(Q33773061)

29 July 2017

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title

Huntington's disease: from molecular pathogenesis to clinical treatment (English)

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29 July 2017

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2

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29 July 2017

1

Christopher A Ross

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29 July 2017

1 January 2011

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29 July 2017

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29 July 2017

10

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29 July 2017

83-98

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29 July 2017

1

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

29 July 2017

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG length.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

The association of CAG repeat length with clinical progression in Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

1 reference

7 January 2021

Huntington disease and the related disorder, dentatorubral-pallidoluysian atrophy (DRPLA).

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Unified Huntington's disease rating scale: Reliability and consistency

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Predictors of neuropathological severity in 100 patients with Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Rate of functional decline in Huntington's disease. Huntington Study Group

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Symptomatic treatment of Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

A systematic review of the treatment studies in Huntington's disease since 1990.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

A Europe-wide assessment of current medication choices in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Tetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trial

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Comprehensive care in Huntington's disease: a physician's perspective

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntingtin-protein interactions and the pathogenesis of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntingtin in health and disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Normal huntingtin function: an alternative approach to Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Palmitoylation of huntingtin by HIP14 is essential for its trafficking and function

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Mechanisms of disease: Histone modifications in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

A role for huntington disease protein in dendritic RNA granules

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Polyglutamine diseases: emerging concepts in pathogenesis and therapy

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntington's disease: the challenge for cell biologists

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Impairment of developmental stem cell-mediated striatal neurogenesis and pluripotency genes in a knock-in model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Disease-specific induced pluripotent stem cells

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Rodent genetic models of Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Longitudinal characterization of brain atrophy of a Huntington's disease mouse model by automated morphological analyses of magnetic resonance images

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Beyond the brain: widespread pathology in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Biomarkers for Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Regional specificity of brain atrophy in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntington disease models and human neuropathology: similarities and differences

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Selective neuronal degeneration in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Interacting proteins as genetic modifiers of Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntingtin-associated protein-1 is a modifier of the age-at-onset of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

PGC-1alpha as modifier of onset age in Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Intercellular miscommunication in polyglutamine pathogenesis

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

The functional anatomy of basal ganglia disorders

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Small molecule drug discovery for Huntington's Disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Discovering novel ligands for macromolecules using X-ray crystallographic screening

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Composite primary neuronal high-content screening assay for Huntington's disease incorporating non-cell-autonomous interactions

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Selective inhibitors of death in mutant huntingtin cells

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

A cell-based screen for drugs to treat Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Tiagabine is neuroprotective in the N171-82Q and R6/2 mouse models of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Drug discovery and natural products: end of an era or an endless frontier?

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntington's disease: silencing a brutal killer

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Wild-type huntingtin plays a role in brain development and neuronal survival

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Misfolded proteins partition between two distinct quality control compartments

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Global changes to the ubiquitin system in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Chemical inducers of autophagy that enhance the clearance of mutant proteins in neurodegenerative diseases.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Overexpression of yeast hsp104 reduces polyglutamine aggregation and prolongs survival of a transgenic mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Small molecule inducers of heat-shock response reduce polyQ-mediated huntingtin aggregation. A possible therapeutic strategy.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Secondary Structure of Huntingtin Amino-Terminal Region

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

A structure-based analysis of huntingtin mutant polyglutamine aggregation and toxicity: evidence for a compact beta-sheet structure.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Protein aggregation and neurodegenerative disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

The chaperonin TRiC blocks a huntingtin sequence element that promotes the conformational switch to aggregation

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Monoclonal antibodies recognize distinct conformational epitopes formed by polyglutamine in a mutant huntingtin fragment

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Opinion: What is the role of protein aggregation in neurodegeneration?

