(Q34408222)

2 August 2017

Modeling Huntington's disease in cells, flies, and mice (English)

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2 August 2017

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2

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2 August 2017

S Sipione

1

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2 August 2017

1 February 2001

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2 August 2017

Molecular Neurobiology

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2 August 2017

23

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2 August 2017

21-51

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2 August 2017

1

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https://scigraph.springernature.com/pub.10.1385/mn:23:1:21

2 August 2017

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cites work

Generation of neuronal intranuclear inclusions by polyglutamine-GFP: analysis of inclusion clearance and toxicity as a function of polyglutamine length.

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12 December 2020

Inhibition of polyglutamine protein aggregation and cell death by novel peptides identified by phage display screening

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12 December 2020

Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation.

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12 December 2020

HEAT repeats in the Huntington's disease protein

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12 December 2020

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

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12 December 2020

Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity

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12 December 2020

Juvenile Huntington disease

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12 December 2020

Genotypes at the GluR6 kainate receptor locus are associated with variation in the age of onset of Huntington disease

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12 December 2020

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

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12 December 2020

Genetic suppression of polyglutamine toxicity in Drosophila

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12 December 2020

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

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12 December 2020

Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy

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12 December 2020

Huntington disease: clinical, genetic, and social aspects

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12 December 2020

HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain

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12 December 2020

CAG expansion affects the expression of mutant Huntingtin in the Huntington's disease brain

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12 December 2020

Transcriptional activation modulated by homopolymeric glutamine and proline stretches

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12 December 2020

Tight control of gene expression in mammalian cells by tetracycline-responsive promoters

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12 December 2020

Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy.

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12 December 2020

Diabetes mellitus in Huntington disease

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12 December 2020

Body weight and dietary factors in Huntington's disease patients compared with matched controls

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12 December 2020

Inhibition of ICE slows ALS in mice

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12 December 2020

SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes

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12 December 2020

Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

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12 December 2020

Life without huntingtin: normal differentiation into functional neurons

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12 December 2020

Abnormalities of striatal projection neurons and N-methyl-D-aspartate receptors in presymptomatic Huntington's disease

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12 December 2020

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

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12 December 2020

Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization

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12 December 2020

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

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12 December 2020

Reversing neurodegeneration: a promise unfolds

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12 December 2020

Analysis of the 5' upstream sequence of the Huntington's disease (HD) gene shows six new rare alleles which are unrelated to the age at onset of HD.

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12 December 2020

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

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12 December 2020

Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

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12 December 2020

Huntington's disease gene (IT15) is widely expressed in human and rat tissues

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12 December 2020

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

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12 December 2020

A short term analysis of the behaviour of conditionally immortalized neuronal progenitors and primary neuroepithelial cells implanted into the fetal rat brain.

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12 December 2020

DNA-binding properties of the product of the testis-determining gene and a related protein

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12 December 2020

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

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12 December 2020

Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid

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12 December 2020

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

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12 December 2020

Expression of extended polyglutamine sequentially activates initiator and effector caspases

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12 December 2020

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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12 December 2020

A polymorphic DNA marker genetically linked to Huntington's disease

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12 December 2020

Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease

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12 December 2020

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

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12 December 2020

Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues

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12 December 2020

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

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12 December 2020

Differential 3' polyadenylation of the Huntington disease gene results in two mRNA species with variable tissue expression

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12 December 2020

Expression of normal and mutant huntingtin in the developing brain

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12 December 2020

A huntingtin-associated protein enriched in brain with implications for pathology

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme

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12 December 2020

ST14A cells have properties of a medium-size spiny neuron.

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12 December 2020

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

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12 December 2020

Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.

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12 December 2020

Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?

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12 December 2020

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

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12 December 2020

mRNAs encoding a von Ebner's-like protein and the Huntington disease protein are induced in rat male germ cells by Sertoli cells

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12 December 2020

Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila.

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12 December 2020

Ancestral differences in the distribution of the delta 2642 glutamic acid polymorphism is associated with varying CAG repeat lengths on normal chromosomes: insights into the genetic evolution of Huntington disease

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12 December 2020

Instability of CAG repeats in Huntington's disease: relation to parental transmission and age of onset

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12 December 2020

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

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12 December 2020

Huntingtin is required for normal hematopoiesis

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12 December 2020

Aggregates in neurodegenerative disease: crowds and power?

