(Q37217991)

19 August 2017

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title

Neuropathology of Huntington's disease. (English)

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19 August 2017

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Jean Paul G Vonsattel

series ordinal

1

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19 August 2017

Christian Keller

series ordinal

2

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19 August 2017

Maria Del Pilar Amaya

series ordinal

3

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19 August 2017

publication date

1 January 2008

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Handbook of clinical neurology / edited by P.J. Vinken and G.W. Bruyn

19 August 2017

published in

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19 August 2017

volume

89

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19 August 2017

page(s)

599-618

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Selective neurodegeneration in Huntington's disease

19 August 2017

cites work

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The functional anatomy of basal ganglia disorders

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

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Functional architecture of basal ganglia circuits: neural substrates of parallel processing

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7 January 2021

CAG expansion affects the expression of mutant Huntingtin in the Huntington's disease brain

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7 January 2021

Energetics in the pathogenesis of neurodegenerative diseases

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Somatostatin and neuropeptide Y concentrations in pathologically graded cases of Huntington's disease

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7 January 2021

Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid

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7 January 2021

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

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7 January 2021

Dentatorubral and pallidoluysian atrophy (DRPLA). Clinical and neuropathological findings in genetically confirmed North American and European pedigrees

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7 January 2021

Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length

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7 January 2021

Neuronal types in the striatum of man

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7 January 2021

Allocortical involvement in Huntington's disease

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7 January 2021

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

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7 January 2021

The Haw River syndrome: dentatorubropallidoluysian atrophy (DRPLA) in an African-American family

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7 January 2021

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.

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7 January 2021

Huntington's disease in children. Neuropathologic study of four cases

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7 January 2021

The rigid form of Huntington's disease

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7 January 2021

Transcriptional dysregulation in Huntington's disease

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7 January 2021

Diagnosis and evaluation of dementia

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7 January 2021

Degeneration of pyramidal projection neurons in Huntington's disease cortex

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7 January 2021

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

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7 January 2021

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

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7 January 2021

A Golgi study of neuronal types in the neostriatum of monkeys

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Inactivation of the mouse Huntington's disease gene homolog Hdh

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7 January 2021

Ataxia, chorea, seizures, and dementia. Pathologic features of a newly defined familial disorder.

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7 January 2021

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

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7 January 2021

Sparing of acetylcholinesterase-containing striatal neurons in Huntington's disease

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Concentric hyalin intraneuronal inclusions of Lewy type in the brains of elderly persons (50 incidental cases): relationship to parkinsonism.

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7 January 2021

Monozygotic twins discordant for Huntington disease after 7 years

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7 January 2021

Relationship between trinucleotide repeats and neuropathological changes in Huntington's disease

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7 January 2021

The neostriatal mosaic: II. Patch- and matrix-directed mesostriatal dopaminergic and non-dopaminergic systems.

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7 January 2021

The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson's disease

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7 January 2021

The pattern of neurodegeneration in Huntington's disease: a comparative study of cannabinoid, dopamine, adenosine and GABA(A) receptor alterations in the human basal ganglia in Huntington's disease

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7 January 2021

Cytoarchitectonic heterogeneity of the primate neostriatum: subdivision into Island and Matrix cellular compartments.

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7 January 2021

Profound loss of layer II entorhinal cortex neurons occurs in very mild Alzheimer's disease.

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7 January 2021

Functional anatomy of the basal ganglia in X-linked recessive dystonia-parkinsonism

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7 January 2021

Neuronal distribution of intranuclear inclusions in Huntington's disease with adult onset.

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7 January 2021

A Golgi study of the human neostriatum: neurons and afferent fibers

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7 January 2021

Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease

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7 January 2021

Neurotransmitters and neuromodulators in the basal ganglia

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7 January 2021

The basal ganglia and adaptive motor control

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7 January 2021

Mitochondrial defect in Huntington's disease caudate nucleus

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7 January 2021

Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies

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7 January 2021

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

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7 January 2021

Regional specificity of brain atrophy in Huntington's disease.

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7 January 2021

Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease

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7 January 2021

Hirano bodies and related neuronal inclusions

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7 January 2021

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

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7 January 2021

A repeat expansion in the gene encoding junctophilin-3 is associated with Huntington disease-like 2.

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7 January 2021

Neurochemical architecture of the human striatum

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7 January 2021

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

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7 January 2021

HUNTINGTON'S CHOREA IN CHILDHOOD.

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7 January 2021

Reduced Purkinje cell density in Huntington's disease

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7 January 2021

Localization of the gene for a novel autosomal recessive neurodegenerative Huntington-like disorder to 4p15.3.

