Are Huntington's and polyglutamine-based ataxias proteasome storage diseases? (Q35097613)

6 August 2017

1 reference

title

Are Huntington's and polyglutamine-based ataxias proteasome storage diseases? (English)

1 reference

6 August 2017

0 references

Geoffrey M Goellner

1

1 reference

PubMed ID

12672449

6 August 2017

Martin Rechsteiner

series ordinal

2

1 reference

6 August 2017

1 May 2003

1 reference

International Journal of Biochemistry & Cell Biology

6 August 2017

1 reference

6 August 2017

35

1 reference

6 August 2017

5

1 reference

6 August 2017

562-571

1 reference

Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice.

6 August 2017

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Mammalian, yeast, bacterial, and chemical chaperones reduce aggregate formation and death in a cell model of oculopharyngeal muscular dystrophy

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Novel therapies in the search for a cure for Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Impairment of the ubiquitin-proteasome system by protein aggregation

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Severe deficiencies in dopamine signaling in presymptomatic Huntington's disease mice

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Truncated forms of the androgen receptor are associated with polyglutamine expansion in X-linked spinal and bulbar muscular atrophy

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Rescue of polyglutamine-mediated cytotoxicity by double-stranded RNA-mediated RNA interference

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Epidermal immunization by a needle-free powder delivery technology: Immunogenicity of influenza vaccine and protection in mice

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Tissue transglutaminase does not contribute to the formation of mutant huntingtin aggregates

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

Identification and characterization of an ataxin-1-interacting protein: A1Up, a ubiquitin-like nuclear protein

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

Polyglutamine-mediated aggregation and cell death

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Polyglutamine expansion, protein aggregation, proteasome activity, and neural survival.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

The human 26 S and 20 S proteasomes generate overlapping but different sets of peptide fragments from a model protein substrate.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Transplanted fetal striatum in Huntington's disease: phenotypic development and lack of pathology

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Transcriptional activation modulated by homopolymeric glutamine and proline stretches

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Human genetic diseases due to codon reiteration: relationship to an evolutionary mechanism

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Amyloid beta-protein inhibits ubiquitin-dependent protein degradation in vitro

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Binding of amyloid beta protein to the 20 S proteasome

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

The behavior of calpain-generated N- and C-terminal fragments of talin in integrin-mediated signaling pathways

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

Simultaneous binding of PA28 and PA700 activators to 20 S proteasomes

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

Therapeutic opportunities in polyglutamine disease

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Aberrant protein deposition and neurological disease

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

A bivalent Huntingtin binding peptide suppresses polyglutamine aggregation and pathogenesis in Drosophila

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Genetic suppression of polyglutamine toxicity in Drosophila

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Reconstitution of hybrid proteasomes from purified PA700-20 S complexes and PA28alphabeta activator: ultrastructure and peptidase activities

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

Parkinson's disease: one biochemical pathway to fit all genes?

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Human single-chain Fv intrabodies counteract in situ huntingtin aggregation in cellular models of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Molecular dissection of the 11S REG (PA28) proteasome activators

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Expansion explosion: new clues to the pathogenesis of repeat expansion neurodegenerative diseases

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Expanded polyglutamine peptides alone are intrinsically cytotoxic and cause neurodegeneration in Drosophila

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

Huntington toxicity in yeast model depends on polyglutamine aggregation mediated by a prion-like protein Rnq1

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Growth retardation in mice lacking the proteasome activator PA28gamma

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Inhibition of polyglutamine protein aggregation and cell death by novel peptides identified by phage display screening

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Cleavage motifs of the yeast 20S proteasome beta subunits deduced from digests of enolase 1.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

The role of chaperones in polyglutamine disease

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Glutamine repeats and neurodegenerative diseases: molecular aspects

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Arfaptin 2 regulates the aggregation of mutant huntingtin protein

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Calpain cleavage of integrin beta cytoplasmic domains

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

The proteasome activator 11 S REG (PA28) and class I antigen presentation

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Protein surveillance machinery in brains with spinocerebellar ataxia type 3: redistribution and differential recruitment of 26S proteasome subunits and chaperones to neuronal intranuclear inclusions

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Intraneuronal aggregate formation and cell death after viral expression of expanded polyglutamine tracts in the adult rat brain.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Inducible expression of mutant alpha-synuclein decreases proteasome activity and increases sensitivity to mitochondria-dependent apoptosis

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Altered acetylation in polyglutamine disease: an opportunity for therapeutic intervention?

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Toxic proteins in neurodegenerative disease

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Discrete cleavage motifs of constitutive and immunoproteasomes revealed by quantitative analysis of cleavage products

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Aggregate formation inhibits proteasomal degradation of polyglutamine proteins.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

PQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

PQBP-1/Npw38, a nuclear protein binding to the polyglutamine tract, interacts with U5-15kD/dim1p via the carboxyl-terminal domain

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

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%2FS1357-2725%2802%2900388-6

7 January 2021

Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

p62 Is a common component of cytoplasmic inclusions in protein aggregation diseases

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

Glutamine repeats and neurodegeneration

1 reference

https://api.crossref.org/works/10.1016%2FS1357-2725%2802%2900388-6

7 January 2021

10.1016/S1357-2725(02)00388-6

Identifiers

DOI

1 reference

6 August 2017

1 reference

PubMed ID

12672449