Neurotoxic protein oligomerisation associated with polyglutamine diseases.
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Valosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3Novel polyglutamine model uncouples proteotoxicity from agingRNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosisCo-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapyThe Protein Structure Context of PolyQ RegionsStable polyglutamine dimers can contain β-hairpins with interdigitated side chains-but not α-helices, β-nanotubes, β-pseudohelices, or steric zippers.Polyglutamine repeats are associated to specific sequence biases that are conserved among eukaryotes.Polyglutamine toxicity is controlled by prion composition and gene dosage in yeast.Expanded ataxin-7 cause toxicity by inducing ROS production from NADPH oxidase complexes in a stable inducible Spinocerebellar ataxia type 7 (SCA7) model.Fragments of HdhQ150 mutant huntingtin form a soluble oligomer pool that declines with aggregate deposition upon agingSystematic interaction network filtering identifies CRMP1 as a novel suppressor of huntingtin misfolding and neurotoxicityPolyglutamine expansion alters the dynamics and molecular architecture of aggregates in dentatorubropallidoluysian atrophyProteins with Intrinsically Disordered Domains Are Preferentially Recruited to Polyglutamine Aggregates.Reduction of mutant ataxin-7 expression restores motor function and prevents cerebellar synaptic reorganization in a conditional mouse model of SCA7Effects of Pin1 Loss in Hdh(Q111) Knock-in Mice.Expanded polyglutamine-containing N-terminal huntingtin fragments are entirely degraded by mammalian proteasomes.Aggregation formation in the polyglutamine diseases: protection at a cost?Autophagy and polyglutamine diseases.An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicityThe role of amyloidogenic protein oligomerization in neurodegenerative disease.Class A β-lactamases as versatile scaffolds to create hybrid enzymes: applications from basic research to medicine.Proteins Containing Expanded Polyglutamine Tracts and Neurodegenerative Disease.In vitro and in vivo aggregation of a fragment of huntingtin protein directly causes free radical production.Reversible aggregation of PABPN1 pre-inclusion structures.In vitro aggregating β-lactamase-polyQ chimeras do not induce toxic effects in an in vivo Caenorhabditis elegans model.Differential degradation of full-length and cleaved ataxin-7 fragments in a novel stable inducible SCA7 modelPrefibrillar huntingtin oligomers isolated from HD brain potently seed amyloid formation.Atomistic mechanisms of huntingtin N-terminal fragment insertion on a phospholipid bilayer revealed by molecular dynamics simulations.Concluding the amyloid formation pathway of a coiled-coil-based peptide from the size of the critical nucleus.(1)H, (13)C and (15)N backbone resonance assignments of the β-lactamase BlaP from Bacillus licheniformis 749/C and two mutational variants.On the distribution of intranuclear and cytoplasmic aggregates in the brainstem of patients with spinocerebellar ataxia type 2 and 3.Unexpected impact of the number of glutamine residues on metal complex stability.Insights into the Aggregation Mechanism of PolyQ Proteins with Different Glutamine Repeat Lengths.Inhibitory effect of hydrophobic fullerenes on the β-sheet-rich oligomers of a hydrophilic GNNQQNY peptide revealed by atomistic simulations
P2860
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P2860
Neurotoxic protein oligomerisation associated with polyglutamine diseases.
description
article científic
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article scientifique
@fr
articolo scientifico
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artigo científico
@pt
bilimsel makale
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scientific article published on June 2010
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Neurotoxic protein oligomerisation associated with polyglutamine diseases.
@en
Neurotoxic protein oligomerisation associated with polyglutamine diseases.
@nl
type
label
Neurotoxic protein oligomerisation associated with polyglutamine diseases.
@en
Neurotoxic protein oligomerisation associated with polyglutamine diseases.
@nl
prefLabel
Neurotoxic protein oligomerisation associated with polyglutamine diseases.
@en
Neurotoxic protein oligomerisation associated with polyglutamine diseases.
@nl
P2860
P1476
Neurotoxic protein oligomerisation associated with polyglutamine diseases.
@en
P2093
Andreas Wyttenbach
Sarah L Hands
P2860
P2888
P304
P356
10.1007/S00401-010-0703-0
P577
2010-06-01T00:00:00Z