Longitudinal behavioral, cross-sectional transcriptional and histopathological characterization of a knock-in mouse model of Huntington's disease with 140 CAG repeats (Q34699467)

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English	Longitudinal behavioral, cross-sectional transcriptional and histopathological characterization of a knock-in mouse model of Huntington's disease with 140 CAG repeats	scientific article

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scholarly article

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Europe PubMed Central

PubMed publication ID

4 August 2017

Longitudinal behavioral, cross-sectional transcriptional and histopathological characterization of a knock-in mouse model of Huntington's disease with 140 CAG repeats (English)

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Europe PubMed Central

PubMed publication ID

4 August 2017

Huntington's disease

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author name string

Aaron C Rising

1

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Europe PubMed Central

PubMed publication ID

4 August 2017

Jia Xu

2

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Europe PubMed Central

PubMed publication ID

4 August 2017

Aaron Carlson

3

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Europe PubMed Central

PubMed publication ID

4 August 2017

Vincent V Napoli

4

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Europe PubMed Central

PubMed publication ID

4 August 2017

Eileen M Denovan-Wright

5

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Europe PubMed Central

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4 August 2017

Ronald J Mandel

6

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4 August 2017

language of work or name

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publication date

28 December 2010

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Europe PubMed Central

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4 August 2017

Experimental Neurology

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4 August 2017

228

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Europe PubMed Central

PubMed publication ID

4 August 2017

2

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Europe PubMed Central

PubMed publication ID

4 August 2017

173-182

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Europe PubMed Central

PubMed publication ID

4 August 2017

The Huntington's disease mutation impairs Huntingtin's role in the transport of NF-κB from the synapse to the nucleus.

1 reference

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7 July 2018

Onset and progression of pathologic atrophy in Huntington disease: a longitudinal MR imaging study

1 reference

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7 July 2018

Distinct contributions of the hippocampus and medial prefrontal cortex to the "what-where-when" components of episodic-like memory in mice

1 reference

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7 July 2018

Altered CB1 receptor and endocannabinoid levels precede motor symptom onset in a transgenic mouse model of Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Sustained effects of nonallele-specific Huntingtin silencing

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

The current clinical management of Huntington's disease

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7 July 2018

Cerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Implicit and explicit aspects of sequence learning in pre-symptomatic Huntington's disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits

1 reference

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7 July 2018

Phosphodiesterase 10A inhibition is associated with locomotor and cognitive deficits and increased anxiety in mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Widespread disruption of repressor element-1 silencing transcription factor/neuron-restrictive silencer factor occupancy at its target genes in Huntington's disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Sex differences in behavior and striatal ascorbate release in the 140 CAG knock-in mouse model of Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Cyclic nucleotide phosphodiesterases: molecular regulation to clinical use

1 reference

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7 July 2018

Preclinical Huntington's disease: compensatory brain responses during learning

1 reference

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7 July 2018

Locomotor effects of a D1R agonist are DARPP-32 dependent in adult but not weanling mice

1 reference

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7 July 2018

Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6/1 Huntington's disease transgenic mice

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7 July 2018

RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model

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7 July 2018

Polyglutamine represses cAMP-responsive-element-mediated transcription without aggregate formation.

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7 July 2018

Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation

1 reference

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7 July 2018

Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset

1 reference

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7 July 2018

Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats

1 reference

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7 July 2018

Stimulation of NeuroD activity by huntingtin and huntingtin-associated proteins HAP1 and MLK2

1 reference

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7 July 2018

The predominantly HEAT-like motif structure of huntingtin and its association and coincident nuclear entry with dorsal, an NF-kB/Rel/dorsal family transcription factor

1 reference

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7 July 2018

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

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

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

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7 July 2018

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

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7 July 2018

Huntington's disease: a review of the literature on prevalence and treatment of neuropsychiatric phenomena

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7 July 2018

Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Cannabinoid receptor messenger RNA levels decrease in a subset of neurons of the lateral striatum, cortex and hippocampus of transgenic Huntington's disease mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

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

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

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

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7 July 2018

Isolation and characterization of PDE10A, a novel human 3', 5'-cyclic nucleotide phosphodiesterase

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7 July 2018

Cloning and characterization of a novel human phosphodiesterase that hydrolyzes both cAMP and cGMP (PDE10A)

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7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

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

1 reference

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7 July 2018

Evidence for specific cognitive deficits in preclinical Huntington's disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length

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7 July 2018

Genomic structure and chromosome location of the murine PDE1B phosphodiesterase gene

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7 July 2018

Immunohistochemical distribution of cannabinoid CB1 receptors in the rat central nervous system

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7 July 2018

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

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7 July 2018

Concurrent stimulation of cannabinoid CB1 and dopamine D2 receptors augments cAMP accumulation in striatal neurons: evidence for a Gs linkage to the CB1 receptor.

