Destabilization of non-pathological variants of ataxin-3 by metal ions results in aggregation/fibrillogenesis.

No abstract is available for this paper.

• Publication year

  2007

• Venue

  International Journal of Biochemistry and Cell Biology

• Publication date

  Unknown publication date

• Fields of study

  Biology, Medicine, Chemistry

• Identifiers
  DOI 10.1016/J.BIOCEL.2007.01.012PMID 17300980
• 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.

• Heterogeneous amyloid-formed ion channels as a common cytotoxic mechanism

  2007cited by this paper
• Interaction of metals with prion protein: possible role of divalent cations in the pathogenesis of prion diseases.

  2006cited by this paper
• Aggregation/fibrillogenesis of recombinant human prion protein and Gerstmann-Sträussler-Scheinker disease peptides in the presence of metal ions.

  2006cited by this paper
• The Two-stage Pathway of Ataxin-3 Fibrillogenesis Involves a Polyglutamine-independent Step*

  2006cited by this paper
• Aluminum-triggered structural modifications and aggregation of beta-amyloids.

  2005cited by this paper
• The solution structure of the Josephin domain of ataxin-3: structural determinants for molecular recognition.

  2005cited by this paper
• Nature, nurture and neurology: gene–environment interactions in neurodegenerative disease

  2005cited by this paper
• Aluminum-triggered structural modifications and aggregation of β-amyloids

  2005cited by this paper
• Towards a structural understanding of the fibrillization pathway in Machado-Joseph's disease: trapping early oligomers of non-expanded ataxin-3.

  2005cited by this paper
• The crucial role of metal ions in neurodegeneration: the basis for a promising therapeutic strategy

  2005cited by this paper
• Amyloid accomplices and enforcers

  2005cited by this paper
• Metals and amyloid‐β in Alzheimer's disease

  2005cited by this paper
• Copper(II) inhibits in vitro conversion of prion protein into amyloid fibrils.

  2005cited by this paper
• Calcium Dysregulation and Membrane Disruption as a Ubiquitous Neurotoxic Mechanism of Soluble Amyloid Oligomers*♦

  2005cited by this paper
• Controlling (cid:1) -Amyloid Oligomerization by the Use of Naphthalene Sulfonates TRAPPING LOW MOLECULAR WEIGHT OLIGOMERIC SPECIES *

  2005cited by this paper
• Destabilization of a non-pathological variant of ataxin-3 results in fibrillogenesis via a partially folded intermediate: a model for misfolding in polyglutamine disease.

  2004cited by this paper
• Structural and functional analysis of ataxin-2 and ataxin-3.

  2004cited by this paper
• Structural and functional analysis of the Josephin domain of the polyglutamine protein ataxin-3.

  2004cited by this paper
• Aluminium in Alzheimer’s disease: are we still at a crossroad?

  2004cited by this paper
• Characterization of the structure and the amyloidogenic properties of the Josephin domain of the polyglutamine-containing protein ataxin-3.

  2004cited by this paper
• Protein aggregation and neurodegenerative disease

  2004cited by this paper
• Protein aggregation and aggregate toxicity: new insights into protein folding, misfolding diseases and biological evolution

  2003cited by this paper
• An automated method for high-throughput protein purification applied to a comparison of His-tag and GST-tag affinity chromatography

  2003cited by this paper
• Unfolding the role of protein misfolding in neurodegenerative diseases

  2003cited by this paper
• Copper, beta-amyloid, and Alzheimer's disease: tapping a sensitive connection.

  2003cited by this paper
• Beta-amyloid protein oligomers induced by metal ions and acid pH are distinct from those generated by slow spontaneous ageing at neutral pH.

  2003cited by this paper
• Copper, β-amyloid, and Alzheimer's disease: Tapping a sensitive connection

  2003cited by this paper
• Elucidation of ataxin-3 and ataxin-7 function by integrative bioinformatics.

  2003cited by this paper
• Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates

  2003cited by this paper
• Temperature-dependent, irreversible formation of amyloid fibrils by a soluble human ataxin-3 carrying a moderately expanded polyglutamine stretch (Q36).

  2003cited by this paper
• Structural Instability and Fibrillar Aggregation of Non-expanded Human Ataxin-3 Revealed under High Pressure and Temperature*

  2003cited by this paper
• Domain architecture of the polyglutamine protein ataxin‐3: a globular domain followed by a flexible tail

  2003cited by this paper
• Imaging linear birefringence and dichroism in cerebral amyloid pathologies

  2003cited by this paper
• The metallobiology of Alzheimer's disease.

