Polyglutamine protein aggregation and toxicity are linked to the cellular stress response.

No abstract is available for this paper.

• Publication year

  2003

• Venue

  Human Molecular Genetics

• Publication date

  2003-06-15

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1093/HMG/DDG151PMID 12783846
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Molecular Chaperones in the Cytosol: from Nascent Chain to Folded Protein

  2002cited by this paper
• Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells.

  2002cited by this paper
• Invited review: Interplay between molecular chaperones and signaling pathways in survival of heat shock.

  2002cited by this paper
• Genetic modulation of polyglutamine toxicity by protein conjugation pathways in Drosophila.

  2002cited by this paper
• Polyglutamine Expansion, Protein Aggregation, Proteasome Activity, and Neural Survival*

  2002cited by this paper
• ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats.

  2002cited by this paper
• Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice.

  2001influential reference
• Intracellular Aggregation of Polypeptides with Expanded Polyglutamine Domain Is Stimulated by Stress-Activated Kinase Mekk1

  2001cited by this paper
• Glucocorticoid modulation of androgen receptor nuclear aggregation and cellular toxicity is associated with distinct forms of soluble expanded polyglutamine protein.

  2001cited by this paper
• Genetic suppression of polyglutamine toxicity in Drosophila.

  2000cited by this paper
• Molecular genetics: Unmasking polyglutamine triggers in neurodegenerative disease

  2000cited by this paper
• Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins.

  2000cited by this paper
• HSP72 can protect cells from heat-induced apoptosis by accelerating the inactivation of stress kinase JNK

  2000cited by this paper
• Chaperones Hsp70 and Hsp40 Suppress Aggregate Formation and Apoptosis in Cultured Neuronal Cells Expressing Truncated Androgen Receptor Protein with Expanded Polyglutamine Tract*

  2000cited by this paper
• Polyglutamine diseases: protein cleavage and aggregation.

  1999cited by this paper
• Analysis of the Role of Heat Shock Protein (Hsp) Molecular Chaperones in Polyglutamine Disease

  1999cited by this paper
• Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.

  1999cited by this paper
• Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone.

  1999cited by this paper
• Nuclear inclusions in spinocerebellar ataxia type 1

  1999cited by this paper
• Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro.

  1999cited by this paper
• Triggering of neuronal cell death by accumulation of activated SEK1 on nuclear polyglutamine aggregations in PML bodies

  1999cited by this paper
• Kennedy's Disease

  1999cited by this paper
• Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70

  1999cited by this paper
• Mammalian cytosolic DnaJ homologues affect the hsp70 chaperone-substrate reaction cycle, but do not interact directly with nascent or newly synthesized proteins.

  1998cited by this paper
• Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1

  1998cited by this paper
• Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy

  1998cited by this paper
• Neuronal distribution of intranuclear inclusions in Huntington's disease with adult onset

  1998cited by this paper
• Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length.

  1998cited by this paper
• Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions.

  1998cited by this paper
• Expression of Polyglutamine-expanded Huntingtin Activates the SEK1-JNK Pathway and Induces Apoptosis in a Hippocampal Neuronal Cell Line*

  1998cited by this paper
• Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy.

  1998cited by this paper
• Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.

  1997cited by this paper
• Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.

  1997cited by this paper
• Characterization of an expanded glutamine repeat androgen receptor in a neuronal cell culture system.

  1997cited by this paper
• Stability of an expanded trinucleotide repeat in the androgen receptor gene in transgenic mice

  1995cited by this paper
• Role of the major heat shock proteins as molecular chaperones.

  1993cited by this paper
• The constitutive and stress inducible forms of hsp 70 exhibit functional similarities and interact with one another in an ATP- dependent fashion

  1993cited by this paper
• Is hsp70 the cellular thermometer?

  1991cited by this paper
• Cellular and biochemical events in mammalian cells during and after recovery from physiological stress

  1986cited by this paper
• Nuclear and nucleolar localization of the 72,000-dalton heat shock protein in heat-shocked mammalian cells.

  1984cited by this paper

• Oxidative stress-induced proinflammatory secretion from retinal pigment epithelium disrupts NAD+ metabolism via CD38 on macrophages leading to immune microenvironment dysregulation.

  2025cites this paper
• The stress granule protein G3BP1 alleviates spinocerebellar ataxia-associated deficits

  2022cites this paper
• HSF1 and Its Role in Huntington's Disease Pathology.

  2022cites this paper
• Stress granules, RNA-binding proteins and polyglutamine diseases: too much aggregation?

  2021cites this paper
• Early Downregulation of p75NTR by Genetic and Pharmacological Approaches Delays the Onset of Motor Deficits and Striatal Dysfunction in Huntington’s Disease Mice

  2018cites this paper
• X-Linked Spinal and Bulbar Muscular Atrophy: From Clinical Genetic Features and Molecular Pathology to Mechanisms Underlying Disease Toxicity.

