Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse.
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From animal models to human disease: a genetic approach for personalized medicine in ALSMechanisms of mutant SOD1 induced mitochondrial toxicity in amyotrophic lateral sclerosisCharacterization of a Covalent Polysulfane Bridge in Copper−Zinc Superoxide Dismutase,Structures of mouse SOD1 and human/mouse SOD1 chimerasThe complex molecular biology of amyotrophic lateral sclerosis (ALS)Intermolecular transmission of superoxide dismutase 1 misfolding in living cells.Palmitoylation of superoxide dismutase 1 (SOD1) is increased for familial amyotrophic lateral sclerosis-linked SOD1 mutantsWild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS.Conformational specificity of the C4F6 SOD1 antibody; low frequency of reactivity in sporadic ALS cases.Mutant SOD1 knockdown in all cell types ameliorates disease in G85R SOD1 mice with a limited additional effect over knockdown restricted to motor neuronsDirect observation of defects and increased ion permeability of a membrane induced by structurally disordered Cu/Zn-superoxide dismutase aggregates.Cu,Zn-superoxide dismutase increases toxicity of mutant and zinc-deficient superoxide dismutase by enhancing protein stability.Huntingtin fragments and SOD1 mutants form soluble oligomers in the cellAn examination of wild-type SOD1 in modulating the toxicity and aggregation of ALS-associated mutant SOD1The unfolded protein response in familial amyotrophic lateral sclerosis.Genotype-property patient-phenotype relations suggest that proteome exhaustion can cause amyotrophic lateral sclerosis.Direct and indirect mechanisms for wild-type SOD1 to enhance the toxicity of mutant SOD1 in bigenic transgenic mice.A fruitful endeavor: modeling ALS in the fruit flyModel systems of motor neuron diseases as a platform for studying pathogenic mechanisms and searching for therapeutic agents.Loss of RAD-23 Protects Against Models of Motor Neuron Disease by Enhancing Mutant Protein ClearanceMotor neuron-immune interactions: the vicious circle of ALS.An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man studyIs SOD1 loss of function involved in amyotrophic lateral sclerosis?The effect of mutant SOD1 dismutase activity on non-cell autonomous degeneration in familial amyotrophic lateral sclerosisAn enhanced integrated stress response ameliorates mutant SOD1-induced ALS.SOD1 and TDP-43 animal models of amyotrophic lateral sclerosis: recent advances in understanding disease toward the development of clinical treatments.Emerging drugs for amyotrophic lateral sclerosis.Generalization of the prion hypothesis to other neurodegenerative diseases: an imperfect fit.An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis.Prion-like activity of Cu/Zn superoxide dismutase: implications for amyotrophic lateral sclerosis.The interplay between metabolic homeostasis and neurodegeneration: insights into the neurometabolic nature of amyotrophic lateral sclerosis.Simple animal models for amyotrophic lateral sclerosis drug discovery.Mutant superoxide dismutase-1 indistinguishable from wild-type causes ALS.Antisense Oligonucleotides: Translation from Mouse Models to Human Neurodegenerative Diseases.System xC- is a mediator of microglial function and its deletion slows symptoms in amyotrophic lateral sclerosis mice.Features of wild-type human SOD1 limit interactions with misfolded aggregates of mouse G86R Sod1.S-acylation of SOD1, CCS, and a stable SOD1-CCS heterodimer in human spinal cords from ALS and non-ALS subjects.Cellular toxicity of mutant SOD1 protein is linked to an easily soluble, non-aggregated form in vitro.Prognostic role of "prion-like propagation" in SOD1-linked familial ALS: an alternative view.Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice.
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Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse.
description
article científic
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article scientifique
@fr
articolo scientifico
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artigo científico
@pt
bilimsel makale
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scientific article published on 19 February 2009
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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
Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse.
@en
Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse.
@nl
type
label
Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse.
@en
Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse.
@nl
prefLabel
Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse.
@en
Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse.
@nl
P2093
P2860
P356
P1476
Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse
@en
P2093
Gabriella Grisotti
Lijun Wang
Raymond P Roos
P2860
P304
P356
10.1093/HMG/DDP085
P577
2009-02-19T00:00:00Z