuron subpopulations in rigid Huntington's disease: implications for the functional anatomy of chorea and rigidity-akinesia

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7 January 2021

Preferential loss of striato-external pallidal projection neurons in presymptomatic Huntington's disease

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7 January 2021

Structure and expression of the Huntington's disease gene: evidence against simple inactivation due to an expanded CAG repeat

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7 January 2021

Extensive co-occurrence of substance P and dynorphin in striatal projection neurons: An evolutionarily conserved feature of basal ganglia organization

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7 January 2021

Are there multiple pathways in the pathogenesis of Huntington's disease?

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7 January 2021

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

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7 January 2021

Early striatal dendrite deficits followed by neuron loss with advanced age in the absence of anterograde cortical brain-derived neurotrophic factor.

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7 January 2021

Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease

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7 January 2021

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

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7 January 2021

Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation

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7 January 2021

Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation.

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7 January 2021

Normal huntingtin function: an alternative approach to Huntington's disease

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7 January 2021

Calbindin D-28k and parvalbumin in the rat nervous system

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7 January 2021

Monoclonal antibodies directed against the calcium binding protein parvalbumin

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7 January 2021

Monoclonal antibodies directed against the calcium binding protein Calbindin D-28k

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7 January 2021

NMDA receptor function in mouse models of Huntington disease

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7 January 2021

Transient and progressive electrophysiological alterations in the corticostriatal pathway in a mouse model of Huntington's disease.

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Evidence for the preferential localization of glutamate receptor-1 subunits of AMPA receptors to the dendritic spines of medium spiny neurons in rat striatum.

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7 January 2021

Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation

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7 January 2021

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

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7 January 2021

Polyglutamine expansion of huntingtin impairs its nuclear export

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7 January 2021

Detection of substance P in the central nervous system by a monoclonal antibody

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7 January 2021

Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.

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7 January 2021

Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

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7 January 2021

Monoclonal antibodies specific for glial fibrillary acidic (GFA) protein and for each of the neurofilament triplet polypeptides

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7 January 2021

Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease

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7 January 2021

Differential loss of striatal projection systems in Huntington's disease: a quantitative immunohistochemical study

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7 January 2021

Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons

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7 January 2021

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

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7 January 2021

Inactivation of the mouse Huntington's disease gene homolog Hdh

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7 January 2021

Molecular profile of reactive astrocytes--implications for their role in neurologic disease

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7 January 2021

Immunohistochemical studies using antibodies to leucine-enkephalin: initial observations on the nervous system of the rat

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7 January 2021

GFAP and astrogliosis.

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7 January 2021

Huntingtin interacts with a family of WW domain proteins

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7 January 2021

Selective sparing of a class of striatal neurons in Huntington's disease

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7 January 2021

Topography of enkephalin, substance P and acetylcholinesterase staining in Huntington's disease striatum

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7 January 2021

Morphologic and histochemical characteristics of a spared subset of striatal neurons in Huntington's disease

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7 January 2021

Sparing of acetylcholinesterase-containing striatal neurons in Huntington's disease

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7 January 2021

Relative survival of striatal projection neurons and interneurons after intrastriatal injection of quinolinic acid in rats

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7 January 2021

Relative resistance of striatal neurons containing calbindin or parvalbumin to quinolinic acid-mediated excitotoxicity compared to other striatal neuron types.

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7 January 2021

Promoter traps in embryonic stem cells: a genetic screen to identify and mutate developmental genes in mice

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7 January 2021

Cellular localization of huntingtin in striatal and cortical neurons in rats: lack of correlation with neuronal vulnerability in Huntington's disease.

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7 January 2021

Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules

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7 January 2021

Distribution of striatonigral and striatopallidal peptidergic neurons in both patch and matrix compartments: an in situ hybridization histochemistry and fluorescent retrograde tracing study

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7 January 2021

The pattern of neurodegeneration in Huntington's disease: a comparative study of cannabinoid, dopamine, adenosine and GABA(A) receptor alterations in the human basal ganglia in Huntington's disease

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7 January 2021

Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract

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7 January 2021

Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease

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7 January 2021

Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production

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7 January 2021

Trinucleotide instability: a repeating theme in human inherited disorders

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7 January 2021

Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.

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7 January 2021

The fatal attraction of polyglutamine-containing proteins.

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7 January 2021

The influence of huntingtin protein size on nuclear localization and cellular toxicity.

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7 January 2021

In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease.

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7 January 2021

Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease

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7 January 2021

Standardization and statistical approaches to therapeutic trials in the R6/2 mouse

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7 January 2021

A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.

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7 January 2021

Distribution of methionine and leucine enkephalin neurons within the social behavior circuitry of the male Syrian hamster brain

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7 January 2021

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

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7 January 2021

Early alterations in gene expression and cell morphology in a mouse model of Huntington's disease.

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7 January 2021

Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons.

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7 January 2021

A simple and sensitive antigen retrieval method for free-floating and slide-mounted tissue sections

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7 January 2021

Striatal interneurones: chemical, physiological and morphological characterization

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7 January 2021

Neuronal intranuclear inclusions in polyglutamine diseases: nuclear weapons or nuclear fallout?