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7 July 2018

Executive and mnemonic functions in early Huntington's disease

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7 July 2018

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 July 2018

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

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7 July 2018

Clinical consequences of isolating the gene for Huntington's disease

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7 July 2018

DARPP-32, a dopamine- and adenosine 3':5'-monophosphate-regulated phosphoprotein enriched in dopamine-innervated brain regions. I. Regional and cellular distribution in the rat brain

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7 July 2018

Opposing roles for D-1 and D-2 dopamine receptors in efflux of cyclic AMP from rat neostriatum

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7 July 2018

Development of Neuropsychological Deficits in Huntington's Disease

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7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

D-2 receptor stimulation inhibits cyclic AMP formation brought about by D-1 receptor stimulation in rat neostriatum but not nucleus accumbens

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Huntington disease in Maryland: clinical aspects of racial variation

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Neuropathological classification of Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Differential loss of striatal projection neurons in Huntington disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Dopamine receptor gene expression by enkephalin neurons in rat forebrain.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Neuronal localization of cannabinoid receptors in the basal ganglia of the rat.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Preproenkephalin messenger RNA?containing neurons in striatum of patients with symptomatic and presymptomatic huntington's disease: An in situ hybridization study

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Phenotypical characterization of the rat striatal neurons expressing the D1 dopamine receptor gene

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Cannabinoid Receptor-Regulated Cyclic AMP Accumulation in the Rat Striatum

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

7 July 2018

Dynamic regulation of NGFI-A (zif268, egr1) gene expression in the striatum

1 reference

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7 July 2018

Quantitative comparison of phosphodiesterase mRNA distribution in human brain and peripheral tissues

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

The progression of regional atrophy in premanifest and early Huntington's disease: a longitudinal voxel-based morphometry study

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Prefrontal cortex volume reduction on MRI in preclinical Huntington's disease relates to visuomotor performance and CAG number

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Early cognitive deficits in Swedish gene carriers of Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Striatal and extrastriatal atrophy in Huntington's disease and its relationship with length of the CAG repeat

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Executive dysfunction in early stages of Huntington's disease is associated with striatal and insular atrophy: a neuropsychological and voxel-based morphometric study.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Normative estimates of cross-sectional and longitudinal brain volume decline in aging and AD.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Mutant huntingtin affects the rate of transcription of striatum-specific isoforms of phosphodiesterase 10A.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Structure, expression and regulation of the cannabinoid receptor gene (CB1) in Huntington's disease transgenic mice

1 reference

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26 October 2018

Striatal phosphodiesterase mRNA and protein levels are reduced in Huntington's disease transgenic mice prior to the onset of motor symptoms

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Decreased cAMP response element-mediated transcription: an early event in exon 1 and full-length cell models of Huntington's disease that contributes to polyglutamine pathogenesis

1 reference

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26 October 2018

A longitudinal study of brain volume changes in normal aging using serial registered magnetic resonance imaging

1 reference

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26 October 2018

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

1 reference

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26 October 2018

Longitudinal Magnetic Resonance Imaging Studies of Older Adults: A Shrinking Brain

1 reference

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26 October 2018

Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice

1 reference

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26 October 2018

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

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26 October 2018

Behavior in Huntington's disease: dissociating cognition-based and mood-based changes

1 reference

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26 October 2018

Behavioral changes in Huntington Disease

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26 October 2018

Behavioral and neurochemical changes following predatory stress in mice.

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26 October 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

N-Acetylaspartate and DARPP-32 levels decrease in the corpus striatum of Huntington's disease mice

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Regional specificity of brain atrophy in Huntington's disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Bidirectional regulation of DARPP-32 phosphorylation by dopamine.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Dopamine D1 and D2 receptor gene expression in the striatum in Huntington's disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Intact mirror-tracing and impaired rotary-pursuit skill learning in patients with Huntington's disease: evidence for dissociable memory systems in skill learning

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3060971

26 October 2018

Identifiers

10.1016/J.EXPNEUROL.2010.12.017

1 reference

Europe PubMed Central

PubMed publication ID

4 August 2017

PMC publication ID

1 reference

Europe PubMed Central

PubMed publication ID

4 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

4 August 2017

ResearchGate publication ID

0 references

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