Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels, and protein length. (Q40644113)

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scientific article published on May 2002

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English	Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels, and protein length.	scientific article published on May 2002

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10 December 2019

Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels, and protein length (English)

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10 December 2019

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Luis Pereira de Almeida

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10 December 2019

Christopher A Ross

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10 December 2019

Diana Zala

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10 December 2019

Patrick Aebischer

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10 December 2019

Nicole Déglon

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10 December 2019

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1 May 2002

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10 December 2019

Journal of Neuroscience

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10 December 2019

22

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10 December 2019

9

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10 December 2019

3473-3483

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10 December 2019

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

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Distinct behavioral and neuropathological abnormalities in transgenic mouse models of HD and DRPLA.

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Reduced cerebral glucose metabolism in asymptomatic subjects at risk for Huntington's disease

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12 December 2020

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

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

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Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

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Rate of caudate atrophy in presymptomatic and symptomatic stages of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

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Self-inactivating lentiviral vectors with enhanced transgene expression as potential gene transfer system in Parkinson's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

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Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

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Neuronal intranuclear inclusions in spinocerebellar ataxia type 2: triple-labeling immunofluorescent study

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

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Complete and long-term rescue of lesioned adult motoneurons by lentiviral-mediated expression of glial cell line-derived neurotrophic factor in the facial nucleus.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Fluoro-Jade: a novel fluorochrome for the sensitive and reliable histochemical localization of neuronal degeneration

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Localization of rabbit huntingtin using a new panel of monoclonal antibodies

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Dopamine modulates the susceptibility of striatal neurons to 3-nitropropionic acid in the rat model of Huntington's disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

High level expression of expanded full-length ataxin-3 in vitro causes cell death and formation of intranuclear inclusions in neuronal cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

In vivo gene delivery and stable transduction of nondividing cells by a lentiviral vector

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neuroprotective effect of a CNTF-expressing lentiviral vector in the quinolinic acid rat model of Huntington's disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Quantitative neuropathological changes in presymptomatic Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A cellular model that recapitulates major pathogenic steps of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

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Recent advances in understanding the pathogenesis of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

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Subset of neurons characterized by the presence of NADPH-diaphorase in human substantia innominata

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

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Beyond the dopamine receptor: the DARPP-32/protein phosphatase-1 cascade

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A comparison of neurological, metabolic, structural, and genetic evaluations in persons at risk for Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Intranuclear inclusions in subtypes of striatal neurons in Huntington's disease transgenic mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mutation size and age at onset in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntington's disease progression. PET and clinical observations.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurodegeneration prevented by lentiviral vector delivery of GDNF in primate models of Parkinson's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Highly efficient and sustained gene transfer in adult neurons with a lentivirus vector.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Lentiviruses as gene transfer agents for delivery to non-dividing cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Toward an understanding of polyglutamine neurodegeneration

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Intraneuronal aggregate formation and cell death after viral expression of expanded polyglutamine tracts in the adult rat brain.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntington disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Striatal glucose metabolism and dopamine D2 receptor binding in asymptomatic gene carriers and patients with Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Relationship between trinucleotide repeats and neuropathological changes in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Transgenic mice expressing mutated full-length HD cDNA: a paradigm for locomotor changes and selective neuronal loss in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Normal and expanded Huntington's disease gene alleles produce distinguishable proteins due to translation across the CAG repeat.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Lentiviral vectors: regulated gene expression

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Fluoro-Jade B: a high affinity fluorescent marker for the localization of neuronal degeneration

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A stable human-derived packaging cell line for production of high titer retrovirus/vesicular stomatitis virus G pseudotypes

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Reduced basal ganglia volume associated with the gene for Huntington's disease in asymptomatic at-risk persons.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntington aggregates may not predict neuronal death in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11978824

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1523/JNEUROSCI.22-09-03473.2002

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:11978824%20AND%20SRC:MED&resulttype=core&format=json

10 December 2019

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:11978824%20AND%20SRC:MED&resulttype=core&format=json

10 December 2019

PubMed publication ID

1 reference

Europe PubMed Central

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:11978824%20AND%20SRC:MED&resulttype=core&format=json

10 December 2019

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