Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species
about
TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.Autophagosome dynamics in neurodegeneration at a glanceLegal but lethal: functional protein aggregation at the verge of toxicityParkinson Disease-linked Vps35 R524W Mutation Impairs the Endosomal Association of Retromer and Induces α-Synuclein AggregationLoss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin-2 to induce motor neuron dysfunction and cell deathDistinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusionsProtein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia ContinuumFormation and spreading of TDP-43 aggregates in cultured neuronal and glial cells demonstrated by time-lapse imaging.Drosophila lines with mutant and wild type human TDP-43 replacing the endogenous gene reveals phosphorylation and ubiquitination in mutant lines in the absence of viability or lifespan defects.p62 plays a protective role in the autophagic degradation of polyglutamine protein oligomers in polyglutamine disease model flies.UBE2E ubiquitin-conjugating enzymes and ubiquitin isopeptidase Y regulate TDP-43 protein ubiquitination.Novel mutations support a role for Profilin 1 in the pathogenesis of ALS.Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways.PABPN1 suppresses TDP-43 toxicity in ALS disease models.Activation of ER Stress and Autophagy Induced by TDP-43 A315T as Pathogenic Mechanism and the Corresponding Histological Changes in Skin as Potential Biomarker for ALS with the MutationAltered localization and functionality of TAR DNA Binding Protein 43 (TDP-43) in niemann- pick disease type CLoss of RAD-23 Protects Against Models of Motor Neuron Disease by Enhancing Mutant Protein ClearanceTDP-43 or FUS-induced misfolded human wild-type SOD1 can propagate intercellularly in a prion-like fashionDefective recognition of LC3B by mutant SQSTM1/p62 implicates impairment of autophagy as a pathogenic mechanism in ALS-FTLD.An insoluble frontotemporal lobar degeneration-associated TDP-43 C-terminal fragment causes neurodegeneration and hippocampus pathology in transgenic miceFunctional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43.Autophagy in neuronal cells: general principles and physiological and pathological functions.Prion degradation pathways: Potential for therapeutic intervention.Modelling FUS Mislocalisation in an In Vitro Model of Innervated Human Muscle.Identification of biological pathways regulated by PGRN and GRN peptide treatments using transcriptome analysis.Mahogunin Ring Finger-1 (MGRN1), a Multifaceted Ubiquitin Ligase: Recent Unraveling of Neurobiological Mechanisms.Exosome secretion is a key pathway for clearance of pathological TDP-43.Stress granules at the intersection of autophagy and ALS.The cleavage pattern of TDP-43 determines its rate of clearance and cytotoxicity.TDP-43/FUS in motor neuron disease: Complexity and challenges.Autophagy and Its Impact on Neurodegenerative Diseases: New Roles for TDP-43 and C9orf72.TDP-43 in the spectrum of MND-FTLD pathologies.The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosisProteostasis disturbance in amyotrophic lateral sclerosis.Clusterin protects neurons against intracellular proteotoxicity.Endocytosis regulates TDP-43 toxicity and turnover.Is amyotrophic lateral sclerosis/frontotemporal dementia an autophagy disease?Ubiquitin-Binding Protein CG5445 Suppresses Aggregation and Cytotoxicity of Amyotrophic Lateral Sclerosis-linked TDP-43 in Drosophila.The Effect of Different Types of Nanoparticles on FUS and TDP-43 Solubility and Subcellular Localization.Inhibition of Drp1/Fis1 interaction slows progression of amyotrophic lateral sclerosis.
P2860
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P2860
Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species
description
2014 nî lūn-bûn
@nan
2014 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2014 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2014年の論文
@ja
2014年論文
@yue
2014年論文
@zh-hant
2014年論文
@zh-hk
2014年論文
@zh-mo
2014年論文
@zh-tw
2014年论文
@wuu
name
Differential roles of the ubiq ...... and aggregated TDP-43 species
@ast
Differential roles of the ubiq ...... and aggregated TDP-43 species
@en
type
label
Differential roles of the ubiq ...... and aggregated TDP-43 species
@ast
Differential roles of the ubiq ...... and aggregated TDP-43 species
@en
prefLabel
Differential roles of the ubiq ...... and aggregated TDP-43 species
@ast
Differential roles of the ubiq ...... and aggregated TDP-43 species
@en
P2093
P2860
P50
P356
P1476
Differential roles of the ubiq ...... and aggregated TDP-43 species
@en
P2093
Agnes L Nishimura
David C Rubinsztein
Han-Jou Chen
Hazel Urwin
Jacqueline C Mitchell
Valentina Sardone
P2860
P304
P356
10.1242/JCS.140087
P407
P577
2014-01-14T00:00:00Z