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12 December 2020

Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron

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12 December 2020

Quinolinic acid: an endogenous metabolite that produces axon-sparing lesions in rat brain

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12 December 2020

Energetics in the pathogenesis of neurodegenerative diseases

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12 December 2020

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

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12 December 2020

Differential loss of striatal projection neurons in Huntington disease

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12 December 2020

Replicating Huntington's disease phenotype in experimental animals

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12 December 2020

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

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12 December 2020

Characteristics of individuals with Huntington disease in long-term care

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12 December 2020

Polyglutamine-induced ion channels: a possible mechanism for the neurotoxicity of Huntington and other CAG repeat diseases

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12 December 2020

Activation of MLK2-mediated signaling cascades by polyglutamine-expanded huntingtin

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12 December 2020

Huntington's disease. In reverse gear

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12 December 2020

Towards prediction of cognate complexes between the WW domain and proline-rich ligands

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12 December 2020

Transcriptional dysregulation in Huntington's disease

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12 December 2020

Fast transport and retrograde movement of huntingtin and HAP 1 in axons.

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12 December 2020

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

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12 December 2020

Expression of polyglutamine-expanded Huntingtin activates the SEK1-JNK pathway and induces apoptosis in a hippocampal neuronal cell line

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12 December 2020

Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases

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12 December 2020

Expression of the Huntington disease gene in rodents: cloning the rat homologue and evidence for downregulation in non-neuronal tissues during development

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12 December 2020

Dramatic mutation instability in HD mouse striatum: does polyglutamine load contribute to cell-specific vulnerability in Huntington's disease?

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12 December 2020

Role of Hsp70 in regulation of stress-kinase JNK: implications in apoptosis and aging

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12 December 2020

Huntingtin interacts with a family of WW domain proteins

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12 December 2020

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

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12 December 2020

Neuronal degeneration in the basal ganglia and loss of pallido-subthalamic synapses in mice with targeted disruption of the Huntington's disease gene

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12 December 2020

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

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12 December 2020

Survival, integration, and differentiation of neural stem cell lines after transplantation to the adult rat striatum

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12 December 2020

A one-hit model of cell death in inherited neuronal degenerations

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12 December 2020

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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12 December 2020

SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates

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12 December 2020

Unstable triplet repeat diseases.

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12 December 2020

Higher sedentary energy expenditure in patients with Huntington's disease

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin

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12 December 2020

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12 December 2020

Huntingtin interacts with cystathionine beta-synthase

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12 December 2020

Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo

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12 December 2020

Motor disorder in Huntington's disease begins as a dysfunction in error feedback control.

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12 December 2020

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

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12 December 2020

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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12 December 2020

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

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12 December 2020

The identification of a functional nuclear localization signal in the Huntington disease protein

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12 December 2020

Differential distribution of the normal and mutated forms of huntingtin in the human brain

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12 December 2020

Loss of normal huntingtin function: new developments in Huntington's disease research

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12 December 2020

Immortalized murine striatal neuronal cell lines expressing dopamine receptors and cholinergic properties

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12 December 2020

Genetic polymorphisms adjacent to the CAG repeat influence clinical features at onset in Huntington's disease

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12 December 2020

Establishment of a noradrenergic clonal line of rat adrenal pheochromocytoma cells which respond to nerve growth factor

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12 December 2020

Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease

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12 December 2020

Isolation of a 40-kDa Huntingtin-associated protein

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12 December 2020

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

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12 December 2020

Mitogen-activated protein kinase kinase 7 is an activator of the c-Jun NH2-terminal kinase

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12 December 2020

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

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12 December 2020

A CCG repeat polymorphism adjacent to the CAG repeat in the Huntington disease gene: implications for diagnostic accuracy and predictive testing

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12 December 2020

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

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12 December 2020

Formation of polyglutamine inclusions in non-CNS tissue

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12 December 2020

Characterization of a novel protein-binding module--the WW domain

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12 December 2020

Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells

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12 December 2020

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

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12 December 2020

Huntingtin's WW domain partners in Huntington's disease post-mortem brain fulfill genetic criteria for direct involvement in Huntington's disease pathogenesis

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12 December 2020

Anticipation and instability of IT-15 (CAG)n repeats in parent-offspring pairs with Huntington disease

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12 December 2020

Activation of the JAK/STAT pathway leads to proliferation of ST14A central nervous system progenitor cells

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12 December 2020

Expression of the Huntington's disease gene is regulated in astrocytes in the arcuate nucleus of the hypothalamus of postpartum rats.

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12 December 2020

Weight loss in Huntington's disease.

