Peripheral administration of SOD1 aggregates does not transmit pathogenic aggregation to the CNS of SOD1 transgenic mice [PDF]
Acta Neuropathologica Communications, 2021 AbstractThe deposition of aggregated proteins is a common neuropathological denominator for neurodegenerative disorders. Experimental evidence suggests that disease propagation involves prion-like mechanisms that cause the spreading of template-directed aggregation of specific disease-associated proteins.
Isil Keskin +6 more
openaire +5 more sources
Examination of SOD1 aggregation modulators and their effect on SOD1 enzymatic activity as a proxy for potential toxicity [PDF]
The FASEB Journal, 2020 Small-molecule inhibitors of abnormal protein self-assembly are promising candidates for developing therapy against proteinopathies. Such compounds have been examined primarily as inhibitors of amyloid β-protein (Aβ), whereas testing of inhibitors of other amyloidogenic proteins has lagged behind. An important issue with screening compound libraries is
Malik, Ravinder +5 more
openaire +5 more sources
Inhibition of TXNRD or SOD1 overcomes NRF2-mediated resistance to β-lapachone [PDF]
, 2020 Alterations in the NRF2/KEAP1 pathway result in the constitutive activation of NRF2, leading to the aberrant induction of antioxidant and detoxification enzymes, including NQO1.
Boothman, David A. +6 more
core +1 more source
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
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
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
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
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
Brain Sciences, 2023 Mutations in superoxide dismutase 1 (SOD1) result in misfolding and aggregation of the protein, causing neurodegenerative amyotrophic lateral sclerosis (ALS).
Sharad Kumar Suthar, Sang-Yoon Lee
doaj +1 more source
Letter to the editor: autoimmune pathogenic mechanisms in amyotrophic lateral sclerosis [PDF]
, 2018 The innate immune system may affect the function and survival of motor neurons in ALS by at least three mechanisms. First, there is evidence to suggest that aggregates of mutant SOD1—which is derived from microglial and astroglial cells—activate ...
de Vincentiis, M. +5 more
core +1 more source