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   Articles with "hsod1" as a keyword



Mutant superoxide dismutase aggregates from human spinal cord transmit amyotrophic lateral sclerosis

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recommendations! Published in 2018 at "Acta Neuropathologica"

DOI: 10.1007/s00401-018-1915-y

Abstract: Motor neurons containing aggregates of superoxide dismutase 1 (SOD1) are hallmarks of amyotrophic lateral sclerosis (ALS) caused by mutations in the gene encoding SOD1. We have previously reported that two strains of mutant human (h)… read more here.

Keywords: seed; amyotrophic lateral; hsod1; superoxide dismutase ... See more keywords

α-Lipoic acid attenuates oxidative stress and neurotoxicity via the ERK/Akt-dependent pathway in the mutant hSOD1 related Drosophila model and the NSC34 cell line of amyotrophic lateral sclerosis

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recommendations! Published in 2018 at "Brain Research Bulletin"

DOI: 10.1016/j.brainresbull.2018.05.019

Abstract: Amyotrophic lateral sclerosis (ALS) is a degenerative disease with a progressive loss of motor neurons in the central nervous system (CNS). However, there are unsolved problems with the therapies for this disease. α-Lipoic acid (LA)… read more here.

Keywords: antioxidant; erk akt; pathway; hsod1 ... See more keywords

Structure and Molecular Basis for Inhibiting Human SOD1 Aggregation by a Promising Decan Derivative Modulator: A Potential Therapeutic Strategy for Treating Amyotrophic Lateral Sclerosis (ALS).

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recommendations! Published in 2025 at "ACS chemical neuroscience"

DOI: 10.1021/acschemneuro.5c00168

Abstract: Misfolding and aggregation of the human superoxide dismutase (hSOD1) protein are pathological hallmarks of amyotrophic lateral sclerosis (ALS), a motor neuron disease. The structural stabilization and enzymatic activation of hSOD1 occur upon binding to Cu/Zn… read more here.

Keywords: hsod1; structure; sclerosis als; aggregation ... See more keywords

Theme 6 Tissue Biomarkers

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recommendations! Published in 2024 at "Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration"

DOI: 10.1080/21678421.2024.2403303

Abstract: Background: Mutations in the human superoxide dismutase-1 (hSOD1) gene are a main causative factor in familial ALS and can even be identified in sporadic ALS cases. Despite this, questions remain regarding the pathogenesis but is… read more here.

Keywords: hsod1; mice; theme tissue; tissue biomarkers ... See more keywords

Aggregate-selective antibody attenuates seeded aggregation but not spontaneously evolving disease in SOD1 ALS model mice

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recommendations! Published in 2020 at "Acta Neuropathologica Communications"

DOI: 10.1186/s40478-020-01032-2

Abstract: Increasing evidence suggests that propagation of the motor neuron disease amyotrophic lateral sclerosis (ALS) involves the pathogenic aggregation of disease-associated proteins that spread in a prion-like manner. We have identified two aggregate strains of human… read more here.

Keywords: seed; aggregation; disease; aggregate selective ... See more keywords