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Dysregulated axonal RNA translation in amyotrophic lateral sclerosisImpaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron DegenerationNeuronal response in Alzheimer's and Parkinson's disease: the effect of toxic proteins on intracellular pathwaysUBA1: At the Crossroads of Ubiquitin Homeostasis and NeurodegenerationAutophagy and Neurodegeneration: Insights from a Cultured Cell Model of ALSBrain-Specific Cytoskeletal Damage Markers in Cerebrospinal Fluid: Is There a Common Pattern between Amyotrophic Lateral Sclerosis and Primary Progressive Multiple Sclerosis?Oxidative Stress in Neurodegenerative DiseasesPathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disordersAmyotrophic lateral sclerosis-linked mutant VAPB inclusions do not interfere with protein degradation pathways or intracellular transport in a cultured cell modelCalcium dysregulation links ALS defective proteins and motor neuron selective vulnerabilityPrion-like Mechanism in Amyotrophic Lateral Sclerosis: are Protein Aggregates the Key?Cryptic Amyloidogenic Elements in the 3' UTRs of Neurofilament Genes Trigger Axonal NeuropathyALS as a distal axonopathy: molecular mechanisms affecting neuromuscular junction stability in the presymptomatic stages of the diseaseSolid-state NMR studies of metal-free SOD1 fibrillar structures.Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells.Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?The Hsp70/Hsp90 Chaperone Machinery in Neurodegenerative DiseasesBlood biomarkers for amyotrophic lateral sclerosis: myth or reality?Formation and spreading of TDP-43 aggregates in cultured neuronal and glial cells demonstrated by time-lapse imaging.Amyotrophic lateral sclerosis patient iPSC-derived astrocytes impair autophagy via non-cell autonomous mechanisms.MTOR-independent, autophagic enhancer trehalose prolongs motor neuron survival and ameliorates the autophagic flux defect in a mouse model of amyotrophic lateral sclerosis.TDP-43 toxicity proceeds via calcium dysregulation and necrosis in aging Caenorhabditis elegans motor neurons.The ubiquitin C-terminal hydrolase L1 (UCH-L1) C terminus plays a key role in protein stability, but its farnesylation is not required for membrane association in primary neurons.PPAR gamma activation is neuroprotective in a Drosophila model of ALS based on TDP-43.C9ORF72 GGGGCC Expanded Repeats Produce Splicing Dysregulation which Correlates with Disease Severity in Amyotrophic Lateral SclerosisHuman immunodeficiency virus-associated dementia: a link between accumulation of viral proteins and neuronal degeneration.ALS mutations in TLS/FUS disrupt target gene expression.Golgi fragmentation in amyotrophic lateral sclerosis, an overview of possible triggers and consequences.TDP-43 protein variants as biomarkers in amyotrophic lateral sclerosis.Macrophage-mediated inflammation and glial response in the skeletal muscle of a rat model of familial amyotrophic lateral sclerosis (ALS)Developmentally Regulated RNA-binding Protein 1 (Drb1)/RNA-binding Motif Protein 45 (RBM45), a Nuclear-Cytoplasmic Trafficking Protein, Forms TAR DNA-binding Protein 43 (TDP-43)-mediated Cytoplasmic AggregatesComparative interactomics analysis of different ALS-associated proteins identifies converging molecular pathwaysLentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteinsThe role of gigaxonin in the degradation of the glial-specific intermediate filament protein GFAP.Dendritic retraction, but not atrophy, is consistent in amyotrophic lateral sclerosis-comparison between Onuf's neurons and other sacral motor neurons.RNA metabolism in ALS: when normal processes become pathological.The role of FUS gene variants in neurodegenerative diseases.RNA-binding proteins associated molecular mechanisms of motor neuron degeneration pathogenesis.MicroRNAs as potential circulating biomarkers for amyotrophic lateral sclerosis.Stem cells for amyotrophic lateral sclerosis modeling and therapy: myth or fact?
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P2860
description
article científic
@ca
article scientifique
@fr
articol științific
@ro
articolo scientifico
@it
artigo científico
@gl
artigo científico
@pt
artigo científico
@pt-br
artikel ilmiah
@id
artikull shkencor
@sq
artículo científico
@es
name
Protein aggregation in amyotrophic lateral sclerosis.
@en
type
label
Protein aggregation in amyotrophic lateral sclerosis.
@en
prefLabel
Protein aggregation in amyotrophic lateral sclerosis.
@en
P2860
P50
P1476
Protein aggregation in amyotrophic lateral sclerosis
@en
P2093
Anna M Blokhuis
Leonard H van den Berg
P2860
P2888
P304
P356
10.1007/S00401-013-1125-6
P577
2013-05-15T00:00:00Z
P5875
P6179
1013099122