(Q34308028)

1 August 2017

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title

The energetics of Huntington's disease (English)

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1 August 2017

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2

M Flint Beal

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1 August 2017

Susan E Browne

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1 August 2017

publication date

1 March 2004

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1 August 2017

published in

Neurochemical Research

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1 August 2017

volume

29

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1 August 2017

issue

3

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1 August 2017

page(s)

531-546

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Neurotoxicity at the N-methyl-D-aspartate receptor in energy-compromised neurons. An hypothesis for cell death in aging and disease.

1 August 2017

cites work

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12 December 2020

Nitric oxide, mitochondria, and cell death

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12 December 2020

Huntington's disease progression. PET and clinical observations.

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12 December 2020

Coenzyme Q10 and nicotinamide block striatal lesions produced by the mitochondrial toxin malonate

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12 December 2020

Reduction of glyceraldehyde-3-phosphate dehydrogenase activity in Alzheimer's disease and in Huntington's disease fibroblasts

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12 December 2020

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Lessons from animal models of Huntington's disease

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Biochemical abnormalities and excitotoxicity in Huntington's disease brain.

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12 December 2020

Evidence for the GluR6 gene associated with younger onset age of Huntington's disease

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12 December 2020

Effect of peroxynitrite on the mitochondrial respiratory chain: differential susceptibility of neurones and astrocytes in primary culture

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12 December 2020

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

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12 December 2020

Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice

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12 December 2020

Analysis of neurotransmitter metabolites and adenosine 3',5'-monophosphate in the CSF of patients with extrapyramidal motor disorders

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12 December 2020

Health implications of creatine: can oral creatine supplementation protect against neurological and atherosclerotic disease?

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12 December 2020

Trinucleotide (CAG) repeat length is positively correlated with the degree of DNA fragmentation in Huntington's disease striatum

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12 December 2020

Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse.

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12 December 2020

Involvement of free radicals in excitotoxicity in vivo.

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12 December 2020

Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects

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12 December 2020

Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Brain-derived neurotrophic factor, neurotrophin-3, and neurotrophin-4/5 prevent the death of striatal projection neurons in a rodent model of Huntington's disease

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12 December 2020

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

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12 December 2020

Partial inhibition of brain succinate dehydrogenase by 3-nitropropionic acid is sufficient to initiate striatal degeneration in rat.

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12 December 2020

Neuroprotective strategies for treatment of lesions produced by mitochondrial toxins: implications for neurodegenerative diseases

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12 December 2020

Raised intracellular glucose concentrations reduce aggregation and cell death caused by mutant huntingtin exon 1 by decreasing mTOR phosphorylation and inducing autophagy

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12 December 2020

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

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12 December 2020

Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy

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12 December 2020

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

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12 December 2020

Mice transgenic for the Huntington's disease mutation are resistant to chronic 3-nitropropionic acid-induced striatal toxicity.

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12 December 2020

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

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12 December 2020

Potential for creatine and other therapies targeting cellular energy dysfunction in neurological disorders

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12 December 2020

Local cerebral glucose utilization in symptomatic and presymptomatic Huntington's disease

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12 December 2020

Glutamate becomes neurotoxic via the N-methyl-D-aspartate receptor when intracellular energy levels are reduced

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12 December 2020

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

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12 December 2020

Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease

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12 December 2020

Mitochondrial defect in Huntington's disease caudate nucleus

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12 December 2020

Age-dependent and tissue-specific CAG repeat instability occurs in mouse knock-in for a mutant Huntington's disease gene

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12 December 2020

Toxin-induced mitochondrial dysfunction

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Oxidative stress in Huntington's disease

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12 December 2020

Striatal and nigral neuron subpopulations in rigid Huntington's disease: implications for the functional anatomy of chorea and rigidity-akinesia

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12 December 2020

3‐Nitropropionic Acid Toxicity in the Striatum

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Positron emission tomographic scan investigations of Huntington's disease: cerebral metabolic correlates of cognitive function

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12 December 2020

Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease

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12 December 2020

Brain-derived neurotrophic factor in Huntington disease

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12 December 2020

When more is less: pathogenesis of glutamine repeat neurodegenerative diseases

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12 December 2020

Heterogeneous topographic and cellular distribution of huntingtin expression in the normal human neostriatum.

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12 December 2020

Pathogenesis of inclusion bodies in (CAG)n/Qn-expansion diseases with special reference to the role of tissue transglutaminase and to selective vulnerability

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12 December 2020

Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice

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12 December 2020

Oxidative damage to mitochondrial DNA in Huntington's disease parietal cortex

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12 December 2020

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

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12 December 2020

Creatine-supplemented diet extends Purkinje cell survival in spinocerebellar ataxia type 1 transgenic mice but does not prevent the ataxic phenotype.

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12 December 2020

Excitatory amino acid binding sites in the caudate nucleus and frontal cortex of Huntington's disease

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Energy metabolism defects in Huntington's disease and effects of coenzyme Q10.

