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Huntingtin proteolysis in Huntington disease
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instance of
scholarly article
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title
Huntingtin proteolysis in Huntington disease
(English)
0 references
main subject
Huntington's disease
1 reference
based on heuristic
inferred from title
proteolysis
1 reference
based on heuristic
inferred from title
author
Blair R Leavitt
series ordinal
3
object named as
Blair R. Leavitt
0 references
Michael R. Hayden
series ordinal
6
object named as
Michael R. Hayden
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Lisa M Ellerby
series ordinal
2
object named as
Lisa M. Ellerby
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Christelle Sophie Roy
series ordinal
4
object named as
Sophie Roy
0 references
Cheryl L Wellington
series ordinal
1
object named as
Cheryl L. Wellington
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author name string
Donald W. Nicholson
series ordinal
5
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publication date
September 2003
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published in
Clinical neuroscience research
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volume
3
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issue
3
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page(s)
129-139
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cites work
Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Calpain activation in Huntington's disease.
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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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
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.
1 reference
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7 January 2021
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Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
1 reference
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7 January 2021
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Impairment of the ubiquitin-proteasome system by protein aggregation
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7 January 2021
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Subtype-specific enhancement of NMDA receptor currents by mutant huntingtin
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Mutant protein in Huntington disease is resistant to proteolysis in affected brain
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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The influence of huntingtin protein size on nuclear localization and cellular toxicity.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Huntingtin interacting protein 1 induces apoptosis via a novel caspase-dependent death effector domain
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
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
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition.
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis
1 reference
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Crossref
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
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
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Crossref
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Nuclear targeting of mutant Huntingtin increases toxicity
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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The caspase family of cysteine proteases
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Life and death decisions: regulation of apoptosis by proteolysis of signaling molecules
1 reference
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Crossref
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Decreased apoptosis in the brain and premature lethality in CPP32-deficient mice
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Apaf1 is required for mitochondrial pathways of apoptosis and brain development
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Reduced apoptosis and cytochrome c-mediated caspase activation in mice lacking caspase 9
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Apoptotic and anti-apoptotic synaptic signaling mechanisms
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7 January 2021
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Expression of active caspase-3 in mitotic and postmitotic cells of the rat forebrain
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Maintenance of caspase-3 proenzyme dormancy by an intrinsic "safety catch" regulatory tripeptide
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7 January 2021
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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
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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HIP1 functions in clathrin-mediated endocytosis through binding to clathrin and adaptor protein 2
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Caspase-8 is required for cell death induced by expanded polyglutamine repeats.
1 reference
stated in
Crossref
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.
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7 January 2021
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Differential sparing of somatostatin-neuropeptide Y and cholinergic neurons following striatal excitotoxin lesions
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Degeneration of pyramidal projection neurons in Huntington's disease cortex
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Impaired glutamate uptake in the R6 Huntington's disease transgenic mice.
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Impaired synaptic plasticity in mice carrying the Huntington's disease mutation
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Mutant huntingtin enhances excitotoxic cell death.
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Calpastatin is up-regulated in response to hypoxia and is a suicide substrate to calpain after neonatal cerebral hypoxia-ischemia.
1 reference
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Crossref
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Synergistic activation of caspase-3 by m-calpain after neonatal hypoxia-ischemia: a mechanism of "pathological apoptosis"?
1 reference
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7 January 2021
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inferred from DOI database lookup
How calpain is activated by calcium
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Calpain-dependent cleavage of cain/cabin1 activates calcineurin to mediate calcium-triggered cell death
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Cross-talk between two cysteine protease families. Activation of caspase-12 by calpain in apoptosis
1 reference
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7 January 2021
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Calpain and caspase: can you tell the difference?
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7 January 2021
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Differential sensitivity of medium- and large-sized striatal neurons to NMDA but not kainate receptor activation in the rat.
1 reference
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7 January 2021
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Transient and progressive electrophysiological alterations in the corticostriatal pathway in a mouse model of Huntington's disease.
1 reference
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7 January 2021
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Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
In vitro effects of polyglutamine tracts on Ca2+-dependent depolarization of rat and human mitochondria: relevance to Huntington's disease.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Regional calpain and caspase-3 proteolysis of alpha-spectrin after traumatic brain injury
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
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Widespread activation of calcium-activated neutral proteinase (calpain) in the brain in Alzheimer disease: a potential molecular basis for neuronal degeneration
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Polyglutamine repeat length-dependent proteolysis of huntingtin
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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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
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Aggresomes: a cellular response to misfolded proteins
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
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Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Aggregate formation inhibits proteasomal degradation of polyglutamine proteins.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Wild-type huntingtin protects from apoptosis upstream of caspase-3.
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Expansion of polyglutamine repeat in huntingtin leads to abnormal protein interactions involving calmodulin
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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inferred from DOI database lookup
HAP1-huntingtin interactions do not contribute to the molecular pathology in Huntington's disease transgenic mice
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Huntingtin interacts with cystathionine beta-synthase
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
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Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.
1 reference
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7 January 2021
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An actin-binding protein of the Sla2/Huntingtin interacting protein 1 family is a novel component of clathrin-coated pits and vesicles
1 reference
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
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Huntingtin interacts with a family of WW domain proteins
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes
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7 January 2021
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PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains
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https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
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Arfaptin 2 regulates the aggregation of mutant huntingtin protein
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reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
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Huntingtin interacting protein 1 Is a clathrin coat binding protein required for differentiation of late spermatogenic progenitors
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
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Identification of the full-length huntingtin- interacting protein p231HBP/HYPB as a DNA-binding factor
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
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HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
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Localization of huntingtin-interacting protein-2 (Hip-2) mRNA in the developing mouse brain
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
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Bassoon, a novel zinc-finger CAG/glutamine-repeat protein selectively localized at the active zone of presynaptic nerve terminals
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
The huntingtin interacting protein HIP1 is a clathrin and alpha-adaptin-binding protein involved in receptor-mediated endocytosis
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionation
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
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Nuclear relocation of normal huntingtin
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
inferred from DOI database lookup
Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicity
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
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Kennedy's disease: caspase cleavage of the androgen receptor is a crucial event in cytotoxicity
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
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7 January 2021
based on heuristic
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Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disorders
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
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Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: implications for Huntington's disease therapy
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Inhibition of polyglutamine aggregation in R6/2 HD brain slices-complex dose-response profiles
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS1566-2772%2803%2900055-0
retrieved
7 January 2021
based on heuristic
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DOI
10.1016/S1566-2772(03)00055-0
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