Huntington's disease and dentatorubral-pallidoluysian atrophy: proteins, pathogenesis and pathology (Q41527116)

2 October 2017

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title

Huntington's disease and dentatorubral-pallidoluysian atrophy: proteins, pathogenesis and pathology (English)

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dentatorubral-pallidoluysian atrophy

2 October 2017

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Ross CA

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2 October 2017

Becher MW

2

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2 October 2017

Colomer V

3

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2 October 2017

Engelender S

4

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2 October 2017

Wood JD

5

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2 October 2017

Sharp AH

6

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2 October 2017

1 July 1997

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2 October 2017

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2 October 2017

7

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2 October 2017

3

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2 October 2017

1003-1016

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Androgen-specific gene activation via a consensus glucocorticoid response element is determined by interaction with nonreceptor factors

2 October 2017

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Novel proteins that interact with the COOH-terminal cytosolic routing determinants of an integral membrane peptide-processing enzyme

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Alternative excitotoxic hypotheses

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Selective neurodegeneration in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Structure and expression of the Huntington's disease gene: evidence against simple inactivation due to an expanded CAG repeat

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

HEAT repeats in the Huntington's disease protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Expansion of polyglutamine repeat in huntingtin leads to abnormal protein interactions involving calmodulin

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Age-dependent striatal excitotoxic lesions produced by the endogenous mitochondrial inhibitor malonate

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

The WW domain: a signalling site in dystrophin?

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Nitric oxide, a novel neuronal messenger

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Age-dependent vulnerability of the striatum to the mitochondrial toxin 3-nitropropionic acid.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Huntington's chorea in children. Report of four cases

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Glutamate neurotoxicity and diseases of the nervous system

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Mitochondrial DNA deletions in human brain: regional variability and increase with advanced age

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Oxidative stress, glutamate, and neurodegenerative disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

The multidomain protein Trio binds the LAR transmembrane tyrosine phosphatase, contains a protein kinase domain, and has separate rac-specific and rho-specific guanine nucleotide exchange factor domains

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Transcriptional activation modulated by homopolymeric glutamine and proline stretches

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Dentatorubropallidoluysian degeneration: clinical, neuro-ophthalmologic, biochemical, and pathologic studies on autosomal dominant form.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Mitochondrial defect in Huntington's disease caudate nucleus

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

NPl1, an essential yeast gene involved in induced degradation of Gap1 and Fur4 permeases, encodes the Rsp5 ubiquitin-protein ligase.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Quinolinic acid-induced increases in calbindin D28k immunoreactivity in rat striatal neurons in vivo and in vitro mimic the pattern seen in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

A family of proteins structurally and functionally related to the E6-AP ubiquitin-protein ligase

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Mitogenic signaling mediated by oxidants in Ras-transformed fibroblasts

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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/10.1111%2FJ.1750-3639.1997.TB00898.X

Non-invasive neurochemical analysis of focal excitotoxic lesions in models of neurodegenerative illness using spectroscopic imaging

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

HUNTINGTON'S CHOREA IN CHILDHOOD.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Evidence from antibody studies that the CAG repeat in the Huntington disease gene is expressed in the protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

cDNA cloning and characterization of an atrophin-1 (DRPLA disease gene)-related protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

DNA analysis in hereditary dentatorubral-pallidoluysian atrophy: correlation between CAG repeat length and phenotypic variation and the molecular basis of anticipation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Spinocerebellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Regulation of receptor-mediated endocytosis by Rho and Rac.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Trinucleotide repeat expansion in neurological disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Huntington's disease gene (IT15) is widely expressed in human and rat tissues

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Huntingtin-associated protein (HAP1): discrete neuronal localizations in the brain resemble those of neuronal nitric oxide synthase

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Cloning and expression of the rat atrophin-I (DRPLA disease gene) homologue

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

The Rho's progress: a potential role during neuritogenesis for the Rho family of GTPases

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

DRPLA gene (atrophin-1) sequence and mRNA expression in human brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Kainate-induced degeneration of neostriatal neurons: dependency upon corticostriatal tract

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Structure and expression of the gene responsible for the triplet repeat disorder, dentatorubral and pallidoluysian atrophy (DRPLA)

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Familial myoclonus epilepsy and choreoathetosis: hereditary dentatorubral-pallidoluysian atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Sequence and domain structure of talin

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Cerebellar atrophy in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Huntingtin: new marker along the road to death?

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Neurological sequelae of cyanide intoxication--the patterns of clinical, magnetic resonance imaging, and positron emission tomography findings.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

From genes to mechanisms to therapies: lessons to be learned from neurological disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

When more is less: pathogenesis of glutamine repeat neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Expression of the Huntington's disease (IT15) protein product in HD patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Expression of the Huntington disease gene in rodents: cloning the rat homologue and evidence for downregulation in non-neuronal tissues during development

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Neurobiology of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Minireview. Emerging new functions of the glycolytic protein, glyceraldehyde-3-phosphate dehydrogenase, in mammalian cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Unusual form of cerebellar ataxia; combined dentato-rubral and pallido-Luysian degeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Evidence for neuronal degeneration and dendritic plasticity in cortical pyramidal neurons of Huntington's disease: a quantitative Golgi study.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Correlation between the onset age of Huntington's disease and length of the trinucleotide repeat in IT-15.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Incorporation of glutamine repeats makes protein oligomerize: implications for neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Proton magnetic resonance spectroscopy in Huntington's disease: evidence in favour of the glutamate excitotoxic theory

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.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/10.1111%2FJ.1750-3639.1997.TB00898.X

Cyanide-induced parkinsonism: a clinicopathologic report.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Dentatorubral-pallidoluysian atrophy (DRPLA): clinical, genetic, and neuroradiologic studies in a family.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Autosomal-dominant dentatorubropallidoluysian atrophy in the United Kingdom

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Trinucleotide repeat expansions in neurological disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Partial characterisation of murine huntingtin and apparent variations in the subcellular localisation of huntingtin in human, mouse and rat brain.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Abnormal gene product identified in hereditary dentatorubral-pallidoluysian atrophy (DRPLA) brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Excitatory amino acid receptors in the brain: membrane binding and receptor autoradiographic approaches

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

Kalirin, a cytosolic protein with spectrin-like and GDP/GTP exchange factor-like domains that interacts with peptidylglycine alpha-amidating monooxygenase, an integral membrane peptide-processing enzyme.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1997.TB00898.X

10.1111/J.1750-3639.1997.TB00898.X

21 January 2018

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2 October 2017

1 reference