The paradigm of Huntington's disease: therapeutic opportunities in neurodegeneration (Q36045254)

11 August 2017

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title

The paradigm of Huntington's disease: therapeutic opportunities in neurodegeneration (English)

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11 August 2017

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Julie Leegwater-Kim

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PMC publication ID

534918

11 August 2017

Jang-Ho J Cha

series ordinal

2

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11 August 2017

1 January 2004

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11 August 2017

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11 August 2017

1

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11 August 2017

1

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11 August 2017

128-138

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Increased cell proliferation and neurogenesis in the adult human Huntington's disease brain

11 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

The production and use of cells as therapeutic agents in neurodegenerative diseases

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Apoptosis and caspases in neurodegenerative diseases

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Unilateral transplantation of human primary fetal tissue in four patients with Huntington's disease: NEST-UK safety report ISRCTN no 36485475.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Calpain activation in Huntington's disease.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Toxic proteins in neurodegenerative disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Neuroprotection by neurotrophins and GDNF family members in the excitotoxic model of Huntington's disease.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Interaction of Huntington disease protein with transcriptional activator Sp1

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Histone deacetylase inhibitors reduce polyglutamine toxicity

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Altered transcription in yeast expressing expanded polyglutamine

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Riluzole stimulates nerve growth factor, brain-derived neurotrophic factor and glial cell line-derived neurotrophic factor synthesis in cultured mouse astrocytes

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Mutant huntingtin enhances excitotoxic cell death.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Huntington disease: new insights on the role of huntingtin cleavage

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Transplanted fetal striatum in Huntington's disease: phenotypic development and lack of pathology

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

CREB-binding protein sequestration by expanded polyglutamine

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Transcriptional dysregulation in Huntington's disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Neuronal cell death in Huntington's disease: a potential role for dopamine

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Huntingtin is required for normal hematopoiesis

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Huntington's disease progression. PET and clinical observations.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

A tetracycline derivative, minocycline, reduces inflammation and protects against focal cerebral ischemia with a wide therapeutic window

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Polyglutamine pathogenesis.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

The role of excitotoxicity in neurodegenerative disease: implications for therapy

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Subtype-specific enhancement of NMDA receptor currents by mutant huntingtin

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Mitochondria in neurodegeneration: acute ischemia and chronic neurodegenerative diseases

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Tetracyclines inhibit microglial activation and are neuroprotective in global brain ischemia

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

2 references

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

2 October 2017

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

A controlled trial of idebenone in Huntington's disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Trinucleotide repeats in neurogenetic disorders

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Implants of encapsulated human CNTF-producing fibroblasts prevent behavioral deficits and striatal degeneration in a rodent model of Huntington's disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Trinucleotide repeats in neurologic diseases: an hypothesis concerning the pathogenesis of Huntington's disease, Kennedy's disease, and spinocerebellar ataxia type I.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

A polymorphic DNA marker genetically linked to Huntington's disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Construction of a genetic linkage map in man using restriction fragment length polymorphisms

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Glutamate neurotoxicity and diseases of the nervous system

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

NMDA receptor losses in putamen from patients with Huntington's disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

The quinolinic acid hypothesis in Huntington's chorea

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Molecular Approaches to Hereditary Diseases of the Nervous System: Huntington's Disease as a Paradigm

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Excitotoxic injury of the neostriatum: a model for Huntington's disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Mechanisms of excitotoxicity in neurologic diseases

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Alternative excitotoxic hypotheses

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Glutamate neurotoxicity and Huntington's chorea

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Brain neurotransmitter deficits in mice transgenic for the Huntington's disease mutation

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Riluzole therapy in Huntington's disease (HD).

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Expression of NMDA glutamate receptor subunit mRNAs in neurochemically identified projection and interneurons in the striatum of the rat.

