Huntingtin proteolysis in Huntington disease (Q57839949)

6

Michael R. Hayden

0 references

Lisa M Ellerby

2

Lisa M. Ellerby

0 references

Christelle Sophie Roy

4

Sophie Roy

0 references

Cheryl L Wellington

1

Cheryl L. Wellington

0 references

author name string

Donald W. Nicholson

Clinical neuroscience research

5

0 references

publication date

September 2003

0 references

published in

0 references

volume

3

0 references

issue

3

0 references

page(s)

129-139

0 references

cites work

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Calpain activation in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Impairment of the ubiquitin-proteasome system by protein aggregation

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Subtype-specific enhancement of NMDA receptor currents by mutant huntingtin

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Huntingtin interacting protein 1 induces apoptosis via a novel caspase-dependent death effector domain

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Nuclear targeting of mutant Huntingtin increases toxicity

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

The caspase family of cysteine proteases

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Life and death decisions: regulation of apoptosis by proteolysis of signaling molecules

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Apaf1 is required for mitochondrial pathways of apoptosis and brain development

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Reduced apoptosis and cytochrome c-mediated caspase activation in mice lacking caspase 9

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Apoptotic and anti-apoptotic synaptic signaling mechanisms

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Expression of active caspase-3 in mitotic and postmitotic cells of the rat forebrain

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

HIP1 functions in clathrin-mediated endocytosis through binding to clathrin and adaptor protein 2

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Differential sparing of somatostatin-neuropeptide Y and cholinergic neurons following striatal excitotoxin lesions

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Degeneration of pyramidal projection neurons in Huntington's disease cortex

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Impaired glutamate uptake in the R6 Huntington's disease transgenic mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Impaired synaptic plasticity in mice carrying the Huntington's disease mutation

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Mutant huntingtin enhances excitotoxic cell death.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Calpastatin is up-regulated in response to hypoxia and is a suicide substrate to calpain after neonatal cerebral hypoxia-ischemia.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Synergistic activation of caspase-3 by m-calpain after neonatal hypoxia-ischemia: a mechanism of "pathological apoptosis"?

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

How calpain is activated by calcium

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Calpain-dependent cleavage of cain/cabin1 activates calcineurin to mediate calcium-triggered cell death

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Cross-talk between two cysteine protease families. Activation of caspase-12 by calpain in apoptosis

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Calpain and caspase: can you tell the difference?

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Differential sensitivity of medium- and large-sized striatal neurons to NMDA but not kainate receptor activation in the rat.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Transient and progressive electrophysiological alterations in the corticostriatal pathway in a mouse model of Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

In vitro effects of polyglutamine tracts on Ca2+-dependent depolarization of rat and human mitochondria: relevance to Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Regional calpain and caspase-3 proteolysis of alpha-spectrin after traumatic brain injury

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Widespread activation of calcium-activated neutral proteinase (calpain) in the brain in Alzheimer disease: a potential molecular basis for neuronal degeneration

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Polyglutamine repeat length-dependent proteolysis of huntingtin

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Comparison of cathepsin protease activities in brain tissue from normal cases and cases with Alzheimer's disease, Lewy body dementia, Parkinson's disease and Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Aggresomes: a cellular response to misfolded proteins

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Aggregate formation inhibits proteasomal degradation of polyglutamine proteins.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

HAP1-huntingtin interactions do not contribute to the molecular pathology in Huntington's disease transgenic mice

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Huntingtin interacts with cystathionine beta-synthase

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

An actin-binding protein of the Sla2/Huntingtin interacting protein 1 family is a novel component of clathrin-coated pits and vesicles

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Huntingtin interacts with a family of WW domain proteins

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Arfaptin 2 regulates the aggregation of mutant huntingtin protein

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Huntingtin interacting protein 1 Is a clathrin coat binding protein required for differentiation of late spermatogenic progenitors

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Identification of the full-length huntingtin- interacting protein p231HBP/HYPB as a DNA-binding factor

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Localization of huntingtin-interacting protein-2 (Hip-2) mRNA in the developing mouse brain

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Bassoon, a novel zinc-finger CAG/glutamine-repeat protein selectively localized at the active zone of presynaptic nerve terminals

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

The huntingtin interacting protein HIP1 is a clathrin and alpha-adaptin-binding protein involved in receptor-mediated endocytosis

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionation

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Nuclear relocation of normal huntingtin

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: implications for Huntington's disease therapy

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

Inhibition of polyglutamine aggregation in R6/2 HD brain slices-complex dose-response profiles

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0

7 January 2021