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12 December 2020

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

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12 December 2020

Abnormalities in the functioning of adipocytes from R6/2 mice that are transgenic for the Huntington's disease mutation

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12 December 2020

12-O-tetradecanoyl-phorbol-13-acetate down-regulates the Huntingtin promoter at Sp1 sites

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12 December 2020

Morphometric analysis of the prefrontal cortex in Huntington's disease

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12 December 2020

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

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12 December 2020

Activation of G1 progression, JNK mitogen-activated protein kinase, and actin filament assembly by the exchange factor FGD1

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12 December 2020

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

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12 December 2020

Of mice and men: solving the molecular mysteries of Huntington's disease

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Polyglutamine diseases: protein cleavage and aggregation.

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12 December 2020

CAG repeat size and clinical presentation in Huntington's disease

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12 December 2020

Generation and characterization of embryonic striatal conditionally immortalized ST14A cells

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12 December 2020

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

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12 December 2020

A single allele from the polymorphic CCG rich sequence immediately 3' to the unstable CAG trinucleotide in the IT15 cDNA shows almost complete disequilibrium with Huntington's disease chromosomes in the Scottish population

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12 December 2020

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

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12 December 2020

Gene therapy of experimental brain tumors using neural progenitor cells

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12 December 2020

Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells

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12 December 2020

DNA haplotype analysis of Huntington disease reveals clues to the origins and mechanisms of CAG expansion and reasons for geographic variations of prevalence

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12 December 2020

Expansion of polyglutamine repeat in huntingtin leads to abnormal protein interactions involving calmodulin

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12 December 2020

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

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12 December 2020

Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles

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12 December 2020

Isolation and characterization of the rat huntingtin promoter.

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12 December 2020

Analysis of the huntingtin gene reveals a trinucleotide-length polymorphism in the region of the gene that contains two CCG-rich stretches and a correlation between decreased age of onset of Huntington's disease and CAG repeat number

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12 December 2020

DNA Binding Activity of Recombinant SRY from Normal Males and XY Females

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12 December 2020

Trinucleotide repeat length and clinical progression in Huntington's disease

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12 December 2020

HAP1-huntingtin interactions do not contribute to the molecular pathology in Huntington's disease transgenic mice

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12 December 2020

Structural analysis of the 5' region of mouse and human Huntington disease genes reveals conservation of putative promoter region and di- and trinucleotide polymorphisms

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12 December 2020

The normal Huntington disease (HD) allele, or a closely linked gene, influences age at onset of HD

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12 December 2020

Mouse mutant embryos lacking huntingtin are rescued from lethality by wild-type extraembryonic tissues.

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12 December 2020

Fourteen and counting: unraveling trinucleotide repeat diseases

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12 December 2020

Neurobiology of Huntington's disease

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12 December 2020

Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis

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12 December 2020

Excitotoxin lesions in primates as a model for Huntington's disease: histopathologic and neurochemical characterization

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12 December 2020

Huntingtin interacting protein 1 induces apoptosis via a novel caspase-dependent death effector domain

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12 December 2020

Expression of huntingtin-associated protein-1 in neuronal cells implicates a role in neuritic growth

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12 December 2020

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

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12 December 2020

HIP-I: a huntingtin interacting protein isolated by the yeast two-hybrid system

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12 December 2020

Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease

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12 December 2020

Inactivation of the mouse Huntington's disease gene homolog Hdh

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12 December 2020

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

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12 December 2020

Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease

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12 December 2020

Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis.

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12 December 2020

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

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12 December 2020

Attenuation of transient focal cerebral ischemic injury in transgenic mice expressing a mutant ICE inhibitory protein.

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12 December 2020

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

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12 December 2020

Structural view of the Ran-Importin beta interaction at 2.3 A resolution

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12 December 2020

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Peptides containing glutamine repeats as substrates for transglutaminase-catalyzed cross-linking: relevance to diseases of the nervous system

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12 December 2020

Molecular genetics of Huntington's disease

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12 December 2020

Forskolin and dopamine D1 receptor activation increase huntingtin's association with endosomes in immortalized neuronal cells of striatal origin.

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12 December 2020

Aberrant amplification of A(2A) receptor signaling in striatal cells expressing mutant huntingtin

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12 December 2020

Identifiers

DOI

10.1385/MN:23:1:21

1 reference

PubMed ID

11642542

Dimensions Publication ID

2 August 2017

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1 reference

PubMed ID

11642542