  2003cited by this paper
• The plasma membrane: a target and hurdle for the development of anti-Abeta drugs?

  2002cited by this paper
• Ataxin-3 Is a Histone-binding Protein with Two Independent Transcriptional Corepressor Activities*

  2002cited by this paper
• Amyloid-like features of polyglutamine aggregates and their assembly kinetics.

  2002cited by this paper
• Polyglutamine fibrillogenesis: The pathway unfolds

  2002cited by this paper
• The structural basis of protein folding and its links with human disease.

  2001cited by this paper
• Metal-triggered structural transformations, aggregation, and fibrillation of human alpha-synuclein. A possible molecular NK between Parkinson's disease and heavy metal exposure.

  2001cited by this paper
• Aluminium and Alzheimer's Disease: The Science that Describes the Link

  2001cited by this paper
• Amino acid sequences flanking polyglutamine stretches influence their potential for aggregate formation

  2001cited by this paper
• Polyglutamine expansion neurodegenerative disease.

  2001cited by this paper
• Beyond the Qs in the polyglutamine diseases.

  2001cited by this paper
• Alzheimer's disease: genes, proteins, and therapy.

  2001cited by this paper
• Temperature-dependent β-sheet formation in β-amyloid Aβ1–40 peptide in water: uncoupling β-structure folding from aggregation

  2000cited by this paper
• Fresh and globular amyloid β protein (1–42) induces rapid cellular degeneration: evidence for AβP channel‐mediated cellular toxicity

  2000cited by this paper
• Fourteen and counting: unraveling trinucleotide repeat diseases.

  2000cited by this paper
• Review: history of the amyloid fibril.

  2000cited by this paper
• Metals and neuroscience.

  2000cited by this paper
• Fresh and globular amyloid beta protein (1-42) induces rapid cellular degeneration: evidence for AbetaP channel-mediated cellular toxicity.

  2000cited by this paper
• Ataxin-3 is translocated into the nucleus for the formation of intranuclear inclusions in normal and Machado-Joseph disease brains.

  2000cited by this paper
• Temperature-dependent beta-sheet formation in beta-amyloid Abeta(1-40) peptide in water: uncoupling beta-structure folding from aggregation.

  2000cited by this paper
• Polyglutamine‐induced ion channels: a possible mechanism for the neurotoxicity of huntington and other CAG repeat diseases

  2000cited by this paper
• The role of metals in neurodegenerative diseases.

  2000cited by this paper
• Peptide models for inherited neurodegenerative disorders: conformation and aggregation properties of long polyglutamine peptides with and without interruptions

  1999cited by this paper
• Ataxin-3 with an altered conformation that exposes the polyglutamine domain is associated with the nuclear matrix.

  1999cited by this paper
• Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold.

  1999cited by this paper
• Protein misfolding, evolution and disease.

  1999cited by this paper
• Quantifying amyloid by congo red spectral shift assay.

  1999cited by this paper
• Molecular basis of the neurodegenerative disorders.

  1999cited by this paper
• Glutamine repeats and neurodegenerative diseases: molecular aspects.

  1999cited by this paper
• Nobel Lecture: Prions

  1998cited by this paper
• Heterogeneous intracellular localization and expression of ataxin-3.

  1998cited by this paper
• Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

  1997cited by this paper
• Prion protein and the transmissible spongiform encephalopathies.

  1997cited by this paper
• Characterization of the interactions of Alzheimer beta-amyloid peptides with phospholipid membranes.

  1997cited by this paper
• Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo.

  1997cited by this paper
• Machado‐Joseph disease gene product is a cytoplasmic protein widely expressed in brain

  1997cited by this paper
• Glutamine repeats and inherited neurodegenerative diseases: molecular aspects.

  1996cited by this paper
• Recent Advances in the Genetics of Alzheimer's Disease and Vascular Dementia with an Emphasis on Gene‐Environment Interactions

  1996cited by this paper
• Alternative conformations of amyloidogenic proteins govern their behavior.

  1996cited by this paper
• Mobility and Proximity in Biological Membranes

  1994cited by this paper
• Activation of acetylcholinesterase by aluminium(III): the relevance of the metal species.

