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Huntington disease

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scholarly article

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Europe PubMed Central

Huntington disease (English)

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Huntington's disease

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based on heuristic

inferred from title

James F. Gusella

3

object named as

James F. Gusella

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Christopher A Ross

8

object named as

Christopher A. Ross

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Blair R Leavitt

6

object named as

Blair R. Leavitt

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Sarah J. Tabrizi

12

object named as

Sarah J. Tabrizi

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Michael R. Hayden

4

object named as

Michael R. Hayden

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1

object named as

Gillian P. Bates

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2

object named as

Ray Dorsey

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10

object named as

Ronald Wetzel

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5

object named as

Chris Kay

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author name string

Martha Nance

7

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Rachael I. Scahill

9

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Edward J. Wild

11

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publication date

23 April 2015

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Nature Reviews Disease Primers

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15005

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https://scigraph.springernature.com/pub.10.1038/nrdp.2015.5

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Multisource ascertainment of Huntington disease in Canada: prevalence and population at risk

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Uptake of Huntington disease predictive testing in a complete population

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Prevalence of adult Huntington's disease in the UK based on diagnoses recorded in general practice records

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Huntington disease: natural history, biomarkers and prospects for therapeutics

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Prevalence estimates of Huntington disease in Caucasian populations are gross underestimates

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

High incidence rate and absent family histories in one quarter of patients newly diagnosed with Huntington disease in British Columbia

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Incidence and mutation rates of Huntington's disease in Spain: experience of 9 years of direct genetic testing

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Late-onset Huntington's disease: diagnostic and prognostic considerations

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

The incidence and prevalence of Huntington's disease: a systematic review and meta-analysis

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

CAG expansion in the Huntington disease gene is associated with a specific and targetable predisposing haplogroup

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

The CAG repeat at the Huntington disease gene in the Portuguese population: insights into its dynamics and to the origin of the mutation

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

CAG size-specific risk estimates for intermediate allele repeat instability in Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

High frequency of intermediate alleles on Huntington disease-associated haplotypes in British Columbia's general population

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

HTT haplotypes contribute to differences in Huntington disease prevalence between Europe and East Asia

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Huntington disease in the South African population occurs on diverse and ethnically distinct genetic haplotypes

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Factors associated with HD CAG repeat instability in Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Genetic criteria for Huntington's disease pathogenesis

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Genetic modifiers of Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Age, CAG repeat length, and clinical progression in Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Association between Age and Striatal Volume Stratified by CAG Repeat Length in Prodromal Huntington Disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Striatal neurons expressing full-length mutant huntingtin exhibit decreased N-cadherin and altered neuritogenesis

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

HEAT repeats in the Huntington's disease protein

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Phosphorylation of threonine 3: implications for Huntingtin aggregation and neurotoxicity

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Polyglutamine expansion of huntingtin impairs its nuclear export

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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/10.1038%2FNRDP.2015.5

21 January 2018

SUMO modification of Huntingtin and Huntington's disease pathology

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Slow amyloid nucleation via α-helix-rich oligomeric intermediates in short polyglutamine-containing huntingtin fragments.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Membrane interactions of the amphipathic amino terminus of huntingtin

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Physical chemistry of polyglutamine: intriguing tales of a monotonous sequence

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Huntingtin interacts with a family of WW domain proteins

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtin

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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/10.1038%2FNRDP.2015.5

21 January 2018

Fragments of HdhQ150 mutant huntingtin form a soluble oligomer pool that declines with aggregate deposition upon aging

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

A two-step path to inclusion formation of huntingtin peptides revealed by number and brightness analysis

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Huntington's disease gene: regional and cellular expression in brain of normal and affected individuals

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Linear and extended: a common polyglutamine conformation recognized by the three antibodies MW1, 1C2 and 3B5H10

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

DNA instability in postmitotic neurons

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Msh2 deficiency prevents in vivo somatic instability of the CAG repeat in Huntington disease transgenic mice

