Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models. (Q40148564)

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28 December 2019

title

Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models (English)

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28 December 2019

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3

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28 December 2019

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Christopher A Ross

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28 December 2019

author name string

Gabriele Schilling

1

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28 December 2019

Alexandra Klevytska

2

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28 December 2019

Katrin Juenemann

4

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28 December 2019

Jillian Cooper

5

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28 December 2019

Victoria Gonzales

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28 December 2019

Hilda Slunt

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28 December 2019

Michelle Poirer

8

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28 December 2019

David R Borchelt

10

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28 December 2019

language of work or name

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1 April 2007

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Journal of Neuropathology & Experimental Neurology

28 December 2019

published in

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28 December 2019

volume

66

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28 December 2019

issue

4

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28 December 2019

page(s)

313-320

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

28 December 2019

cites work

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When more is less: pathogenesis of glutamine repeat neurodegenerative diseases

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Trinucleotide repeats in neurogenetic disorders

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Clinical and neuropathologic assessment of severity in Huntington's disease

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity.

21 January 2018

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Progressive phenotype and nuclear accumulation of an amino-terminal cleavage fragment in a transgenic mouse model with inducible expression of full-length mutant huntingtin

21 January 2018

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

21 January 2018

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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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions

21 January 2018

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

21 January 2018

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Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus

21 January 2018

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Polyglutamine repeat length-dependent proteolysis of huntingtin

21 January 2018

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Lysosomal proteases are involved in generation of N-terminal huntingtin fragments

21 January 2018

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Isolation of a 40-kDa Huntingtin-associated protein

21 January 2018

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

21 January 2018

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Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4

21 January 2018

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

21 January 2018

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Precursor convertases in the secretory pathway, cytosol and extracellular milieu

21 January 2018

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On the sequential determinants of calpain cleavage

21 January 2018

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10.1097/NEN.0B013E318040B2C8

21 January 2018

Identifiers

DOI

1 reference

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28 December 2019

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