Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases.
about
The importance of integrating basic and clinical research toward the development of new therapies for Huntington diseaseSecondary Structure of Huntingtin Amino-Terminal RegionBeta conformation of polyglutamine track revealed by a crystal structure of Huntingtin N-terminal region with insertion of three histidine residuesDe novo generation of prion strainsThe ubiquitin proteasome system in glia and its role in neurodegenerative diseasesEffect of charged amino acid side chain length on lateral cross-strand interactions between carboxylate- and guanidinium-containing residues in a β-hairpin.Therapeutic prospects for spinocerebellar ataxia type 2 and 3.Composite primary neuronal high-content screening assay for Huntington's disease incorporating non-cell-autonomous interactions.Polyglutamine induced misfolding of huntingtin exon1 is modulated by the flanking sequences.Evaluating nuclei concentration in amyloid fibrillation reactions using back-calculation approach.Prion formation and polyglutamine aggregation are controlled by two classes of genes.Differential vulnerability of neurons in Huntington's disease: the role of cell type-specific features.An independent study of the preclinical efficacy of C2-8 in the R6/2 transgenic mouse model of Huntington's diseaseThe interplay of turn formation and hydrophobic interactions on the early kinetic events in protein folding.Cystamine and intrabody co-treatment confers additional benefits in a fly model of Huntington's disease.Pathogenic polyglutamine expansion length correlates with polarity of the flanking sequences.Pathogenesis and molecular targeted therapy of spinal and bulbar muscular atrophy (SBMA).Multivesicular bodies in neurons: distribution, protein content, and trafficking functions.Opposing effects of glutamine and asparagine govern prion formation by intrinsically disordered proteins.Changes in BiP availability reveal hypersensitivity to acute endoplasmic reticulum stress in cells expressing mutant huntingtin.Polyglutamine- and temperature-dependent conformational rigidity in mutant huntingtin revealed by immunoassays and circular dichroism spectroscopyDifferential activation of the ER stress factor XBP1 by oligomeric assembliesSequestration of Sup35 by aggregates of huntingtin fragments causes toxicity of [PSI+] yeast.The sirtuin 2 inhibitor AK-7 is neuroprotective in Huntington's disease mouse models.Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtinNeuropathology and therapeutic intervention in spinal and bulbar muscular atrophy.Single homopolypeptide chains collapse into mechanically rigid conformations.Computational approaches to understanding protein aggregation in neurodegeneration.Advances in huntington disease drug discovery: novel approaches to model disease phenotypes.Primary cilia and autophagic dysfunction in Huntington's disease.Functionally different α-synuclein inclusions yield insight into Parkinson's disease pathology.Kinase inhibitors modulate huntingtin cell localization and toxicity.Interfacial interaction and lateral association of cross-seeding assemblies between hIAPP and rIAPP oligomers.Rab11 rescues synaptic dysfunction and behavioural deficits in a Drosophila model of Huntington's disease.SUMOylation attenuates the aggregation propensity and cellular toxicity of the polyglutamine expanded ataxin-7.Manganese and the Insulin-IGF Signaling Network in Huntington's Disease and Other Neurodegenerative Disorders.The composition of the polyglutamine-containing proteins influences their co-aggregation properties.Conformations of the Huntingtin N-term in aqueous solution from atomistic simulations.
P2860
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P2860
Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases.
description
2008 nî lūn-bûn
@nan
2008 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
name
Huntington's disease: revisiti ...... ne neurodegenerative diseases.
@ast
Huntington's disease: revisiti ...... ne neurodegenerative diseases.
@en
Huntington's disease: revisiti ...... ne neurodegenerative diseases.
@nl
type
label
Huntington's disease: revisiti ...... ne neurodegenerative diseases.
@ast
Huntington's disease: revisiti ...... ne neurodegenerative diseases.
@en
Huntington's disease: revisiti ...... ne neurodegenerative diseases.
@nl
prefLabel
Huntington's disease: revisiti ...... ne neurodegenerative diseases.
@ast
Huntington's disease: revisiti ...... ne neurodegenerative diseases.
@en
Huntington's disease: revisiti ...... ne neurodegenerative diseases.
@nl
P2093
P2860
P1433
P1476
Huntington's disease: revisiti ...... ne neurodegenerative diseases.
@en
P2093
Carly Desmond
Lise Munsie
Randy Singh Atwal
Ray Truant
P2860
P304
P356
10.1111/J.1742-4658.2008.06561.X
P407
P577
2008-07-15T00:00:00Z