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

A toxic monomeric conformer of the polyglutamine protein

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Tracking mutant huntingtin aggregation kinetics in cells reveals three major populations that include an invariant oligomer pool.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Conformation sensors that distinguish monomeric proteins from oligomers in live cells

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

EGCG redirects amyloidogenic polypeptides into unstructured, off-pathway oligomers

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntingtin phosphorylation on serine 421 is significantly reduced in the striatum and by polyglutamine expansion in vivo

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Phosphorylation of mutant huntingtin at S421 restores anterograde and retrograde transport in neurons.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

The role of post-translational modifications of huntingtin in the pathogenesis of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Phosphorylation of threonine 3: implications for Huntingtin aggregation and neurotoxicity

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Phosphorylation of huntingtin reduces the accumulation of its nuclear fragments

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Acetylation targets mutant huntingtin to autophagosomes for degradation

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

SUMO modification of Huntingtin and Huntington's disease pathology

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntington's disease: seeing the pathogenic process through a genetic lens

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

The crystal structure of caspase-6, a selective effector of axonal degeneration

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Crystal structures of human caspase 6 reveal a new mechanism for intramolecular cleavage self-activation

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

N-terminal proteolysis of full-length mutant huntingtin in an inducible PC12 cell model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Mutant huntingtin N-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Histone deacetylase inhibitors as therapeutics for polyglutamine disorders

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Drug targeting of dysregulated transcription in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Regional and cellular gene expression changes in human Huntington's disease brain

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntingtin modulates transcription, occupies gene promoters in vivo, and binds directly to DNA in a polyglutamine-dependent manner

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Inhibition of specific HDACs and sirtuins suppresses pathogenesis in a Drosophila model of Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

HDAC6 is a microtubule-associated deacetylase

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Brain-derived neurotrophic factor in neurodegenerative diseases

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Brain-derived neurotrophic factor over-expression in the forebrain ameliorates Huntington's disease phenotypes in mice

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Sertraline slows disease progression and increases neurogenesis in N171-82Q mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Up-regulating BDNF with an ampakine rescues synaptic plasticity and memory in Huntington's disease knockin mice

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntingtin as an essential integrator of intracellular vesicular trafficking

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Rab5 modulates aggregation and toxicity of mutant huntingtin through macroautophagy in cell and fly models of Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Neuroprotective effects of inositol 1,4,5-trisphosphate receptor C-terminal fragment in a Huntington's disease mouse model

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

CEP-1347 reduces mutant huntingtin-associated neurotoxicity and restores BDNF levels in R6/2 mice

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Mitochondria and Huntington's disease pathogenesis: insight from genetic and chemical models

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Huntington's disease: from pathology and genetics to potential therapies

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Therapeutic role of sirtuins in neurodegenerative disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Coenzyme Q treatment of neurodegenerative diseases of aging

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Combination therapy with coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson's and Huntington's diseases

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Rosiglitazone treatment prevents mitochondrial dysfunction in mutant huntingtin-expressing cells: possible role of peroxisome proliferator-activated receptor-gamma (PPARgamma) in the pathogenesis of Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Exendin-4 improves glycemic control, ameliorates brain and pancreatic pathologies, and extends survival in a mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Of mice, rats and men: Revisiting the quinolinic acid hypothesis of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Riluzole in Huntington's disease: a 3-year, randomized controlled study

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Microglial activation in presymptomatic Huntington's disease gene carriers

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Microglial activation correlates with severity in Huntington disease: a clinical and PET study.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Effect of fetal neural transplants in patients with Huntington's disease 6 years after surgery: a long-term follow-up study

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Striatal progenitors derived from human ES cells mature into DARPP32 neurons in vitro and in quinolinic acid-lesioned rats.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Emerging drug therapies in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Preparing for preventive clinical trials: the Predict-HD study

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Magnetic resonance imaging of Huntington's disease: preparing for clinical trials

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Functional imaging in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Striatal and white matter predictors of estimated diagnosis for Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Longitudinal change in regional brain volumes in prodromal Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Caudate volume as an outcome measure in clinical trials for Huntington's disease: a pilot study

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Onset and rate of striatal atrophy in preclinical Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Detection of Huntington's disease decades before diagnosis: the Predict-HD study

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Cerebral cortex structure in prodromal Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Regional white matter change in pre-symptomatic Huntington's disease: a diffusion tensor imaging study

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

In vivo evidence for the selective subcortical degeneration in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Contrasting gray and white matter changes in preclinical Huntington disease: an MRI study

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Functional brain changes in presymptomatic Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

Is brain lactate increased in Huntington's disease?

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2810%2970245-3

7 January 2021

10.1016/S1474-4422(10)70245-3

Identifiers

DOI

1 reference

29 July 2017

1 reference

PubMed ID

21163446