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Mitochondrial dysfunction in neurodegeneration

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

The CAG/polyglutamine tract diseases: gene products and molecular pathogenesis

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Striatal interneurons in Huntington's disease: selective increase in the density of calretinin-immunoreactive medium-sized neurons

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Chronic 3-nitropropionic acid treatment in baboons replicates the cognitive and motor deficits of Huntington's disease

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Neuropathological classification of Huntington's disease

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Degeneration of pyramidal projection neurons in Huntington's disease cortex

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Chronic quinolinic acid lesions in rats closely resemble Huntington's disease

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Genetic neurodegenerative diseases: the human illness and transgenic models

27 July 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Experimental therapeutics of neurodegenerative disorders: unmet needs

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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/10.1602%2FNEURORX.1.1.128

Dopamine D1 and D2 receptor gene expression in the striatum in Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

[11C]raclopride-PET studies of the Huntington's disease rate of progression: relevance of the trinucleotide repeat length

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Cannabinoid receptor messenger RNA levels decrease in a subset of neurons of the lateral striatum, cortex and hippocampus of transgenic Huntington's disease mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Molecular analysis of new mutations for Huntington's disease: intermediate alleles and sex of origin effects.

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Calcium-dependent cleavage of endogenous wild-type huntingtin in primary cortical neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Nuclear targeting of mutant Huntingtin increases toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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/10.1602%2FNEURORX.1.1.128

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/10.1602%2FNEURORX.1.1.128

Impaired glutamate transport and glutamate-glutamine cycling: downstream effects of the Huntington mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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/10.1602%2FNEURORX.1.1.128

Cytochrome C and caspase-9 expression in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Mitochondrial function in Huntington's disease: clues for pathogenesis and prospects for treatment

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Mitochondrial dysfunction in neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Mitochondrial defect in Huntington's disease caudate nucleus

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Malonate produces striatal lesions by indirect NMDA receptor activation

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

N-acetylaspartylglutamate (NAAG) protects against rat striatal quinolinic acid lesions in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Riluzole in Huntington's disease (HD): an open label study with one year follow up.

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Neuroprotective effects of the alpha2-adrenoceptor antagonists, (+)-efaroxan and (+/-)-idazoxan, against quinolinic acid-induced lesions of the rat striatum

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

The role of dopamine in motor symptoms in the R6/2 transgenic mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Trial of d-alpha-tocopherol in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Bilateral human fetal striatal transplantation in Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

We need something better, and we need it now: fetal striatal transplantation in Huntington's disease?

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Administration of recombinant human Activin-A has powerful neurotrophic effects on select striatal phenotypes in the quinolinic acid lesion model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Anterior cingulate cortical transplantation in transgenic Huntington's disease mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Therapeutic effects of cystamine in a murine model of Huntington's disease.

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Inhibition of mitochondrial complex II induces a long-term potentiation of NMDA-mediated synaptic excitation in the striatum requiring endogenous dopamine.

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Porcine xenografts in Parkinson's disease and Huntington's disease patients: preliminary results

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Effects of biologically delivered NGF, BDNF and bFGF on striatal excitotoxic lesions

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models.

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

In situ evidence for DNA fragmentation in Huntingtonʼs disease striatum and Alzheimerʼs disease temporal lobes

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

NMDA receptor subunit mRNA expression by projection neurons and interneurons in rat striatum.

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Decreased receptor-binding sites for kainic acid in brains of patients with Huntington's disease

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

Application of recombinant DNA techniques to neurogenetic disorders

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=534918

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

23 September 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Characterization of the excitotoxic potential of the reversible succinate dehydrogenase inhibitor malonate

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Metabotropic receptors in excitotoxicity: (S)-4-carboxy-3-hydroxyphenylglycine ((S)-4C3HPG) protects against rat striatal quinolinic acid lesions

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Effect of riluzole on quinolinate-induced neuronal damage in rats: comparison with blockers of glutamatergic neurotransmission

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

Safety and tolerability of the free-radical scavenger OPC-14117 in Huntington's disease. The Huntington Study Group

21 January 2018

1 reference

https://api.crossref.org/works/10.1602%2FNEURORX.1.1.128

21 January 2018

Identifiers

DOI

10.1602/NEURORX.1.1.128

1 reference

11 August 2017

1 reference

PMC publication ID

534918

11 August 2017

PubMed publication ID

15717013

1 reference

PMC publication ID

534918