Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS.
about
BAG3 mediates chaperone-based aggresome-targeting and selective autophagy of misfolded proteinsPrion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cellsUbiquitin-Synaptobrevin Fusion Protein Causes Degeneration of Presynaptic Motor Terminals in MiceThe nucleotide exchange factors of Hsp70 molecular chaperonesRegulation of protein quality control by UBE4B and LSD1 through p53-mediated transcriptionRNA-Seq profiling of spinal cord motor neurons from a presymptomatic SOD1 ALS mouseHSPA8/HSC70 chaperone protein: structure, function, and chemical targeting.Molecular chaperone Hsp110 rescues a vesicle transport defect produced by an ALS-associated mutant SOD1 protein in squid axoplasm.Metal-free ALS variants of dimeric human Cu,Zn-superoxide dismutase have enhanced populations of monomeric speciesTransfer of pathogenic and nonpathogenic cytosolic proteins between spinal cord motor neurons in vivo in chimeric miceThe Hsp70/Hsp90 Chaperone Machinery in Neurodegenerative DiseasesIdentification of compounds protective against G93A-SOD1 toxicity for the treatment of amyotrophic lateral sclerosis.Production of RNA for transcriptomic analysis from mouse spinal cord motor neuron cell bodies by laser capture microdissection.Absence of lipofuscin in motor neurons of SOD1-linked ALS mice.Designing phenotyping studies for genetically engineered miceExposure of hydrophobic surfaces initiates aggregation of diverse ALS-causing superoxide dismutase-1 mutants.Cellular strategies of protein quality control.Metazoan Hsp70 machines use Hsp110 to power protein disaggregationHuntingtin fragments and SOD1 mutants form soluble oligomers in the cellAggregation modulating elements in mutant human superoxide dismutase 1.Dysregulation of the proteasome increases the toxicity of ALS-linked mutant SOD1.Zinc: indications in brain disorders.Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunitDecreased stability and increased formation of soluble aggregates by immature superoxide dismutase do not account for disease severity in ALS.Selective degeneration of a physiological subtype of spinal motor neuron in mice with SOD1-linked ALSSpatial quality control bypasses cell-based limitations on proteostasis to promote prion curing.TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signalingThe structural biochemistry of the superoxide dismutases.Proteins that bind to misfolded mutant superoxide dismutase-1 in spinal cords from transgenic amyotrophic lateral sclerosis (ALS) model mice.Direct and indirect mechanisms for wild-type SOD1 to enhance the toxicity of mutant SOD1 in bigenic transgenic mice.Analysis of mutant SOD1 electrophoretic mobility by Blue Native gel electrophoresis; evidence for soluble multimeric assembliesMacrophage migration inhibitory factor as a chaperone inhibiting accumulation of misfolded SOD1.Structural and kinetic analysis of protein-aggregate strains in vivo using binary epitope mappingSuperoxide dismutase 1 encoding mutations linked to ALS adopts a spectrum of misfolded statesThermodynamics of protein destabilization in live cellsAmyotrophic lateral sclerosis: update and new developments.Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathwaysThe small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro.Establishing a novel C. elegans model to investigate the role of autophagy in amyotrophic lateral sclerosis.Extended survival of misfolded G85R SOD1-linked ALS mice by transgenic expression of chaperone Hsp110
P2860
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P2860
Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS.
description
2009 nî lūn-bûn
@nan
2009 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2009 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2009年の論文
@ja
2009年学术文章
@wuu
2009年学术文章
@zh-cn
2009年学术文章
@zh-hans
2009年学术文章
@zh-my
2009年学术文章
@zh-sg
2009年學術文章
@yue
name
Progressive aggregation despit ...... nsgenic mice that develop ALS.
@ast
Progressive aggregation despit ...... nsgenic mice that develop ALS.
@en
type
label
Progressive aggregation despit ...... nsgenic mice that develop ALS.
@ast
Progressive aggregation despit ...... nsgenic mice that develop ALS.
@en
prefLabel
Progressive aggregation despit ...... nsgenic mice that develop ALS.
@ast
Progressive aggregation despit ...... nsgenic mice that develop ALS.
@en
P2093
P2860
P921
P356
P1476
Progressive aggregation despit ...... nsgenic mice that develop ALS.
@en
P2093
Caroline J Zeiss
D Thomas Rutkowski
Diego J Rodriguez-Gil
George W Farr
Jean H Wilson
John R Yates
Krystyna Furtak
Mel B Feany
Randal J Kaufman
P2860
P304
P356
10.1073/PNAS.0813045106
P407
P577
2009-01-26T00:00:00Z