Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity. (Q36324397)

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20 December 2019

title

Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity (English)

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20 December 2019

5

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20 December 2019

author name string

Hui Zhou

1

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20 December 2019

Fengli Cao

2

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20 December 2019

Zhishan Wang

3

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20 December 2019

Zhao-Xue Yu

4

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20 December 2019

Joy Evans

6

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20 December 2019

Shi-Hua Li

7

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20 December 2019

Xiao-Jiang Li

8

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20 December 2019

language of work or name

English

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publication date

1 October 2003

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20 December 2019

number of pages

10

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inferred from page(s)

published in

Journal of Cell Biology

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20 December 2019

volume

163

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20 December 2019

issue

1

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20 December 2019

page(s)

109-118

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Polyglutamine repeat length-dependent proteolysis of huntingtin

20 December 2019

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1 reference

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

30 September 2017

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

30 September 2017

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30 September 2017

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Ultrastructure of nuclear aggregates formed by expressing an expanded polyglutamine

30 September 2017

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Interaction of Huntington disease protein with transcriptional activator Sp1

30 September 2017

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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.

30 September 2017

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

30 September 2017

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Impairment of the ubiquitin-proteasome system by protein aggregation

30 September 2017

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

30 September 2017

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Beyond the Qs in the polyglutamine diseases

30 September 2017

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Neurological abnormalities in a knock-in mouse model of Huntington's disease

30 September 2017

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

30 September 2017

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Decreased levels of proteasome activity and proteasome expression in aging spinal cord

30 September 2017

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

30 September 2017

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Aggresomes: a cellular response to misfolded proteins

30 September 2017

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Proteasome inhibitors: valuable new tools for cell biologists

30 September 2017

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

30 September 2017

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

30 September 2017

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30 September 2017

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

30 September 2017

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30 September 2017

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30 September 2017

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30 September 2017

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A new inhibitor of the chymotrypsin-like activity of the multicatalytic proteinase complex (20S proteasome) induces accumulation of ubiquitin-protein conjugates in a neuronal cell.

30 September 2017

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30 September 2017

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22 September 2018

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21 January 2018

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21 January 2018

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21 January 2018

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PubMed

https://pubmed.ncbi.nlm.nih.gov/14557250

12 December 2020

inferred from PubMed ID database lookup

Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/14557250

12 December 2020

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Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin

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PubMed

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12 December 2020

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Identifiers

DOI

10.1083/JCB.200306038

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14557250%20AND%20SRC:MED&resulttype=core&format=json

20 December 2019

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20 December 2019

1 reference