Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43.
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The extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivoA novel amyotrophic lateral sclerosis mutation in OPTN induces ER stress and Golgi fragmentation in vitro.Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD.Motor neuron vulnerability and resistance in amyotrophic lateral sclerosis.Augmented quantal release of acetylcholine at the vertebrate neuromuscular junction following tdp-43 depletion.A novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defectsActivation of HIPK2 Promotes ER Stress-Mediated Neurodegeneration in Amyotrophic Lateral Sclerosis.An insoluble frontotemporal lobar degeneration-associated TDP-43 C-terminal fragment causes neurodegeneration and hippocampus pathology in transgenic miceProgression of motor neuron disease is accelerated and the ability to recover is compromised with advanced age in rNLS8 miceEarly Cognitive/Social Deficits and Late Motor Phenotype in Conditional Wild-Type TDP-43 Transgenic Mice.Tdp-43 cryptic exons are highly variable between cell types.Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice.Prions and Protein Assemblies that Convey Biological Information in Health and Disease.Nuclear trafficking in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.Prion-like propagation as a pathogenic principle in frontotemporal dementia.TDP-43/FUS in motor neuron disease: Complexity and challenges.ALS/FTLD: experimental models and reality.Neuron loss and degeneration in the progression of TDP-43 in frontotemporal lobar degeneration.Acetylation-induced TDP-43 pathology is suppressed by an HSF1-dependent chaperone program.Inflammation Induces TDP-43 Mislocalization and Aggregation.TDP-43 loss of function inhibits endosomal trafficking and alters trophic signaling in neurons.Are Astrocytes the Predominant Cell Type for Activation of Nrf2 in Aging and Neurodegeneration?Practical considerations for choosing a mouse model of Alzheimer's disease.Increased cytoplasmic TDP-43 reduces global protein synthesis by interacting with RACK1 on polyribosomes.Depletion of TDP-43 decreases fibril and plaque β-amyloid and exacerbates neurodegeneration in an Alzheimer's mouse model.Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice.Frontotemporal dementia: latest evidence and clinical implications.Robustness and Vulnerability of the Autoregulatory System That Maintains Nuclear TDP-43 Levels: A Trade-off Hypothesis for ALS Pathology Based on in Silico Data.Microglia-mediated recovery from ALS-relevant motor neuron degeneration in a mouse model of TDP-43 proteinopathy.Overexpression of heat shock factor 1 maintains TAR DNA binding protein 43 solubility via induction of inducible heat shock protein 70 in cultured cells.Elimination of TDP-43 inclusions linked to amyotrophic lateral sclerosis by a misfolding-specific intrabody with dual proteolytic signals.TDP-43 induces p53-mediated cell death of cortical progenitors and immature neurons.Patient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivo
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Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43.
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article científic
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article scientifique
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articolo scientifico
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artigo científico
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bilimsel makale
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scientific article published on 22 July 2015
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Functional recovery in new mou ...... thological cytoplasmic TDP-43.
@en
Functional recovery in new mou ...... thological cytoplasmic TDP-43.
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type
label
Functional recovery in new mou ...... thological cytoplasmic TDP-43.
@en
Functional recovery in new mou ...... thological cytoplasmic TDP-43.
@nl
prefLabel
Functional recovery in new mou ...... thological cytoplasmic TDP-43.
@en
Functional recovery in new mou ...... thological cytoplasmic TDP-43.
@nl
P2093
P2860
P1476
Functional recovery in new mou ...... thological cytoplasmic TDP-43.
@en
P2093
Adam K Walker
Allen Zheng
Guanghui Ge
Kalyan Tripathy
Krista J Spiller
Linda K Kwong
Melissa Zhou
Virginia M-Y Lee
P2860
P2888
P304
P356
10.1007/S00401-015-1460-X
P577
2015-07-22T00:00:00Z