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7 January 2021

Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition.

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7 January 2021

Calbindin D28K as a marker for the degeneration of the striatonigral pathway in Huntington's disease

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7 January 2021

Electrophysiological and morphological changes in striatal spiny neurons in R6/2 Huntington's disease transgenic mice

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7 January 2021

Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.

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7 January 2021

Three rat preprotachykinin mRNAs encode the neuropeptides substance P and neurokinin A

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7 January 2021

Restrictive clonal allocation in the chimeric mouse brain

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7 January 2021

Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease

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7 January 2021

Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats

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7 January 2021

Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity

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7 January 2021

Huntington's disease: a synaptopathy?

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7 January 2021

Intermediate filaments in nervous tissues

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7 January 2021

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

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Distribution of inclusions in neuronal nuclei and dystrophic neurites in Huntington disease brain

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7 January 2021

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

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7 January 2021

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

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7 January 2021

Transient global ischemia in rats yields striatal projection neuron and interneuron loss resembling that in Huntington's disease

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7 January 2021

Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice

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7 January 2021

Decrease in striatal enkephalin mRNA in mouse models of Huntington's disease

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7 January 2021

Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice

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7 January 2021

Progressive formation of inclusions in the striatum and hippocampus of mice transgenic for the human Huntington's disease mutation

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7 January 2021

Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes

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7 January 2021

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

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7 January 2021

Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse.

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7 January 2021

Ubiquitin is detected in neurofibrillary tangles and senile plaque neurites of Alzheimer disease brains

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7 January 2021

Differential expression of normal and mutant Huntington's disease gene alleles

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7 January 2021

Basal forebrain neuronal loss in mice lacking neurotrophin receptor p75.

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7 January 2021

Poly- and monoclonal antibodies against recombinant rat brain calbindin D-28K were produced to map its selective distribution in the central nervous system

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7 January 2021

Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction.

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7 January 2021

Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA.

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7 January 2021

The distribution of proenkephalin-derived peptides in the central nervous system of turtles

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7 January 2021

A Comparison of Neurotransmitter-Specific and Neuropeptide-Specific Neuronal Cell Types Present in the Dorsal Cortex in Turtles with Those Present in the Isocortex in Mammals: Implications for the Evolution of Isocortex; pp. 53–72

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7 January 2021

The patterns of neurotransmitter and neuropeptide co-occurrence among striatal projection neurons: conclusions based on recent findings

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7 January 2021

Differential loss of striatal projection neurons in Huntington disease

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7 January 2021

Neurons lacking huntingtin differentially colonize brain and survive in chimeric mice

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7 January 2021

Wild-type huntingtin plays a role in brain development and neuronal survival

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7 January 2021

Brain neurotransmitter deficits in mice transgenic for the Huntington's disease mutation

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7 January 2021

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

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7 January 2021

Wild-type huntingtin protects from apoptosis upstream of caspase-3.

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7 January 2021

Regional cortical thinning in preclinical Huntington disease and its relationship to cognition

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7 January 2021

Caspase-8 is required for cell death induced by expanded polyglutamine repeats.

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7 January 2021

Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis

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7 January 2021

Huntingtin localization in brains of normal and Huntington's disease patients

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7 January 2021

Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease

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7 January 2021

Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.

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7 January 2021

Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo

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7 January 2021

Expression of the Huntington's disease (IT15) protein product in HD patients

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7 January 2021

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin

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7 January 2021

Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice

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7 January 2021

Widespread expression of Huntington's disease gene (IT15) protein product

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7 January 2021

Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein

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7 January 2021

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

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7 January 2021

Nuclear inclusions in glutamine repeat disorders: are they pernicious, coincidental, or beneficial?

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7 January 2021

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

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7 January 2021

Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions

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7 January 2021

Dendritic spine pathology and deficits in experience-dependent dendritic plasticity in R6/1 Huntington's disease transgenic mice

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7 January 2021

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice

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7 January 2021

The cortical lesion of Huntington's disease: further neurochemical characterization, and reproduction of some of the histological and neurochemical features by N-methyl-D-aspartate lesions of rat cortex

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7 January 2021

Differential changes in striatal projection neurons in R6/2 transgenic mice for Huntington's disease

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7 January 2021

Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse

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7 January 2021

Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

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7 January 2021

Microtubule destabilization and nuclear entry are sequential steps leading to toxicity in Huntington's disease

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7 January 2021

Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro

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7 January 2021

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

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7 January 2021

Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways

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7 January 2021

Neuropathological classification of Huntington's disease

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7 January 2021

Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract

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7 January 2021

Homozygotes for Huntington's disease

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7 January 2021

Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion

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7 January 2021

Memory failure in Huntington's disease

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7 January 2021

Partial characterisation of murine huntingtin and apparent variations in the subcellular localisation of huntingtin in human, mouse and rat brain.

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7 January 2021

Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

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7 January 2021

Neuralized functions as an E3 ubiquitin ligase during Drosophila development

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7 January 2021

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

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7 January 2021

Total number of neurons in the habenular nuclei of the rat epithalamus: a stereological study.

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7 January 2021

Identifiers

DOI

10.1002/CNE.21515

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11 December 2017

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