Misfolded SOD1 pathology in sporadic Amyotrophic Lateral Sclerosis (original) (raw)
Lack of evidence of monomer/misfolded superoxide dismutase-1 in sporadic amyotrophic lateral sclerosis
Erik Pioro
Annals of Neurology, 2009
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Identification of a misfolded region in superoxide dismutase 1 that is exposed in amyotrophic lateral sclerosis
Melissa Rotunno
The Journal of biological chemistry, 2014
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Mutant superoxide dismutase-1 indistinguishable from wild-type causes ALS
D. Ronchi
Human Molecular Genetics, 2012
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An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1
Stefania Guareschi, Cristina Cereda
Proceedings of the National Academy of Sciences, 2012
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SOD1, from Bench to Bed: New Role for the Oldest Protein Implicated in ALS
Mauro Ceroni
Update on Amyotrophic Lateral Sclerosis, 2016
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Detergent-insoluble Aggregates Associated with Amyotrophic Lateral Sclerosis in Transgenic Mice Contain Primarily Full-length, Unmodified Superoxide Dismutase-1
Edith Gralla
Journal of Biological Chemistry, 2008
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Strategies for stabilizing superoxide dismutase (SOD1), the protein destabilized in the most common form of familial amyotrophic lateral sclerosis
Gregory Petsko
Proceedings of the National Academy of Sciences, 2010
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A Novel Variant in Superoxide Dismutase 1 Gene (p.V119M) in Als Patients with Pure Lower Motor Neuron Presentation
CLAUDIA RICCI
Genes
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Investigation of some variations of superoxide dismutase gene family in Turkish sporadic amyotrophic lateral sclerosis patients
Ali Sazci
Brain Disorders, 2021
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Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis
Thomas Brännström
Acta Neuropathologica, 2011
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Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity
Michael Guarnieri
Proceedings of the National Academy of Sciences, 1994
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Modification of Superoxide Dismutase 1 (SOD1) Properties by a GFP Tag – Implications for Research into Amyotrophic Lateral Sclerosis (ALS)
Giampietro Schiavo
PLoS ONE, 2010
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Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models
Heather Stewart
Proceedings of the National Academy of Sciences, 2007
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Metal-free superoxide dismutase forms soluble oligomers under physiological conditions: A possible general mechanism for familial ALS
lucia durazo
Proceedings of The National Academy of Sciences, 2007
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Insoluble Mutant SOD1 Is Partly Oligoubiquitinated in Amyotrophic Lateral Sclerosis Mice
M Basso
Journal of Biological Chemistry, 2006
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Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS
Yuyu Song
Nature Neuroscience, 2010
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Superoxide dismutase gene mutations in Italian patients with familial and sporadic amyotrophic lateral sclerosis: identification of three novel missense mutations
B. Castellotti
Neuromuscular Disorders, 2001
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Superoxide dismutase-1 and other proteins in inclusions from transgenic amyotrophic lateral sclerosis model mice: SOD1 inclusions in ALS transgenic mice
Vaibhav Srivastava
Journal of Neurochemistry, 2010
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Identifying, Targeting, and Exploiting a Common Misfolded, Toxic Conformation of SOD1 in ALS: A Dissertation
Melissa Rotunno
2015
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Effects of Cellular Pathway Disturbances on Misfolded Superoxide Dismutase-1 in Fibroblasts Derived from ALS Patients
Anna Birve
PloS one, 2016
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Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS
Deborah Cooper
Proceedings of the National Academy of Sciences, 2012
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Misfolded SOD1 Accumulation and Mitochondrial Association Contribute to the Selective Vulnerability of Motor Neurons in Familial ALS: Correlation to Human Disease
Adrian Israelson
ACS Chem. Neurosci., 2017
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SOD1-positive aggregate accumulation in the CNS predicts slower disease progression and increased longevity in a mutant SOD1 mouse model of ALS
Fernando Vieira
Scientific Reports, 2019
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Novel SOD1 mutation p.V31A identified with a slowly progressive form of amyotrophic lateral sclerosis
Philippe Corcia
Neurobiology of aging, 2014
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A human-derived antibody targets misfolded SOD1 and ameliorates motor symptoms in mouse models of amyotrophic lateral sclerosis
Fernando Vieira
2018
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An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis
Melissa Rotunno
Frontiers in Cellular Neuroscience, 2013
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