  2018cites this paper
• A Variable Polyglutamine Repeat Affects Subcellular Localization and Regulatory Activity of a Populus ANGUSTIFOLIA Protein

  2018cites this paper
• Noncoding RNAs in protein clearance pathways: implications in neurodegenerative diseases

  2017cites this paper
• Etudes des mécanismes à l'origine de l'excès de garçons dans la déficience intellectuelle et les troubles du spectre autistique

  2017cites this paper
• Heat Shock Proteins for Neurological Gene Therapy

  2016cites this paper
• The role of oxidative and inflammatory stress and persistent viral infections in immunosenescence.

  2016cites this paper
• Androgen receptor‐related diseases: what do we know?

  2016cites this paper
• How to Stabilize Both the Proteins and the Membranes: Diverse Effects of sHsps in Neuroprotection

  2015cites this paper
• Oxidative Stress Protection by Exogenous Delivery of rhHsp70 Chaperone to the Retinal Pigment Epithelium (RPE), a Possible Therapeutic Strategy Against RPE Degeneration

  2014cites this paper
• The effects of selenium on methylmercury toxicity in zebrafish

  2014cites this paper
• Increase of oxidation and inflammation in nervous and immune systems with aging and anxiety.

  2014cites this paper
• Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy.

  2013cites this paper
• Consensus Paper: Pathological Mechanisms Underlying Neurodegeneration in Spinocerebellar Ataxias

  2013cites this paper
• Interaction Between DUSP8 and the Polyglutamine Protein Ataxin-1

  2013cites this paper
• Reduction of polyglutamine toxicity by TDP-43, FUS and progranulin in Huntington's disease models.

  2013cites this paper
• Early-forming aberrant aggregates in protein deposition diseases: structural characteristics, interaction with molecular chaperones, ability to trigger inflammation

  2013cites this paper
• Cerebrospinal fluid biomarkers for neurodegenerative disorders

  2013cites this paper
• c-Jun NH2-Terminal Kinase 1/2 and Endoplasmic Reticulum Stress as Interdependent and Reciprocal Causation in Diabetic Embryopathy

  2013cites this paper
• Age-related oxidative stress compromises endosomal proteostasis.

  2012cites this paper
• Implication of oxidative lesions and base excision repair in the tissue selectivity of the somatic instability of CAG repeats in Huntington’s diseease

  2012cites this paper
• Aberrations in Proteostasis Orchestrate: the Genotypic and Phenotypic Changes in Aging

  2012cites this paper
• Natural osmolytes remodel the aggregation pathway of mutant huntingtin exon 1.

  2011cites this paper
• Polyglutamine diseases (PolyQ): construction and characterization of yeast models

  2011cites this paper
• Oxidative stress, inflamm-aging and immunosenescence.

  2011cites this paper
• High-Mobility Group Box 1, Oxidative Stress, and Disease

  2011cites this paper
• Protein homeostasis in models of aging and age-related conformational disease.

  2010cites this paper
• Toxic intermediates and protein quality control in the polyglutamine disease, SCA3

  2010cites this paper
• Oxidative stress-enhanced SUMOylation and aggregation of ataxin-1: Implication of JNK pathway.

  2010cites this paper
• Towards a unifying, systems biology understanding of large-scale cellular death and destruction caused by poorly liganded iron: Parkinson’s, Huntington’s, Alzheimer’s, prions, bactericides, chemical toxicology and others as examples

  2010cites this paper
• Correlation Between Function-Morphology of Effect of the Stress and Flunitrazepam Administered During Gestation in Adult Mouse Ovary

  2010cites this paper
• Heat shock proteins in neurodegenerative diseases: Pathogenic roles and therapeutic implications

  2009cites this paper
• Progress in spinobulbar muscular atrophy research: Insights into neuronal dysfunction caused by the polyglutamine-expanded androgen receptor

  2009influential citation
• The role of molecular chaperones in human misfolding diseases

  2009cites this paper
• Pathogenesis‐targeting therapeutics for spinal and bulbar muscular atrophy (SBMA)

  2009cites this paper
• Investigation of the mechanism of toxicity in newly established models of polyglutamine diseases

  2008cites this paper
• Protein Quality Control and Heat Shock Gene Expression in the Nervous System

  2008cites this paper
• HSP27 and cell death in spinocerebellar ataxia type 3

  2008cites this paper
• PolyQ-expanded ataxin-3 interacts with full-length ataxin-3 in a polyQ length-dependent manner

  2008cites this paper
• Transcriptional malfunctioning of heat shock protein gene expression in spinocerebellar ataxias

  2008cites this paper
• N-terminal polyglutamine-containing fragments inhibit androgen receptor transactivation function

  2008influential citation
• Chaperones and Polyglutamine Expansion Disorders

  2008cites this paper
• PolyQ-expanded ataxin-3 interacts with full-length ataxin-3 in a polyQ length-dependent manner

  2008cites this paper
• Protective up‐regulation of CK2 by mutant huntingtin in cells co‐expressing NMDA receptors

  2007cites this paper
• Polyglutamine diseases: emerging concepts in pathogenesis and therapy.

