(Q55433686)

9 July 2018

11 references

22 April 2019

25 April 2019

12 September 2019

19 September 2019

28 September 2019

12 October 2019

16 November 2019

22 December 2019

26 August 2020

29 April 2021

19 July 2021

The ubiquitin-proteasome pathway in Huntington's disease. (English)

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9 July 2018

biomedical investigative technique

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22 April 2019

25 April 2019

12 September 2019

19 September 2019

28 September 2019

12 October 2019

16 November 2019

22 December 2019

26 August 2020

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12 September 2019

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19 September 2019

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28 September 2019

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12 October 2019

macromolecular substances

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16 November 2019

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22 December 2019

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26 August 2020

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29 April 2021

proteasome endopeptidase complex

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19 July 2021

Steven Finkbeiner

1

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9 July 2018

Siddhartha Mitra

series ordinal

2

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9 July 2018

language of work or name

English

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publication date

20 April 2008

12 references

9 July 2018

The Scientific World Journal

22 April 2019

25 April 2019

12 September 2019

19 September 2019

28 September 2019

12 October 2019

16 November 2019

22 December 2019

26 August 2020

29 April 2021

19 July 2021

12 references

9 July 2018

22 April 2019

25 April 2019

12 September 2019

19 September 2019

28 September 2019

12 October 2019

16 November 2019

22 December 2019

26 August 2020

29 April 2021

19 July 2021

8

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9 July 2018

page(s)

421-433

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9 July 2018

Creative Commons Attribution 3.0 Unported

copyright license

11 references

22 April 2019

25 April 2019

12 September 2019

19 September 2019

28 September 2019

12 October 2019

16 November 2019

22 December 2019

26 August 2020

29 April 2021

on focus list of Wikimedia project

19 July 2021

copyright status

copyrighted

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ScienceSource

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cites work

Global changes to the ubiquitin system in Huntington's disease.

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12 December 2020

Docking of the proteasomal ATPases' carboxyl termini in the 20S proteasome's alpha ring opens the gate for substrate entry

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12 December 2020

Aging and regulated protein degradation: who has the UPPer hand?

1 reference

12 December 2020

Proteasome inhibition up-regulates p53 and apoptosis-inducing factor in chondrocytes causing severe growth retardation in mice

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12 December 2020

BCL-2 family regulation by the 20S proteasome inhibitor bortezomib

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12 December 2020

In vivo disassembly of free polyubiquitin chains by yeast Ubp14 modulates rates of protein degradation by the proteasome

1 reference

12 December 2020

Recent insights into the molecular pathogenesis of Huntington disease

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12 December 2020

Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease

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12 December 2020

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

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12 December 2020

Glutamine repeats and neurodegeneration

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12 December 2020

The 26S proteasome: a molecular machine designed for controlled proteolysis

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12 December 2020

Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease.

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12 December 2020

Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function.

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12 December 2020

A superfamily of protein tags: ubiquitin, SUMO and related modifiers

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12 December 2020

Alzheimer's associated variant ubiquitin causes inhibition of the 26S proteasome and chaperone expression

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12 December 2020

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

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12 December 2020

Huntington's disease: clinical correlates of disability and progression

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12 December 2020

The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity

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12 December 2020

Yeast genes that enhance the toxicity of a mutant huntingtin fragment or alpha-synuclein

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12 December 2020

Protein degradation and protection against misfolded or damaged proteins

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12 December 2020

Proteasomes and their kin: proteases in the machine age

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12 December 2020

A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG length.

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12 December 2020

Ubiquitin as a central cellular regulator.

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12 December 2020

SUMO modification of Huntingtin and Huntington's disease pathology

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12 December 2020

Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins

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12 December 2020

Accumulation of aberrant ubiquitin induces aggregate formation and cell death in polyglutamine diseases

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12 December 2020

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

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12 December 2020

Inefficient degradation of truncated polyglutamine proteins by the proteasome

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12 December 2020

Computed Tomographic, Neurologic, and Neuropsychological Correlates of Huntington'S Disease

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12 December 2020

Components of ubiquitin-protein ligase system. Resolution, affinity purification, and role in protein breakdown

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12 December 2020

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

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12 December 2020

Neuropathological classification of Huntington's disease

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12 December 2020

Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease

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12 December 2020

Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy

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12 December 2020

Purification of an 11 S regulator of the multicatalytic protease

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

PA28 activator protein forms regulatory caps on proteasome stacked rings.

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12 December 2020

Impairment of the ubiquitin-proteasome system by protein aggregation

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12 December 2020

Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease

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12 December 2020

Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease.

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12 December 2020

Effects of intracellular expression of anti-huntingtin antibodies of various specificities on mutant huntingtin aggregation and toxicity

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12 December 2020

Properties of the hybrid form of the 26S proteasome containing both 19S and PA28 complexes.