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12 December 2020

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

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12 December 2020

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

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12 December 2020

NT-3 and BDNF protect CNS neurons against metabolic/excitotoxic insults

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12 December 2020

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12 December 2020

Mechanisms underlying initiation of excitotoxicity associated with metabolic inhibition

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12 December 2020

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

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12 December 2020

Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain

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12 December 2020

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

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12 December 2020

Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice

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12 December 2020

Striatal glucose consumption in chorea-free subjects at risk of Huntington's disease

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12 December 2020

Mitochondrial encephalomyopathy (MELAS): pathological study and successful therapy with coenzyme Q10 and idebenone

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12 December 2020

Mitochondrial involvement in brain function and dysfunction: relevance to aging, neurodegenerative disorders and longevity

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12 December 2020

Metabolic network abnormalities in early Huntington's disease: an [(18)F]FDG PET study.

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12 December 2020

Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease

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12 December 2020

Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease

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12 December 2020

Transglutaminase-catalyzed inactivation of glyceraldehyde 3-phosphate dehydrogenase and alpha-ketoglutarate dehydrogenase complex by polyglutamine domains of pathological length

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12 December 2020

Nitric oxide, superoxide and peroxynitrite: putative mediators of NMDA-induced cell death in cerebellar granule cells

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12 December 2020

Advances in the understanding of early Huntington's disease using the functional imaging techniques of PET and SPET.

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12 December 2020

Neuroprotective effects of creatine and cyclocreatine in animal models of Huntington's disease.

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12 December 2020

Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease

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12 December 2020

Immunoreactive levels of alpha-ketoglutarate dehydrogenase subunits in Friedreich's ataxia and spinocerebellar ataxia type 1.

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12 December 2020

Overexpression and nuclear accumulation of glyceraldehyde-3-phosphate dehydrogenase in a transgenic mouse model of Huntington’s disease

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12 December 2020

Serial changes of cerebral glucose metabolism and caudate size in persons at risk for Huntington's disease.

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12 December 2020

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

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12 December 2020

Modulation of polyglutamine-induced cell death by genes identified by expression profiling

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12 December 2020

Clinical and biochemical correlations in mitochondrial myopathies treated with coenzyme Q10

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12 December 2020

Striatal glucose metabolism and dopamine D2 receptor binding in asymptomatic gene carriers and patients with Huntington's disease

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12 December 2020

Nuclear localization of N-terminal mutant huntingtin is cell cycle dependent

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12 December 2020

Elevated cerebrospinal fluid lactate/pyruvate ratio in Machado-Joseph disease

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12 December 2020

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

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12 December 2020

Weight loss in early stage of Huntington's disease

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12 December 2020

Evidence for a defect in NADH: ubiquinone oxidoreductase (complex I) in Huntington's disease

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12 December 2020

Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production

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12 December 2020

The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin

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12 December 2020

Alternative excitotoxic hypotheses

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12 December 2020

Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice

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12 December 2020

Brain-derived neurotrophic factor-mediated protection of striatal neurons in an excitotoxic rat model of Huntington's disease, as demonstrated by adenoviral gene transfer

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12 December 2020

Transgenic rat model of Huntington's disease

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12 December 2020

Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length

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12 December 2020

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

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12 December 2020

A detailed examination of substance P in pathologically graded cases of Huntington's disease

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12 December 2020

Isolated cerebral and cerebellar mitochondria produce free radicals when exposed to elevated CA2+ and Na+: implications for neurodegeneration

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12 December 2020

Involvement of oxidative stress in 3-nitropropionic acid neurotoxicity

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12 December 2020

In vitro effects of polyglutamine tracts on Ca2+-dependent depolarization of rat and human mitochondria: relevance to Huntington's disease.

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12 December 2020

Mice transgenic for exon 1 of the Huntington's disease gene display reduced striatal sensitivity to neurotoxicity induced by dopamine and 6-hydroxydopamine

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12 December 2020

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

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12 December 2020

CSF and serum metabolic profile of patients with Huntington's chorea: a study by high resolution proton NMR spectroscopy and HPLC.

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12 December 2020

3-Nitropropionic acid-exogenous animal neurotoxin and possible human striatal toxin.

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12 December 2020

Brain-derived neurotrophic factor mediates an excitoprotective effect of dietary restriction in mice

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12 December 2020

Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease

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12 December 2020

Distribution of phosphate-activated glutaminase, succinic dehydrogenase, pyruvate dehydrogenase and gamma-glutamyl transpeptidase in post-mortem brain from Huntington's disease and agonal cases

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12 December 2020

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

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12 December 2020

Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.

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12 December 2020

Inactivation of the mouse Huntington's disease gene homolog Hdh

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12 December 2020

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

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12 December 2020

Apoptosis in Huntington's disease.

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease

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12 December 2020

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.

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12 December 2020

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

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12 December 2020

Cortical and subcortical glucose consumption measured by PET in patients with Huntington's disease.

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12 December 2020

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

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12 December 2020

Nuclear targeting of mutant Huntingtin increases toxicity

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12 December 2020

Alteration of nuclear glyceraldehyde-3-phosphate dehydrogenase structure in Huntington's disease fibroblasts

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12 December 2020

Transient and progressive electrophysiological alterations in the corticostriatal pathway in a mouse model of Huntington's disease.

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12 December 2020

10.1023/B:NERE.0000014824.04728.DD

Identifiers

DOI

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Dimensions Publication ID

1 August 2017

1008565354

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