Huntington's Disease: Mechanisms of Pathogenesis and Therapeutic Strategies (Q39035495)

8 September 2017

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title

Huntington's Disease: Mechanisms of Pathogenesis and Therapeutic Strategies (English)

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8 September 2017

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Maria Jimenez-Sanchez

1

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8 September 2017

Floriana Licitra

2

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8 September 2017

Benjamin R Underwood

3

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8 September 2017

David C Rubinsztein

4

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8 September 2017

publication date

9 December 2016

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Cold Spring Harbor Perspectives in Medicine

8 September 2017

published in

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8 September 2017

volume

7

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8 September 2017

issue

7

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Changes in expression of N-methyl-D-aspartate receptor subunits occur early in the R6/2 mouse model of Huntington's disease

8 September 2017

cites work

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

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Complex I defect in muscle from patients with Huntington's disease.

21 January 2018

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https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Weight loss in Huntington disease increases with higher CAG repeat number

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

A pathogenic mechanism in Huntington's disease involves small CAG-repeated RNAs with neurotoxic activity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

The long-term safety and efficacy of bilateral transplantation of human fetal striatal tissue in patients with mild to moderate Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Impairment of the ubiquitin-proteasome system by protein aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Glutamate receptor abnormalities in the YAC128 transgenic mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Cannabinoid receptor 2 signaling in peripheral immune cells modulates disease onset and severity in mouse models of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mutant huntingtin in glial cells exacerbates neurological symptoms of Huntington disease mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mutations in the TIGR gene in familial primary open-angle glaucoma in Japan

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Imbalance of p75(NTR)/TrkB protein expression in Huntington's disease: implication for neuroprotective therapies.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mutant huntingtin promotes the fibrillogenesis of wild-type huntingtin: a potential mechanism for loss of huntingtin function in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

A randomized feasibility study of a 12-week community-based exercise program for people with Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

The kynurenine pathway modulates neurodegeneration in a Drosophila model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntingtin facilitates dynein/dynactin-mediated vesicle transport.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

NMDA receptor function in mouse models of Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Alpha-synuclein immunoreactivity of huntingtin polyglutamine aggregates in striatum and cortex of Huntington's disease patients and transgenic mouse models

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Amyloid-like features of polyglutamine aggregates and their assembly kinetics

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Expanded-polyglutamine huntingtin protein suppresses the secretion and production of a chemokine (CCL5/RANTES) by astrocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mutant huntingtin is present in neuronal grafts in Huntington disease patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntingtin phosphorylation acts as a molecular switch for anterograde/retrograde transport in neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Polyglutamine expansion of huntingtin impairs its nuclear export

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

α-Synuclein levels modulate Huntington's disease in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Transfer of polyglutamine aggregates in neuronal cells occurs in tunneling nanotubes

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Lesion of striatal neurons with kainic acid provides a model for Huntington's chorea

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Effect of donepezil on motor and cognitive function in Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Aberrant cortical synaptic plasticity and dopaminergic dysfunction in a mouse model of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Identification of a karyopherin β1/β2 proline-tyrosine nuclear localization signal in huntingtin protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Sustained effects of nonallele-specific Huntingtin silencing

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

CYTE-I-HD: phase I dose finding and tolerability study of cysteamine (Cystagon) in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Striatal transplantation in a transgenic mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Glutamate toxicity in the striatum of the R6/2 Huntington's disease transgenic mice is age-dependent and correlates with decreased levels of glutamate transporters

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Altered NMDA receptor trafficking in a yeast artificial chromosome transgenic mouse model of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntington's disease is a four-repeat tauopathy with tau nuclear rods.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Differential clinical and motor control function in a pair of monozygotic twins with Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

A genomic screen in yeast implicates kynurenine 3-monooxygenase as a therapeutic target for Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntingtin is required for mitotic spindle orientation and mammalian neurogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Prions hijack tunnelling nanotubes for intercellular spread

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Six-month partial suppression of Huntingtin is well tolerated in the adult rhesus striatum

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Cell-to-cell transmission of pathogenic proteins in neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Inhibition of mitochondrial fragmentation diminishes Huntington's disease-associated neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

The fragile X syndrome repeats form RNA hairpins that do not activate the interferon-inducible protein kinase, PKR, but are cut by Dicer

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Molecular chaperones in protein folding and proteostasis

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

α-Synuclein modifies huntingtin aggregation in living cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Therapeutic induction of autophagy to modulate neurodegenerative disease progression