  1994cited by this paper
• Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases.

  1994cited by this paper
• Increased body temperature accelerates aggregation of the Leu-68-->Gln mutant cystatin C, the amyloid-forming protein in hereditary cystatin C amyloid angiopathy.

  1994cited by this paper
• Vesicles of variable diameter prepared by a modified injection method.

  1977cited by this paper
• Collisional Perturbation of Spin‐Orbital Coupling and the Mechanism of Fluorescence Quenching. A Visual Demonstration of the Perturbation

  1952cited by this paper

• Rad23B delays Ataxin-3 liquid-to-solid phase transition through heterotypic buffering.

  2025cites this paper
• Fibril-forming motif of non-expanded ataxin-3 revealed by scanning proline mutagenesis

  2024cites this paper
• Big versus small: The impact of aggregate size in disease

  2023cites this paper
• Alteration of Biomolecular Conformation by Aluminum-Implications for Protein Misfolding Disease

  2022cites this paper
• Metal Imbalance in Neurodegenerative Diseases with a Specific Concern to the Brain of Multiple Sclerosis Patients

  2020cites this paper
• Toxicity of heavy metals in plants and animals and their uptake by magnetic iron oxide nanoparticles

  2020cites this paper
• Investigation of the multi-domain aggregation mechanism of ataxin-3

  2017cites this paper
• Effects of Intrinsic and Extrinsic Factors on Aggregation of Physiologically Important Intrinsically Disordered Proteins.

  2017cites this paper
• Josephin Domain Dimerization on a Gold Surface: Evidences for a Double‐Step Binding Pathway

  2017cites this paper
• MODELOS ANIMALES NO TRANSGÉNICOS DE DEMENCIA. CONSIDERACIONES METODOLÓGICAS Y RELEVANCIA FARMACOLÓGICA / NON-TRANSGENIC ANIMAL MODELS OF DEMENTIA. METHODOLOGICAL CONSIDERATIONS AND PHARMACOLOGICAL RELEVANCE

  2015cites this paper
• Moguća povezanost i izloženost aluminija i Alzheimerove bolesti

  2014cites this paper
• Conformational Behavior and Aggregation of Ataxin-3 in SDS

  2013cites this paper
• Genome-Wide Analyses of Metal Responsive Genes in Caenorhabditis elegans

  2012cites this paper
• The coordination chemistry of aluminium in neurodegenerative disease

  2012cites this paper
• Role of metal ions in aggregation of intrinsically disordered proteins in neurodegenerative diseases.

  2011cites this paper
• Link between Aluminum and the Pathogenesis of Alzheimer's Disease: The Integration of the Aluminum and Amyloid Cascade Hypotheses

  2011cites this paper
• Zinc, copper, and carnosine attenuate neurotoxicity of prion fragment PrP106-126.

  2011cites this paper
• Mapping Brain Metals to Evaluate Therapies for Neurodegenerative Disease

  2011cites this paper
• The Role of Mn(II) in Silk Fibroin Based on EPR and NMR Spectroscopy

  2011cites this paper
• Neurotoxic protein oligomerisation associated with polyglutamine diseases

  2010cites this paper
• Ataxin-3 aggregation and its impact on membrane destabilization

  2010cites this paper
• STUDIES ON THE MECHANISM AND PHYSIOLOGICAL ROLE(S) OF THE INTERACTION OF ATAXIN-3 WITH TUBULIN

  2009cites this paper
• Synchrotron X-ray Fluorescence Reveals Abnormal Metal Distributions in Brain and Spinal Cord in Spinocerebellar Ataxia: A Case Report

  2009cites this paper
• Interaction of selected divalent metal ions with human ataxin-3 Q36

  2009cites this paper
• Metal Ion Physiopathology in Neurodegenerative Disorders

  2009cites this paper
• NOVEL SYNCHROTRON-BASED ANALYSES OF METAL PATHOLOGY IN FRIEDREICH'S ATAXIA

  2009cites this paper
• Potential pathogenic role of β-amyloid1–42–aluminum complex in Alzheimer's disease ☆

  2008cites this paper
• Role of metal ions in the abeta oligomerization in Alzheimer's disease and in other neurological disorders.

  2008cites this paper
• Potential pathogenic role of beta-amyloid(1-42)-aluminum complex in Alzheimer's disease.

  2008cites this paper
• Personal Information

  2005cites this paper