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

MSH3 polymorphisms and protein levels affect CAG repeat instability in Huntington's disease mice

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Mismatch repair genes Mlh1 and Mlh3 modify CAG instability in Huntington's disease mice: genome-wide and candidate approaches

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Msh2 acts in medium-spiny striatal neurons as an enhancer of CAG instability and mutant huntingtin phenotypes in Huntington's disease knock-in mice

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Mismatch repair gene Msh2 modifies the timing of early disease in Hdh(Q111) striatum

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Somatic expansion of the Huntington's disease CAG repeat in the brain is associated with an earlier age of disease onset

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Mutant huntingtin is present in neuronal grafts in Huntington disease patients

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Adapting proteostasis for disease intervention

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Huntington's disease: underlying molecular mechanisms and emerging concepts

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Converging pathways in the occurrence of endoplasmic reticulum (ER) stress in Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Ubiquitin-proteasome system involvement in Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Autophagy in Huntington disease and huntingtin in autophagy

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

What have we learned from gene expression profiles in Huntington's disease?

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

PGC-1α, mitochondrial dysfunction, and Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Dysregulation of synaptic proteins, dendritic spine abnormalities and pathological plasticity of synapses as experience-dependent mediators of cognitive and psychiatric symptoms in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Unified Huntington's disease rating scale: Reliability and consistency

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Diagnostic criteria for Huntington's disease based on natural history

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Differential diagnosis of Huntington's disease: what the clinician should know

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

C9orf72 expansions are the most common genetic cause of Huntington disease phenocopies

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Diagnostic genetic testing for Huntington's disease.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Guidelines for the molecular genetics predictive test in Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Experiences of predictive testing in young people at risk of Huntington's disease, familial cardiomyopathy or hereditary breast and ovarian cancer

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Providing predictive testing for Huntington disease via telehealth: results of a pilot study in British Columbia, Canada

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Reproductive options for prospective parents in families with Huntington's disease: clinical, psychological and ethical reflections.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

The uptake and outcome of prenatal and pre-implantation genetic diagnosis for Huntington's disease in the Netherlands (1998-2008).

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Evidence-based genetic counselling implications for Huntington disease intermediate allele predictive test results

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

CAG repeat number governs the development rate of pathology in Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Randomized controlled clinical trial of "virtual house calls" for Parkinson disease.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Clinical and Biomarker Changes in Premanifest Huntington Disease Show Trial Feasibility: A Decade of the PREDICT-HD Study

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Interrater agreement in the assessment of motor manifestations of Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Grasping premanifest Huntington's disease - shaping new endpoints for new trials

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Neurocognitive signs in prodromal Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Cognitive and functional decline in Huntington's disease: dementia criteria revisited

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Episodic memory in dementia: Characteristics of new learning that differentiate Alzheimer's, Huntington's, and Parkinson's diseases

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

"Frontal" behaviors before the diagnosis of Huntington's disease and their relationship to markers of disease progression: evidence of early lack of awareness

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

The cognitive burden in Huntington's disease: pathology, phenotype, and mechanisms of compensation

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Longitudinal evaluation of neuropsychiatric symptoms in Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Current therapeutic options for Huntington's disease: good clinical practice versus evidence-based approaches?

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Neuropsychiatric symptoms in a European Huntington's disease cohort (REGISTRY).

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Huntington's disease: a caring approach to the end of life

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Chorea associated with Huntington's disease: to treat or not to treat?

