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Adaptive preconditioning in neurological diseases – therapeutic insights from proteostatic perturbationsUBA1: At the Crossroads of Ubiquitin Homeostasis and NeurodegenerationIron dysregulation in Huntington's diseaseModeling Huntington's disease with induced pluripotent stem cellsMutant huntingtin gene-dose impacts on aggregate deposition, DARPP32 expression and neuroinflammation in HdhQ150 miceStructural Mechanisms of Mutant Huntingtin Aggregation Suppression by the Synthetic Chaperonin-like CCT5 Complex Explained by Cryoelectron Tomography.Huntington's disease induced cardiac amyloidosis is reversed by modulating protein folding and oxidative stress pathways in the Drosophila heartHuman mutant huntingtin disrupts vocal learning in transgenic songbirdsGlutamine and Asparagine Side Chain Hyperconjugation-Induced Structurally Sensitive Vibrations.The aggregation and inheritance of damaged proteins determines cell fate during mitosis.In Vivo Roles of a Patient-Derived Induced Pluripotent Stem Cell Line (HD72-iPSC) in the YAC128 Model of Huntington's Disease.Metformin intake associates with better cognitive function in patients with Huntington's disease.A selective inhibitor of histone deacetylase 3 prevents cognitive deficits and suppresses striatal CAG repeat expansions in Huntington's disease miceProteostasis in striatal cells and selective neurodegeneration in Huntington's disease.Reducing Igf-1r levels leads to paradoxical and sexually dimorphic effects in HD mice.Prion-like proteins sequester and suppress the toxicity of huntingtin exon 1.A coarse-grained model for polyglutamine aggregation modulated by amphipathic flanking sequencesHuntington's disease (HD): degeneration of select nuclei, widespread occurrence of neuronal nuclear and axonal inclusions in the brainstem.Biochemical characterization of mutants in chaperonin proteins CCT4 and CCT5 associated with hereditary sensory neuropathy.Unmasking the roles of N- and C-terminal flanking sequences from exon 1 of huntingtin as modulators of polyglutamine aggregation.Polyglutamine amyloid core boundaries and flanking domain dynamics in huntingtin fragment fibrils determined by solid-state nuclear magnetic resonance.A phenotypic screening assay for modulators of huntingtin-induced transcriptional dysregulation.Transcription, epigenetics and ameliorative strategies in Huntington's Disease: a genome-wide perspectiveDevelopment of an ELISA assay for the quantification of soluble huntingtin in human blood cells.Proteins in aggregates functionally impact multiple neurodegenerative disease models by forming proteasome-blocking complexes.Phosphorylation of mutant huntingtin at serine 116 modulates neuronal toxicity.Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.Nuclear retention of full-length HTT RNA is mediated by splicing factors MBNL1 and U2AF65Analysis of YFP(J16)-R6/2 reporter mice and postmortem brains reveals early pathology and increased vulnerability of callosal axons in Huntington's disease.The emerging role of the first 17 amino acids of huntingtin in Huntington's diseaseThe dynamics of early-state transcriptional changes and aggregate formation in a Huntington's disease cell modelcAMP-induced phosphorylation of 26S proteasomes on Rpn6/PSMD11 enhances their activity and the degradation of misfolded proteins.Studying polyglutamine aggregation in Caenorhabditis elegans using an analytical ultracentrifuge equipped with fluorescence detection.Effects of Pin1 Loss in Hdh(Q111) Knock-in Mice.Brain intracellular metabolites are freely diffusing along cell fibers in grey and white matter, as measured by diffusion-weighted MR spectroscopy in the human brain at 7 T.Non-random distribution of homo-repeats: links with biological functions and human diseases.TRiC's tricks inhibit huntingtin aggregation.Aggregation formation in the polyglutamine diseases: protection at a cost?Inclusion bodies enriched for p62 and polyubiquitinated proteins in macrophages protect against atherosclerosisscyllo-Inositol promotes robust mutant Huntingtin protein degradation.
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P2860
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on 19 December 2011
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Protein aggregates in Huntington's disease.
@en
Protein aggregates in Huntington's disease.
@nl
type
label
Protein aggregates in Huntington's disease.
@en
Protein aggregates in Huntington's disease.
@nl
prefLabel
Protein aggregates in Huntington's disease.
@en
Protein aggregates in Huntington's disease.
@nl
P2860
P1476
Protein aggregates in Huntington's disease
@en
P2093
Steven Finkbeiner
P2860
P356
10.1016/J.EXPNEUROL.2011.12.013
P407
P577
2011-12-19T00:00:00Z