Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. (Q40704833)

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13 November 2019

title

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease (English)

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13 November 2019

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Blair R Leavitt

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Steven M Hersch

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Michael R Hayden

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Lisa M Ellerby

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Christelle Sophie Roy

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Sophie Roy

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Cheryl L Wellington

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Simon Warby

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Rona K Graham

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Jeremy M Van Raamsdonk

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Jeremy van Raamsdonk

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Claire-Anne Gutekunst

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Claire-Anne Gutekunst

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13 November 2019

author name string

Danny Rogers

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Odell Loubser

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Yu-Zhou Yang

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Juliette Gafni

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Dale Bredesen

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Donald W Nicholson

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language of work or name

English

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publication date

1 September 2002

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13 November 2019

published in

Journal of Neuroscience

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13 November 2019

volume

22

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13 November 2019

issue

18

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13 November 2019

page(s)

7862-7872

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

13 November 2019

cites work

1 reference

12 December 2020

Purification and catalytic properties of human caspase family members

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12 December 2020

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

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12 December 2020

Defects in regulation of apoptosis in caspase-2-deficient mice

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12 December 2020

Accurate determination of the number of CAG repeats in the Huntington disease gene using a sequence-specific internal DNA standard

1 reference

12 December 2020

Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum.

1 reference

12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.

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12 December 2020

Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice

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12 December 2020

Caspase activity plays an essential role in long-term memory.

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12 December 2020

Transgenic mice in the study of polyglutamine repeat expansion diseases

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12 December 2020

Involvement of caspases in proteolytic cleavage of Alzheimer's amyloid-beta precursor protein and amyloidogenic A beta peptide formation

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12 December 2020

Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

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12 December 2020

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

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12 December 2020

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

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12 December 2020

The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease

1 reference

12 December 2020

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

1 reference

12 December 2020

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

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12 December 2020

Caspase-2 mediates neuronal cell death induced by beta-amyloid

1 reference

12 December 2020

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

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12 December 2020

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

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12 December 2020

Caspase and calpain substrates: roles in synaptic plasticity and cell death

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12 December 2020

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

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12 December 2020

Mutant huntingtin enhances excitotoxic cell death.

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

The likelihood of being affected with Huntington disease by a particular age, for a specific CAG size

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12 December 2020

Decreased apoptosis in the brain and premature lethality in CPP32-deficient mice

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12 December 2020

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

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12 December 2020

Subtype-specific enhancement of NMDA receptor currents by mutant huntingtin

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12 December 2020

Mutant protein in Huntington disease is resistant to proteolysis in affected brain

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture.

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12 December 2020

Neuropathological classification of Huntington's disease

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12 December 2020

Seizure-induced neuronal injury: vulnerability to febrile seizures in an immature rat model

1 reference

12 December 2020

Prostaglandin E1 prevents apoptotic cell death in superficial dorsal horn of rat spinal cord

1 reference

12 December 2020

Selective death of hippocampal CA3 pyramidal cells with mossy fiber afferents after CRH-induced status epilepticus in infant rats

1 reference

12 December 2020

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

1 reference

12 December 2020

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

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12 December 2020

Changes in caspase expression in Alzheimer's disease: comparison with development and aging

1 reference

12 December 2020

An immediate morphopathologic response of neurons to electroshock; a reliable model for producing "dark" neurons in experimental neuropathology.

1 reference

12 December 2020

Maintenance of caspase-3 proenzyme dormancy by an intrinsic "safety catch" regulatory tripeptide

1 reference

12 December 2020

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

1 reference

12 December 2020

HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain

1 reference

12 December 2020

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

1 reference

12 December 2020

10.1523/JNEUROSCI.22-18-07862.2002

Identifiers

DOI

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13 November 2019

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13 November 2019

PubMed publication ID

12223539

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