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Deep brain stimulation for Huntington's disease: long-term results of a prospective open-label study.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

An evidence-based approach in the treatment of Huntington's disease.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

An International Survey-based Algorithm for the Pharmacologic Treatment of Irritability in Huntington's Disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

An International Survey-based Algorithm for the Pharmacologic Treatment of Obsessive-Compulsive Behaviors in Huntington's Disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Psychiatric and cognitive difficulties as indicators of juvenile huntington disease onset in 29 patients

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Managing juvenile Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Promoting Excellence in End-of-Life Care: lessons learned from a cohort of nursing home residents with advanced Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Characteristics of individuals with Huntington disease in long-term care

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Palliative care for people with late-stage Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Pharmacological management of Huntington's disease: an evidence-based review

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

The future of orphan drug development

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

A randomized, double-blind, placebo-controlled trial of pridopidine in Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Pridopidine for the treatment of motor function in patients with Huntington's disease (MermaiHD): a phase 3, randomised, double-blind, placebo-controlled trial

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Safety, tolerability, and efficacy of PBT2 in Huntington's disease: a phase 2, randomised, double-blind, placebo-controlled trial

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Phosphodiesterase 10A (PDE10A) localization in the R6/2 mouse model of Huntington's disease.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Adverse childhood experiences of persons at risk for Huntington's disease or BRCA1/2 hereditary breast/ovarian cancer

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Development of the HD-Teen Inventory

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Living at risk: concealing risk and preserving hope in Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

The Huntington's Disease health-related Quality of Life questionnaire (HDQoL): a disease-specific measure of health-related quality of life

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Health-related quality of life in Huntington's disease patients: a comparison of proxy assessment and patient self-rating using the disease-specific Huntington's disease health-related quality of life questionnaire (HDQoL)

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Validation of the first quality-of-life measurement for patients with Huntington's disease: the Huntington Quality of Life Instrument

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Health-related quality of life in Huntington's disease: Which factors matter most?

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Identification of health-related quality of life (HRQOL) issues relevant to individuals with Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Predictors of nursing home placement in Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Factors contributing to institutionalization in patients with Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Thinking about the end of life: a common issue for patients with Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Kynurenine 3-monooxygenase inhibition in blood ameliorates neurodegeneration

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

HD-CAB: a cognitive assessment battery for clinical trials in Huntington's disease 1,2,3.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Development of biomarkers for Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Brain-specific proteins decline in the cerebrospinal fluid of humans with Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Immune markers for Huntington's disease?

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

8OHdG is not a biomarker for Huntington disease state or progression

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Electrophysiological measures as potential biomarkers in Huntington's disease: review and future directions

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Stimulus-response compatibility in Huntington's disease: a cognitive-neurophysiological analysis

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Alterations in voluntary movement execution in Huntington's disease are related to the dominant motor system: evidence from event-related potentials

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Somatosensory evoked potentials correlate with genetics in Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Optimization of an HTRF Assay for the Detection of Soluble Mutant Huntingtin in Human Buffy Coats: A Potential Biomarker in Blood for Huntington Disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Riluzole protects Huntington disease patients from brain glucose hypometabolism and grey matter volume loss and increases production of neurotrophins.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Magnetic resonance spectroscopy biomarkers in premanifest and early Huntington disease.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Brain networks in Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Metabolic network as a progression biomarker of premanifest Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Regional cerebral glucose metabolism after pridopidine (ACR16) treatment in patients with Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Microglial activation in presymptomatic Huntington's disease gene carriers

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

White matter connectivity reflects clinical and cognitive status in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Complex brain networks: graph theoretical analysis of structural and functional systems

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Cortical dopamine dysfunction in symptomatic and premanifest Huntington's disease gene carriers

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Clinical trials in Huntington's disease: Interventions in early clinical development and newer methodological approaches

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Assessment of involuntary choreatic movements in Huntington's disease--toward objective and quantitative measures

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Regulatory innovation and drug development for early-stage Alzheimer's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Prospects for neuroprotective therapies in prodromal Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

A polymorphic DNA marker genetically linked to Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Synthetic zinc finger repressors reduce mutant huntingtin expression in the brain of R6/2 mice

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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/10.1038%2FNRDP.2015.5

21 January 2018

Kinase inhibitors modulate huntingtin cell localization and toxicity.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Suppression of protein aggregation by chaperone modification of high molecular weight complexes

