(Q37798079)

23 August 2017

title

The clinical and genetic features of Huntington disease (English)

1 reference

23 August 2017

0 references

2

Blair R Leavitt

1 reference

23 August 2017

Aaron Sturrock

1

1 reference

23 August 2017

publication date

5 October 2010

1 reference

Journal of Geriatric Psychiatry and Neurology

23 August 2017

published in

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23 August 2017

volume

23

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23 August 2017

issue

4

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23 August 2017

page(s)

243-259

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

23 August 2017

cites work

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DNA sequence variation in a 3.7-kb noncoding sequence 5' of the CYP1A2 gene: implications for human population history and natural selection

21 January 2018

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

21 January 2018

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

21 January 2018

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Huntington's disease in Venezuela: 7 years of follow-up on symptomatic and asymptomatic individuals

21 January 2018

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

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

21 January 2018

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Huntington disease: clinical, genetic, and social aspects

21 January 2018

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Huntington's disease and other choreas.

21 January 2018

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Memory disorders associated with Huntington's disease: verbal recall, verbal recognition and procedural memory

21 January 2018

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An outline for the analysis of dementia: The memory disorder of Huntingtons disease

21 January 2018

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Psychiatric symptoms in Huntington's disease before diagnosis: the predict-HD study

21 January 2018

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Psychopathology in Huntington's disease patients

21 January 2018

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Neuropsychiatric aspects of Huntington's disease.

21 January 2018

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Increased rate of suicide among patients with Huntington's disease

21 January 2018

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Critical periods of suicide risk in Huntington's disease

21 January 2018

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https://api.crossref.org/works/10.1177%2F0891988710383573

Comorbidities of obsessive and compulsive symptoms in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Huntington disease patients and transgenic mice have similar pro-catabolic serum metabolite profiles

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Circulating and cerebrospinal fluid ghrelin and leptin: potential role in altered body weight in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Testicular degeneration in Huntington disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Estimation of fertility and fitness in Huntington disease in New England

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Sleep disturbances and severity of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Disintegration of the sleep-wake cycle and circadian timing in Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Orexin loss in Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Huntington disease in a nonagenarian mistakenly diagnosed as normal pressure hydrocephalus

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Characteristics of individuals with Huntington disease in long-term care

21 January 2018

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https://api.crossref.org/works/10.1177%2F0891988710383573

Juvenile onset Huntington's disease--clinical and research perspectives.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Speech and language delay are early manifestations of juvenile-onset Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Huntington's chorea. Report of 3 cases and review of the literature

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Early onset Huntington disease: a neuronal degeneration syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Highly disabling cerebellar presentation in Huntington disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Huntington disease: finding the gene and after

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Late onset of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

The causes of sporadic and 'senile' chorea

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Hereditary late-onset chorea without significant dementia: genetic evidence for substantial phenotypic variation in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Clinical aspects of CAG repeat diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Evidence for a modifier of onset age in Huntington disease linked to the HD gene in 4p16.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Predictive testing for Huntington disease: interpretation and significance of intermediate alleles.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Mutation in Huntington's chorea

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Regional cortical thinning in preclinical Huntington disease and its relationship to cognition

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Striatal and white matter predictors of estimated diagnosis for Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Relationship between CAG repeat length and brain volume in premanifest and early Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Neuropeptide Y, somatostatin, and reduced nicotinamide adenine dinucleotide phosphate diaphorase in the human striatum: a combined immunocytochemical and enzyme histochemical study.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Differential loss of striatal projection neurons in Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Reduced expression of preproenkephalin in striatal neurons from Huntington's disease patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Selective sparing of a class of striatal neurons in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Survival of basal ganglia neuropeptide Y-somatostatin neurones in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Morphologic and histochemical characteristics of a spared subset of striatal neurons in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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/10.1177%2F0891988710383573

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/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Are there multiple pathways in the pathogenesis of Huntington's disease?

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Transcriptional dysregulation in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Transcriptional dysregulation in striatal projection- and interneurons in a mouse model of Huntington's disease: neuronal selectivity and potential neuroprotective role of HAP1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

The quinolinic acid model of Huntington's disease: locomotor abnormalities

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

A primate model of Huntington's disease: behavioral and anatomical studies of unilateral excitotoxic lesions of the caudate-putamen in the baboon

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases?

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Alternative excitotoxic hypotheses

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Aging, energy, and oxidative stress in neurodegenerative diseases.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Integrating fetal neural transplants into a therapeutic strategy: the example of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Differential susceptibility to excitotoxic stress in YAC128 mouse models of Huntington disease between initiation and progression of disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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/10.1177%2F0891988710383573

NMDA receptor function in mouse models of Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Mitochondria take center stage in aging and neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Mitochondrial sensitivity and altered calcium handling underlie enhanced NMDA-induced apoptosis in YAC128 model of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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/10.1177%2F0891988710383573

Predictive testing for Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Presymptomatic, prenatal, and exclusion testing for Huntington disease using seven closely linked DNA markers

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Predictive testing for Huntington disease in Canada: the experience of those receiving an increased risk

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Predictive testing for Huntington disease in Canada: adverse effects and unexpected results in those receiving a decreased risk

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Perceptions of genetic discrimination among people at risk for Huntington's disease: a cross sectional survey

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Five year study of prenatal testing for Huntington's disease: demand, attitudes, and psychological assessment

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Preimplantation diagnosis for Huntington's disease (HD): clinical application and analysis of the HD expansion in affected embryos.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

New tools for preimplantation genetic diagnosis of Huntington's disease and their clinical applications

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Huntington's disease phenocopy syndromes

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

A South African mixed ancestry family with Huntington disease-like 2: clinical and genetic features

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Huntington's disease like-2 neuropathology

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Huntington disease-like 2: the first patient with apparent European ancestry.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

The Huntington's disease-like syndromes: what to consider in patients with a negative Huntington's disease gene test

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Factors associated with slow progression in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Effect of donepezil on motor and cognitive function in Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Two years' follow-up of rivastigmine treatment in Huntington disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Huntington's disease: progress and potential in the field

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

At risk for Huntington disease: The PHAROS (Prospective Huntington At Risk Observational Study) cohort enrolled

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Basal ganglia volume and proximity to onset in presymptomatic Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Longitudinal change in basal ganglia volume in patients with Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Decreased N-acetyl-aspartate/choline ratio and increased lactate in the frontal lobe of patients with Huntington's disease: a proton magnetic resonance spectroscopy study

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Quantitative proton-decoupled 31P MRS and 1H MRS in the evaluation of Huntington's and Parkinson's diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

1H NMR spectroscopy studies of Huntington's disease: correlations with CAG repeat numbers

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Clinical correlation of striatal 1H MRS changes in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Heterogeneity in 1H-MRS profiles of presymptomatic and early manifest Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Decreased frontal choline and neuropsychological performance in preclinical Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

1H magnetic resonance spectroscopy in preclinical Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Evidence of thalamic dysfunction in Huntington disease by proton magnetic resonance spectroscopy

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Creatine supplementation lowers brain glutamate levels in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

Immune markers for Huntington's disease?

21 January 2018

1 reference

https://api.crossref.org/works/10.1177%2F0891988710383573

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/10.1177%2F0891988710383573

21 January 2018

Identifiers

DOI

10.1177/0891988710383573

1 reference

23 August 2017

1 reference