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7 January 2021

The aging brain. Limitations in our knowledge and future approaches

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Neurons containing NADPH-diaphorase are selectively resistant to quinolinate toxicity

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7 January 2021

The hypothalamic lateral tuberal nucleus and the characteristics of neuronal loss in Huntington's disease

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7 January 2021

A worldwide study of the Huntington's disease mutation. The sensitivity and specificity of measuring CAG repeats

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7 January 2021

Bilateral projections from precentral motor cortex to the putamen and other parts of the basal ganglia. An autoradiographic study in Macaca fascicularis

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7 January 2021

Projections from the primary somatosensory cortex to basal ganglia and thalamus in the monkey

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7 January 2021

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

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7 January 2021

Morphometric studies of the neuropathological changes in choreatic diseases

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7 January 2021

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

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7 January 2021

A huntingtin-associated protein enriched in brain with implications for pathology

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7 January 2021

Distribution of inclusions in neuronal nuclei and dystrophic neurites in Huntington disease brain

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7 January 2021

OBSERVATIONS ON HUNTINGTON'S CHOREA IN CHILDHOOD.

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7 January 2021

Activity of pallidal neurons in the monkey during dyskinesia induced by injection of bicuculline in the external pallidum

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7 January 2021

Cortical peptide changes in Huntington's disease may be independent of striatal degeneration

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7 January 2021

Huntington disease associated with Alzheimer disease

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7 January 2021

Neuropathological background of twenty-seven centenarian brains

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7 January 2021

Huntington disease phenocopy is a familial prion disease

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7 January 2021

Cerebral amyloid deposition and diffuse plaques in "normal" aging: Evidence for presymptomatic and very mild Alzheimer's disease.

1 reference

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7 January 2021

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

1 reference

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7 January 2021

Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p

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7 January 2021

Metabotropic glutamate receptors: a new target for the therapy of neurodegenerative disorders?

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7 January 2021

Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

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7 January 2021

Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold

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7 January 2021

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

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7 January 2021

Huntington's disease CAG trinucleotide repeats in pathologically confirmed post-mortem brains

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7 January 2021

Mutant huntingtin forms in vivo complexes with distinct context-dependent conformations of the polyglutamine segment

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7 January 2021

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

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7 January 2021

Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration

1 reference

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7 January 2021

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

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7 January 2021

Neuronal and glial somal size in the prefrontal cortex: a postmortem morphometric study of schizophrenia and Huntington disease

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7 January 2021

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

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7 January 2021

Differential loss of striatal projection neurons in Huntington disease

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7 January 2021

Dementia of the Alzheimer's type and Huntington's disease

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7 January 2021

Third Dorothy S. Russell memorial lecture. Huntington's disease: some recent neuropathological studies

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7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Preferential loss of preproenkephalin versus preprotachykinin neurons from the striatum of Huntington's disease patients

1 reference

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7 January 2021

Selective loss of striatal preprotachykinin neurons in a phenocopy of Huntington's disease

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7 January 2021

The neuropathology of CAG repeat diseases: review and update of genetic and molecular features

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7 January 2021

Cerebellar atrophy in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Neuronal distribution in the putamen in Huntington's disease

1 reference

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7 January 2021

Striatal volume loss in HD as measured by MRI and the influence of CAG repeat

1 reference

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7 January 2021

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

1 reference

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7 January 2021

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

1 reference

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7 January 2021

Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

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

1 reference

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7 January 2021

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

1 reference

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7 January 2021

Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease

1 reference

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7 January 2021

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

1 reference

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7 January 2021

Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 2-1992. Chorea and progressive dementia in an 88-year-old woman

1 reference

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7 January 2021

Abnormally high neuronal density in the schizophrenic cortex. A morphometric analysis of prefrontal area 9 and occipital area 17.

1 reference

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7 January 2021

'Haw River Syndrome' or Dentato-Rubro-Pallido-Luysian Atrophy?

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Morphometric analysis of the prefrontal cortex in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Huntington's disease: the challenge for cell biologists

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Dentatorubropallidoluysian atrophy: clinicopathological study of dementia and involvement of the nucleus basalis of Meynert in seven autopsy cases

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Molecular genetics of Alzheimer disease: identification of genes and gene mutations

1 reference

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7 January 2021

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

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7 January 2021

Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington's disease

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7 January 2021

Toward understanding the molecular pathology of Huntington's disease.

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7 January 2021

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

1 reference

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7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Widespread occurrence of intranuclear atrophin-1 accumulation in the central nervous system neurons of patients with dentatorubral-pallidoluysian atrophy

1 reference

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7 January 2021

Cortico-striate projections in the rhesus monkey: The organization of certain cortico-caudate connections

1 reference

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7 January 2021

Huntington's disease in Venezuela: neurologic features and functional decline

1 reference

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7 January 2021

The morphology of Marinesco bodies (paranucleolar corpuscles) in the melanin-pigmented nuclei of the brain-stem

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Transglutaminase cross-links in intranuclear inclusions in Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Linkage to the Huntington's disease locus in a family with unusual clinical and pathological features

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Locus coeruleus involvement in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

Neuropathological classification of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0072-9752%2807%2901256-0

7 January 2021

10.1016/S0072-9752(07)01256-0

Identifiers

DOI

1 reference

19 August 2017

1 reference