  2007cites this paper
• Pathogenesis and molecular targeted therapy of spinal and bulbar muscular atrophy

  2007cites this paper
• Intersectin enhances huntingtin aggregation and neurodegeneration through activation of c-Jun-NH2-terminal kinase.

  2007cites this paper
• New Tricks for an Old Dog

  2007cites this paper
• Perturbed signal transduction in neurodegenerative disorders involving aberrant protein aggregation

  2007cites this paper
• Role of heat shock proteins during polyglutamine neurodegeneration

  2007cites this paper
• Soluble Androgen Receptor Oligomers Underlie Pathology in a Mouse Model of Spinobulbar Muscular Atrophy*

  2007cites this paper
• Ataxin-1 Fusion Partners Alter PolyQ Lethality and Aggregation

  2007cites this paper
• Translocation and Release Peroxide )-Induced HMGB 1 Cytoplasmic Regulator of Oxidative Stress ( Hydrogen Nuclear Heat Shock Protein 72 as a Negative

  2007cites this paper
• Phosphorylation of ataxin-3 by glycogen synthase kinase 3beta at serine 256 regulates the aggregation of ataxin-3.

  2007cites this paper
• Nuclear Heat Shock Protein 72 as a Negative Regulator of Oxidative Stress (Hydrogen Peroxide)-Induced HMGB1 Cytoplasmic Translocation and Release1

  2007cites this paper
• Molecular Pathogenesis of the Polyglutamine Disease: Spinal and Bulbar Muscular Atrophy

  2007cites this paper
• Polyglutamine Disorders Including Huntington's Disease

  2007cites this paper
• Aggregationsverhalten des Polyglutaminproteins Ataxin-3 in vitro und in Zellkultur

  2006cites this paper
• A Drosophila ortholog of the human MRJ modulates polyglutamine toxicity and aggregation.

  2006cites this paper
• JNK mediates pathogenic effects of polyglutamine-expanded androgen receptor on fast axonal transport

  2006influential citation
• Biologically active molecules that reduce polyglutamine aggregation and toxicity.

  2006cites this paper
• Mutant huntingtin alters MAPK signaling pathways in PC12 and striatal cells: ERK1/2 protects against mutant huntingtin-associated toxicity.

  2006influential citation
• Altered Expression of the Noncoding hsrω Gene Enhances poly-Q Induced Neurotoxicity in Drosophila

  2006cites this paper
• Pathogenesis, animal models and therapeutics in spinal and bulbar muscular atrophy (SBMA).

  2006cites this paper
• Role of molecular chaperones in neurodegenerative disorders

  2005cites this paper
• Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease.

  2005cites this paper
• Polyglutamine expansion diseases: failing to deliver.

  2005cites this paper
• Regulation of heat shock gene transcription in neuronal cells

  2005cites this paper
• Mitochondrial impairment in patients and asymptomatic mutation carriers of Huntington's disease

  2005cites this paper
• Saccharomyces cerevisiae Hsp104 enhances the chaperone capacity of human cells and inhibits heat stress-induced proapoptotic signaling.

  2004cites this paper
• Role of quality control pathways in human diseases involving protein misfolding.

  2004cites this paper
• Expression and functional analysis of the SCA7 disease protein ataxin-7.

  2004cites this paper
• Stress responses of PML nuclear domains are ablated by ataxin‐1 and other nucleoprotein inclusions

  2004cites this paper
• The great tragedy of science : The slaying of a beautiful hypothesis by an ugly fact

  2004cites this paper
• Desmin aggregate formation by R120G alphaB-crystallin is caused by altered filament interactions and is dependent upon network status in cells.

  2004cites this paper
• Stress under the dam: meeting report of the Fourth International Workshop on the Molecular Biology of Stress Responses

  2004cites this paper
• Heat shock-induced signal transduction in hematopoietic cells

  2003cites this paper
• Gene Expression Profiling in Ataxin‐3 Expressing Cell Lines Reveals Distinct Effects of Normal and Mutant Ataxin‐3

  2003cites this paper
• A fragile balance: FMR1 expression levels.

  2003cites this paper
• [The androgen receptor: molecular pathology].

  2002cites this paper