Microglial HVCN1 Deficiency Improves Movement and Survival of SOD1 G93A ALS Mice by Enhancing Microglial Migration and Neuroprotection [PDF]
Advanced Science, EarlyView.Hydrogen voltage gated channel 1 (HVCN1) is upregulated in microglia of both ALS patients and its mouse model. HVCN1 deficiency enhances microglial migration via suppressing Akt signaling, promotes neurotrophic capacity and motor function, and prolongs survival of the SOD1G93A ALS mice. This study identifies HVCN1 as a novel, promising druggable target
Fan Wang +16 more
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Motor Neuron Generation from iPSCs from Identical Twins Discordant for Amyotrophic Lateral Sclerosis
Cells, 2020 Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disorder characterized by the loss of the upper and lower motor neurons. Approximately 10% of cases are caused by specific mutations in known genes, with the remaining cases having no ...
Emily R. Seminary +10 more
doaj +1 more source
Copper Ions and Parkinson’s Disease: Why Is Homeostasis So Relevant?
Biomolecules, 2020 The involvement of copper in numerous physiological processes makes this metal ion essential for human life. Alterations in copper homeostasis might have deleterious consequences, and several neurodegenerative disorders, including Parkinson’s ...
Marco Bisaglia, Luigi Bubacco
doaj +1 more source
Biomarkers and molecular mechanisms of Amyotrophic Lateral Sclerosis
AIMS Neuroscience, 2022 Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease in adults involving non-demyelinating motor disorders. About 90% of ALS cases are sporadic, while 10–12% of cases are due to some genetic reasons.
Ashok Chakraborty, Anil Diwan
doaj +1 more source
Amyotrophic lateral sclerosis and intestinal microbiota—toward establishing cause and effect
Gut Microbes, 2020 The intestinal microbiota may be involved, through metabolic gut–brain interactions, in a variety of neurological conditions. In this addendum, we summarize the findings of our recent study investigating the potentially modulatory influence of the ...
Marc Gotkine +2 more
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Molecules, 2020 Zinc (II) ions (hereafter simplified as zinc) are important for the structural and functional activity of many proteins. For Cu, Zn superoxide dismutase (Sod1), zinc stabilizes the native structure of each Sod1 monomer, promotes homo-dimerization and ...
Stefanie D. Boyd +5 more
doaj +1 more source
Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials [PDF]
, 2006 Recent studies suggest that the toxicity of familial amyotrophic lateral sclerosis mutant Cu, Zn superoxide dismutase (SOD1) arises from its selective recruitment to mitochondria. Here we demonstrate that each of 12 different familial ALS-mutant SOD1s
Butler Gralla, Edith +8 more
core +2 more sources
Single copy/knock-in models of ALS SOD1 in C. elegans suggest loss and gain of function have different contributions to cholinergic and glutamatergic neurodegeneration [PDF]
, 2018 Mutations in Cu/Zn superoxide dismutase 1 (SOD1) lead to Amyotrophic Lateral Sclerosis (ALS), a neurodegenerative disease that disproportionately affects glutamatergic and cholinergic motor neurons.
Baskoylu, Saba N +10 more
core +3 more sources
Screening for Novel LOX and SOD1 Variants in Keratoconus Patients from Brazil
Journal of Ophthalmic & Vision Research, 2020 Purpose: To investigate the presence of the variants of lysyl oxygenase (LOX) and superoxide dismutase 1 (SOD1) genes in Brazilian patients with advanced keratoconus.
Diego Nery Benevides Gadelha +9 more
doaj +1 more source
Viral delivery of antioxidant genes as a therapeutic strategy in experimental models of amyotrophic lateral sclerosis. [PDF]
, 2013 Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder with no effective treatment to date. Despite its multi-factorial aetiology, oxidative stress is hypothesized to be one of the key pathogenic mechanisms.
Azzouz, M +6 more
core +1 more source