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12 December 2020

Ultrastructure of nuclear aggregates formed by expressing an expanded polyglutamine

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12 December 2020

The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt.

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12 December 2020

The amygdala and reward

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12 December 2020

Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis.

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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

Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease

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12 December 2020

Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

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12 December 2020

Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease

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12 December 2020

Identities of sequestered proteins in aggregates from cells with induced polyglutamine expression

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice

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12 December 2020

Intranuclear ataxin1 inclusions contain both fast- and slow-exchanging components

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12 December 2020

Aggregate formation inhibits proteasomal degradation of polyglutamine proteins.

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12 December 2020

Inactivation of the mouse Huntington's disease gene homolog Hdh

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12 December 2020

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

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12 December 2020

Expression of normal and mutant huntingtin in the developing brain

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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

Human huntingtin derived from YAC transgenes compensates for loss of murine huntingtin by rescue of the embryonic lethal phenotype

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12 December 2020

Ubiquitin-dependent protein degradation

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12 December 2020

UBE3A/E6-AP mutations cause Angelman syndrome

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12 December 2020

Role and function of the 26S proteasome in proliferation and apoptosis.

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12 December 2020

De novo truncating mutations in E6-AP ubiquitin-protein ligase gene (UBE3A) in Angelman syndrome.

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12 December 2020

Heterogeneous topographic and cellular distribution of huntingtin expression in the normal human neostriatum.

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Structure of the proteasome activator REGalpha (PA28alpha)

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12 December 2020

Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism

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12 December 2020

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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

Differences in duration of Huntington's disease based on age at onset

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12 December 2020

A novel ubiquitination factor, E4, is involved in multiubiquitin chain assembly.

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12 December 2020

Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein

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12 December 2020

Polyubiquitin chains: polymeric protein signals

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12 December 2020

Mechanism and function of deubiquitinating enzymes

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12 December 2020

Mobilizing the proteolytic machine: cell biological roles of proteasome activators and inhibitors

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12 December 2020

Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation.

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12 December 2020

Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes.

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12 December 2020

Multiubiquitylation by E4 enzymes: 'one size' doesn't fit all.

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12 December 2020

Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation

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12 December 2020

Structural properties and neuronal toxicity of amyotrophic lateral sclerosis-associated Cu/Zn superoxide dismutase 1 aggregates.

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12 December 2020

Preparation of hybrid (19S-20S-PA28) proteasome complexes and analysis of peptides generated during protein degradation

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12 December 2020

A genomic and functional inventory of deubiquitinating enzymes

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12 December 2020

ATP binding to PAN or the 26S ATPases causes association with the 20S proteasome, gate opening, and translocation of unfolded proteins.

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12 December 2020

Progressive disruption of cellular protein folding in models of polyglutamine diseases.

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12 December 2020

A network of protein interactions determines polyglutamine toxicity

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12 December 2020

A balance of protein synthesis and proteasome-dependent degradation determines the maintenance of LTP.

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12 December 2020

Genetic and expression aberrations of E3 ubiquitin ligases in human breast cancer

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12 December 2020

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

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12 December 2020

Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA)

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12 December 2020

Instability of CAG repeats in Huntington's disease: relation to parental transmission and age of onset

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12 December 2020

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

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12 December 2020

CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1

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12 December 2020

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

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12 December 2020

Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes

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12 December 2020

The roles of intracellular protein-degradation pathways in neurodegeneration

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12 December 2020

Global organization and function of mammalian cytosolic proteasome pools: Implications for PA28 and 19S regulatory complexes

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12 December 2020

Interplay between protein synthesis and degradation in the CNS: physiological and pathological implications

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12 December 2020

Ubiquitin-conjugating enzyme E2-25K increases aggregate formation and cell death in polyglutamine diseases

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12 December 2020

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12 December 2020

RLE-1, an E3 ubiquitin ligase, regulates C. elegans aging by catalyzing DAF-16 polyubiquitination

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12 December 2020

Ubiquitin ligase Hrd1 enhances the degradation and suppresses the toxicity of polyglutamine-expanded huntingtin

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12 December 2020

Puromycin-sensitive aminopeptidase is the major peptidase responsible for digesting polyglutamine sequences released by proteasomes during protein degradation

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12 December 2020

Autophagy and neurodegeneration: when the cleaning crew goes on strike

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12 December 2020

Differential morphology and composition of inclusions in the R6/2 mouse and PC12 cell models of Huntington's disease.

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12 December 2020

Dose-dependent inhibition of proteasome activity by a mutant ubiquitin associated with neurodegenerative disease.

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12 December 2020

HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS

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12 December 2020

Identifiers

DOI

10.1100/TSW.2008.60

1 reference

9 July 2018

PMCID

2637619

1 reference

9 July 2018

PubMed ID

18454252

1 reference

9 July 2018