(Q34216757)

31 July 2017

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title

The selective vulnerability of nerve cells in Huntington's disease (English)

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31 July 2017

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K A Sieradzan

1

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31 July 2017

D M Mann

series ordinal

2

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31 July 2017

publication date

1 February 2001

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Neuropathology and Applied Neurobiology

31 July 2017

published in

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31 July 2017

volume

27

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31 July 2017

issue

1

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31 July 2017

page(s)

1-21

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Preproenkephalin messenger RNA?containing neurons in striatum of patients with symptomatic and presymptomatic huntington's disease: An in situ hybridization study

31 July 2017

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

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Chorea and myoclonus in the monkey induced by gamma-aminobutyric acid antagonism in the lentiform complex. The site of drug action and a hypothesis for the neural mechanisms of chorea

21 January 2018

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Degeneration of pyramidal projection neurons in Huntington's disease cortex

21 January 2018

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Fourteen and counting: unraveling trinucleotide repeat diseases

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Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?

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

21 January 2018

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

21 January 2018

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Inactivation of the mouse Huntington's disease gene homolog Hdh

21 January 2018

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

21 January 2018

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Compartmentalization of excitatory amino acid receptors in human striatum

21 January 2018

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Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicity

21 January 2018

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Kennedy's disease: caspase cleavage of the androgen receptor is a crucial event in cytotoxicity

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Transcriptional activation modulated by homopolymeric glutamine and proline stretches

21 January 2018

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The neostriatal mosaic: multiple levels of compartmental organization

21 January 2018

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

21 January 2018

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

21 January 2018

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Differential distribution of the normal and mutated forms of huntingtin in the human brain

21 January 2018

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

21 January 2018

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

21 January 2018

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The influence of huntingtin protein size on nuclear localization and cellular toxicity.

21 January 2018

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In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease.

21 January 2018

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

21 January 2018

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

21 January 2018

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Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions.

21 January 2018

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

21 January 2018

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

21 January 2018

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Quantitative localization of NMDAR1 receptor subunit immunoreactivity in inferotemporal and prefrontal association cortices of monkey and human.

21 January 2018

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Abnormal gene product identified in Huntington's disease lymphocytes and brain

21 January 2018

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Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity

21 January 2018

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Membrane properties and synaptic responses of rat striatal neurones in vitro

21 January 2018

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Molecular mechanisms controlling calcium entry through AMPA-type glutamate receptor channels

21 January 2018

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Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei

21 January 2018

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Aberrant protein deposition and neurological disease

21 January 2018

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Trinucleotide repeat length and progression of illness in Huntington's disease

21 January 2018

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Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.

21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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

21 January 2018

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Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity

21 January 2018

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Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy

21 January 2018

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Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats

21 January 2018

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

21 January 2018

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Distinct molecular mechanisms and divergent endocytotic pathways of AMPA receptor internalization

21 January 2018

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21 January 2018

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A cellular model that recapitulates major pathogenic steps of Huntington's disease

21 January 2018

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

21 January 2018

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

21 January 2018

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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

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Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates

21 January 2018

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Duplication of biochemical changes of Huntington's chorea by intrastriatal injections of glutamic and kainic acids

21 January 2018

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Life without huntingtin: normal differentiation into functional neurons

21 January 2018

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A monoclonal antibody to non-phosphorylated neurofilament protein marks the vulnerable cortical neurons in Alzheimer's disease.

21 January 2018

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Clinical and neuropathologic assessment of severity in Huntington's disease

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Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes

21 January 2018

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

21 January 2018

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Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse.

21 January 2018

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Reversing neurodegeneration: a promise unfolds

21 January 2018

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Control of gene expression by proteolysis

21 January 2018

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Huntingtin's WW domain partners in Huntington's disease post-mortem brain fulfill genetic criteria for direct involvement in Huntington's disease pathogenesis

21 January 2018

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Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

21 January 2018

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The GluR2 (GluR-B) hypothesis: Ca(2+)-permeable AMPA receptors in neurological disorders

21 January 2018

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

21 January 2018

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Differential expression of normal and mutant Huntington's disease gene alleles

21 January 2018

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

21 January 2018

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Glutamine repeats and inherited neurodegenerative diseases: molecular aspects

21 January 2018

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

21 January 2018

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The metabotropic glutamate receptors, mGluR2 and mGluR3, show unique postsynaptic, presynaptic and glial localizations

21 January 2018

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

21 January 2018

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Differential loss of striatal projection neurons in Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

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

21 January 2018

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https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

The messenger RNAs for the N-methyl-D-aspartate receptor subunits show region-specific expression of different subunit composition in the human brain

21 January 2018

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

21 January 2018

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When more is less: pathogenesis of glutamine repeat neurodegenerative diseases

21 January 2018

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https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Caspase-8 is required for cell death induced by expanded polyglutamine repeats.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

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/10.1046%2FJ.0305-1846.2001.00299.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Formation of polyglutamine inclusions in non-CNS tissue

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Specific coupling of NMDA receptor activation to nitric oxide neurotoxicity by PSD-95 protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

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/10.1046%2FJ.0305-1846.2001.00299.X

Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Molecular biology of glutamate receptors.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Metabotropic glutamate receptors in brain function and pathology

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

On the pathological progression of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Clinical presentation and patterns of regional cerebral atrophy related to the length of trinucleotide repeat expansion in patients with adult onset Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Mitochondrial impairment reduces the threshold for in vivo NMDA-mediated neuronal death in the striatum.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates

21 January 2018

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Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures

21 January 2018

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Morphometric analysis of the prefrontal cortex in Huntington's disease

21 January 2018

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The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

21 January 2018

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Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone

21 January 2018

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Neurochemical substrates of rigidity and chorea in Huntington's disease

21 January 2018

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Biochemical abnormalities and excitotoxicity in Huntington's disease brain.

21 January 2018

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Ataxin-3 is transported into the nucleus and associates with the nuclear matrix

21 January 2018

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Splice variants of glutamate receptor subunits 2 and 3 in striatal projection neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

GABAergic neocortical neurons are resistant to NMDA receptor-mediated injury.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Huntington's disease gene product, huntingtin, associates with microtubules in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Selective loss of [3H]kainic acid and [3H]AMPA binding in layer VI of frontal cortex in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

HIP-I: a huntingtin interacting protein isolated by the yeast two-hybrid system

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Ca2+-Zn2+ permeable AMPA or kainate receptors: possible key factors in selective neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Homozygotes for Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2001.00299.X

10.1046/J.0305-1846.2001.00299.X

21 January 2018

Identifiers

DOI

1 reference

31 July 2017

1 reference