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Combating depression in Huntington's disease: effective antidepressive treatment with venlafaxine XR.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Inefficient degradation of truncated polyglutamine proteins by the proteasome

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

The itinerary of autophagosomes: from peripheral formation to kiss-and-run fusion with lysosomes

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Small-molecule TrkB receptor agonists improve motor function and extend survival in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

siRNA screen identifies QPCT as a druggable target for Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

A microRNA-based gene dysregulation pathway in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Expanded polyglutamine-containing N-terminal huntingtin fragments are entirely degraded by mammalian proteasomes

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

A randomized, placebo-controlled trial of latrepirdine in Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntingtin is degraded to small fragments by calpain after ischemic injury

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Influence of lamotrigine on progression of early Huntington disease: a randomized clinical trial

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Bone marrow transplantation confers modest benefits in mouse models of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mutant huntingtin impairs immune cell migration in Huntington disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Formation and toxicity of soluble polyglutamine oligomers in living cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Detecting soluble polyQ oligomers and investigating their impact on living cells using split-GFP

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Cell-to-cell transmission of non-prion protein aggregates.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Genetic manipulations of mutant huntingtin in mice: new insights into Huntington's disease pathogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Expression and characterization of full-length human huntingtin, an elongated HEAT repeat protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

RNA toxicity is a component of ataxin-3 degeneration in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Impaired glutamate uptake in the R6 Huntington's disease transgenic mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mutant Huntingtin alters retrograde transport of TrkB receptors in striatal dendrites.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Disruption of the nuclear membrane by perinuclear inclusions of mutant huntingtin causes cell-cycle re-entry and striatal cell death in mouse and cell models of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

An evolutionary recent neuroepithelial cell adhesion function of huntingtin implicates ADAM10-Ncadherin

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Cdk5 phosphorylation of huntingtin reduces its cleavage by caspases: implications for mutant huntingtin toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Impaired alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor trafficking and function by mutant huntingtin.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Suppressing aberrant GluN3A expression rescues synaptic and behavioral impairments in Huntington's disease models

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

A myriad of miRNA variants in control and Huntington's disease brain regions detected by massively parallel sequencing

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Accumulation of ubiquitin conjugates in a polyglutamine disease model occurs without global ubiquitin/proteasome system impairment

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Preclinical safety of RNAi-mediated HTT suppression in the rhesus macaque as a potential therapy for Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Histone deacetylase inhibitors reduce polyglutamine toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

MeCP2: a novel Huntingtin interactor

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Duplication of biochemical changes of Huntington's chorea by intrastriatal injections of glutamic and kainic acids

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Amyloid-like inclusions in Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Interaction of Huntingtin-associated protein-1 with kinesin light chain: implications in intracellular trafficking in neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntingtin is required for normal excitatory synapse development in cortical and striatal circuits

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mouse models of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Comprehensive behavioral testing in the R6/2 mouse model of Huntington's disease shows no benefit from CoQ10 or minocycline

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Early development of aberrant synaptic plasticity in a mouse model of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Single neuron ubiquitin-proteasome dynamics accompanying inclusion body formation in huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Pathogenic huntingtin inhibits fast axonal transport by activating JNK3 and phosphorylating kinesin

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Formation of morphologically similar globular aggregates from diverse aggregation-prone proteins in mammalian cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Decreased neuronal and increased oligodendroglial densities in Huntington's disease caudate nucleus

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

HEAT repeats associated with condensins, cohesins, and other complexes involved in chromosome-related functions

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Extensive changes in DNA methylation are associated with expression of mutant huntingtin.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Selective roles of normal and mutant huntingtin in neural induction and early neurogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Potential function for the Huntingtin protein as a scaffold for selective autophagy

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Acute polyglutamine expression in inducible mouse model unravels ubiquitin/proteasome system impairment and permanent recovery attributable to aggregate formation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

The bifunctional microRNA miR-9/miR-9* regulates REST and CoREST and is downregulated in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Microglial CB2 cannabinoid receptors are neuroprotective in Huntington's disease excitotoxicity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Cystathionine γ-lyase deficiency mediates neurodegeneration in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Prion-Like Characteristics of Polyglutamine-Containing Proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Transneuronal propagation of mutant huntingtin contributes to non-cell autonomous pathology in neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Proteomic analysis of protein expression and oxidative modification in r6/2 transgenic mice: a model of Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

The current clinical management of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Impaired TrkB receptor signaling underlies corticostriatal dysfunction in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Dynein mutations impair autophagic clearance of aggregate-prone proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Differential loss of striatal projection neurons in Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