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Exogenous delivery of chaperonin subunit fragment ApiCCT1 modulates mutant Huntingtin cellular phenotypes

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

A potent and selective Sirtuin 1 inhibitor alleviates pathology in multiple animal and cell models of Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Genetic knock-down of HDAC3 does not modify disease-related phenotypes in a mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Hdac6 knock-out increases tubulin acetylation but does not modify disease progression in the R6/2 mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Genetic knock-down of HDAC7 does not ameliorate disease pathogenesis in the R6/2 mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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/10.1038%2FNRDP.2015.5

21 January 2018

Inhibition of Phosphodiesterase 10A Increases the Responsiveness of Striatal Projection Neurons to Cortical Stimulation

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Review: Modulation of striatal neuron activity by cyclic nucleotide signaling and phosphodiesterase inhibition

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Inhibition of the striatal specific phosphodiesterase PDE10A ameliorates striatal and cortical pathology in R6/2 mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

AAV-mediated gene delivery of BDNF or GDNF is neuroprotective in a model of Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Gene delivery of AAV2-neurturin for Parkinson's disease: a double-blind, randomised, controlled trial

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

A monoclonal antibody TrkB receptor agonist as a potential therapeutic for Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Kynurenines in the CNS: recent advances and new questions.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Adenosine and glutamate extracellular concentrations and mitogen-activated protein kinases in the striatum of Huntington transgenic mice. Selective antagonism of adenosine A2A receptors reduces transmitter outflow

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Mutant huntingtin alters MAPK signaling pathways in PC12 and striatal cells: ERK1/2 protects against mutant huntingtin-associated toxicity.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

MAP kinase phosphatase 1 (MKP-1/DUSP1) is neuroprotective in Huntington's disease via additive effects of JNK and p38 inhibition

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Targets for future clinical trials in Huntington's disease: what's in the pipeline?

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Evaluation of multi-modal, multi-site neuroimaging measures in Huntington's disease: Baseline results from the PADDINGTON study

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Levels of brain related proteins in cerebrospinal fluid: an aid in the differential diagnosis of parkinsonian disorders

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Plasma 24S-hydroxycholesterol and caudate MRI in pre-manifest and early Huntington's disease.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

21 January 2018

Cellular inclusion bodies of mutant huntingtin exon 1 obscure small fibrillar aggregate species

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

31 March 2020

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

31 March 2020

Prion-like mechanisms in neurodegenerative diseases

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

31 March 2020

A novel mutation of NKX2-1 affecting 2 generations with hypothyroidism and choreoathetosis: part of the spectrum of brain-thyroid-lung syndrome

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

31 March 2020

Low utilization of prenatal and pre-implantation genetic diagnosis in Huntington disease - risk discounting in preventive genetics.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

31 March 2020

CAG-repeat length and the age of onset in Huntington disease (HD): a review and validation study of statistical approaches

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

31 March 2020

An International Survey-based Algorithm for the Pharmacologic Treatment of Chorea in Huntington's Disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

31 March 2020

Emergence of orphan drugs in the United States: a quantitative assessment of the first 25 years

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

31 March 2020

Neuroprotection by GDNF-secreting stem cells in a Huntington's disease model: optical neuroimage tracking of brain-grafted cells.

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

31 March 2020

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

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

31 March 2020

Therapeutic Strategies for Huntington's Disease

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

31 March 2020

Quality of life in Huntington's disease: a comparative study investigating the impact for those with pre-manifest and early manifest disease, and their partners

1 reference

https://api.crossref.org/works/10.1038%2FNRDP.2015.5

7 January 2021

based on heuristic

inferred from DOI database lookup

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10.1038/NRDP.2015.5

0 references

release_qiv6jovkyvebrol5yrt6gmj7ny

1 reference

https://api.fatcat.wiki/v0/release/qiv6jovkyvebrol5yrt6gmj7ny

24 November 2022

based on heuristic

mapped directly with Wikidata item

PubMed publication ID

0 references

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