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Characterization of Detergent-Insoluble Proteins in ALS Indicates a Causal Link between Nitrative Stress and Aggregation in Pathogenesis
Background Amyotrophic lateral sclerosis (ALS) is a progressive and fatal motor neuron disease, and protein aggregation has been proposed as a possible pathogenetic mechanism. However, the aggregateExpand
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Protein Nitration in a Mouse Model of Familial Amyotrophic Lateral Sclerosis
Multiple mechanisms have been proposed to contribute to amyotrophic lateral sclerosis (ALS) pathogenesis, including oxidative stress. Early evidence of a role for oxidative damage was based on theExpand
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Mutant Copper-Zinc Superoxide Dismutase (SOD1) Induces Protein Secretion Pathway Alterations and Exosome Release in Astrocytes
Background: The mechanism by which astrocytes contribute to disease progression in mutant SOD1 mouse models of ALS is not known. Results: Mutant SOD1 astrocytes release mutant SOD1-containingExpand
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Actin Glutathionylation Increases in Fibroblasts of Patients with Friedreich's Ataxia
Increasing evidence suggests that iron-mediated oxidative stress might underlie the development of neurodegeneration in Friedreich's ataxia (FRDA), an autosomal recessive ataxia caused by decreasedExpand
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Amyotrophic Lateral Sclerosis Multiprotein Biomarkers in Peripheral Blood Mononuclear Cells
Background Amyotrophic lateral sclerosis (ALS) is a fatal progressive motor neuron disease, for which there are still no diagnostic/prognostic test and therapy. Specific molecular biomarkers areExpand
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Extracellular Vesicles and a Novel Form of Communication in the Brain
In numerous neurodegenerative diseases, the interplay between neurons and glia modulates the outcome and progression of pathology. One particularly intriguing mode of interaction between neurons,Expand
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A Novel, Drug-based, Cellular Assay for the Activity of Neurotoxic Mutants of the Prion Protein*
In prion diseases, the infectious isoform of the prion protein (PrPSc) may subvert a normal, physiological activity of the cellular isoform (PrPC). A deletion mutant of the prion protein (Δ105–125)Expand
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Insoluble Mutant SOD1 Is Partly Oligoubiquitinated in Amyotrophic Lateral Sclerosis Mice*
Mutations in the Cu,Zn-superoxide dismutase (SOD1) gene cause a familial form of amyotrophic lateral sclerosis (ALS) through an unknown gain-of-function mechanism. Mutant SOD1 aggregation may be theExpand
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Proteomic analysis of spinal cord of presymptomatic amyotrophic lateral sclerosis G93A SOD1 mouse.
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease, whose primary mechanisms or causes are still not defined and for which no effective treatment is available. We have recentlyExpand
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Synthetic Miniprion PrP106*
Elucidation of structure and biological properties of the prion protein scrapie (PrPSc) is fundamental to an understanding of the mechanism of conformational transition of cellular (PrPC) intoExpand
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