PRECREST: a phase II prevention and biomarker trial of creatine in at-risk Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Familial influence on age of onset among siblings with Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Nanotubular highways for intercellular organelle transport

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Early and progressive accumulation of reactive microglia in the Huntington disease brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntington's disease protein contributes to RNA-mediated gene silencing through association with Argonaute and P bodies.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

A role for huntington disease protein in dendritic RNA granules

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Correction of hemophilia B in canine and murine models using recombinant adeno-associated viral vectors

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntingtin phosphorylation sites mapped by mass spectrometry. Modulation of cleavage and toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

A small molecule TrkB ligand reduces motor impairment and neuropathology in R6/2 and BACHD mouse models of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Proteomic and oxidative stress analysis in human brain samples of Huntington disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Silencing mutant huntingtin by adeno-associated virus-mediated RNA interference ameliorates disease manifestations in the YAC128 mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Tryptophan metabolism and oxidative stress in patients with Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Microglial activation in presymptomatic Huntington's disease gene carriers

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

α-Synuclein accumulates in huntingtin inclusions but forms independent filaments and its deficiency attenuates early phenotype in a mouse model of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Comparison of huntingtin proteolytic fragments in human lymphoblast cell lines and human brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Astrocyte Kir4.1 ion channel deficits contribute to neuronal dysfunction in Huntington's disease model mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Delivery of GABAARs to synapses is mediated by HAP1-KIF5 and disrupted by mutant huntingtin.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Differential activities of the ubiquitin-proteasome system in neurons versus glia may account for the preferential accumulation of misfolded proteins in neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Antioxidants can inhibit basal autophagy and enhance neurodegeneration in models of polyglutamine disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Impaired synaptic plasticity in mice carrying the Huntington's disease mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Targeting H3K4 trimethylation in Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Aggregate formation inhibits proteasomal degradation of polyglutamine proteins.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Effects of overexpression of huntingtin proteins on mitochondrial integrity

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Expression of mutant N-terminal huntingtin fragment (htt552-100Q) in astrocytes suppresses the secretion of BDNF.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Abnormal peripheral chemokine profile in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

α-Synuclein impairs macroautophagy: implications for Parkinson's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

The regulation of autophagosome dynamics by huntingtin and HAP1 is disrupted by expression of mutant huntingtin, leading to defective cargo degradation

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntingtin contains a highly conserved nuclear export signal

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Inhibition of mitochondrial protein import by mutant huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Mitochondrial fission in apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Disrupted GABAAR trafficking and synaptic inhibition in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

In vitro analysis of huntingtin-mediated transcriptional repression reveals multiple transcription factor targets

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Deletion of the huntingtin polyglutamine stretch enhances neuronal autophagy and longevity in mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

Kynurenine 3-monooxygenase inhibition in blood ameliorates neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1101%2FCSHPERSPECT.A024240

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

21 January 2018

1 reference

12 December 2020

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

1 reference

12 December 2020

Alpha-synuclein overexpression promotes aggregation of mutant huntingtin

1 reference

12 December 2020

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

1 reference

12 December 2020

Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum.

1 reference

12 December 2020

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

1 reference

12 December 2020

A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease

1 reference

12 December 2020

Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice.

1 reference

12 December 2020

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

1 reference

12 December 2020

Perinuclear localization of huntingtin as a consequence of its binding to microtubules through an interaction with beta-tubulin: relevance to Huntington's disease

1 reference

12 December 2020

Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease.

1 reference

12 December 2020

Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines.

1 reference

12 December 2020

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

1 reference

12 December 2020

Therapeutic effects of cystamine in a murine model of Huntington's disease.

1 reference

12 December 2020

Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease.

1 reference

12 December 2020

Calpain is a major cell death effector in selective striatal degeneration induced in vivo by 3-nitropropionate: implications for Huntington's disease.

1 reference

12 December 2020

Histone Deacetylase Inhibition by Sodium Butyrate Chemotherapy Ameliorates the Neurodegenerative Phenotype in Huntington's Disease Mice

1 reference

12 December 2020

Ribonuclease dicer cleaves triplet repeat hairpins into shorter repeats that silence specific targets

1 reference

12 December 2020

Neuropsychiatry of Huntington's disease

1 reference

12 December 2020

Mutant huntingtin alters Tau phosphorylation and subcellular distribution

1 reference

12 December 2020

Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

10.1101/CSHPERSPECT.A024240

Identifiers

DOI

1 reference

8 September 2017

0 references

1 reference