Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases. - Wikidata - awgldk - TriplyDB

Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases.

Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases.

http://www.wikidata.org/entity/Q34797845

about

The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease Secondary Structure of Huntingtin Amino-Terminal Region Beta conformation of polyglutamine track revealed by a crystal structure of Huntingtin N-terminal region with insertion of three histidine residues De novo generation of prion strains The ubiquitin proteasome system in glia and its role in neurodegenerative diseases Effect 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 disease The 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 spectroscopy Differential activation of the ER stress factor XBP1 by oligomeric assemblies Sequestration 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 huntingtin Neuropathology 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.

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Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases.

http://www.wikidata.org/entity/Q34797845

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Huntington's disease: revisiti ...... ne neurodegenerative diseases.

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Carly Desmond

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Lise Munsie

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Randy Singh Atwal

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P2860

Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human

An arginine/lysine-rich motif is crucial for VCP/p97-mediated modulation of ataxin-3 fibrillogenesis.

RNA association and nucleocytoplasmic shuttling by ataxin-1

Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity

Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis.

Glutamine-expanded ataxin-7 alters TFTC/STAGA recruitment and chromatin structure leading to photoreceptor dysfunction.

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

Deletion of the triplet repeat encoding polyglutamine within the mouse Huntington's disease gene results in subtle behavioral/motor phenotypes in vivo and elevated levels of ATP with cellular senescence in vitro

Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats

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neurodegeneration

Huntington disease