Expression of a pathogenic mutation of SOD1 sensitizes aprataxin-deficient cells and mice to oxidative stress and triggers hallmarks of premature ageing [PDF]
Aprataxin (APTX) deficiency causes progressive cerebellar degeneration, ataxia and oculomotor apraxia in man. Cell free assays and crystal structure studies demonstrate a role for APTX in resolving 5'-adenylated nucleic acid breaks, however, APTX ...
Bode, David +9 more
core +1 more source
In this study, a bifunctional anti‐swelling hydrogel system is developed, which encapsulated anti‐senescence drugs quercetin, dasatinib, nucleus pulposus‐derived exosomes (NP‐Exo) to clear senescent nucleus pulposus cells, regulate the inflammatory disc microenvironment, and enhance nucleus pulposus cells activity, ultimately promoting the repair of ...
Songfeng Chen +14 more
wiley +1 more source
Features of wild-type human SOD1 limit interactions with misfolded aggregates of mouse G86R Sod1 [PDF]
AbstractMutations in the gene encoding superoxide dismutase 1 (SOD1) account for about 20% of the cases of familial amyotrophic lateral sclerosis (fALS). It is well established that mutations in SOD1, associated with fALS, heighten the propensity of the protein to misfold and aggregate.
David A. Qualls +6 more
openaire +3 more sources
SOD1 Lysine 123 Acetylation in the Adult Central Nervous System
Superoxide dismutase 1 (SOD1) knockout (Sod1-/-) mice exhibit an accelerated aging phenotype. In humans, SOD1 mutations are linked to familial amyotrophic lateral sclerosis (ALS), and post-translational modification (PTM) of wild-type SOD1 has been ...
Michael Kaliszewski +11 more
doaj +1 more source
Screening of the transcriptional regulatory regions of vascular endothelial growth factor receptor 2 (VEGFR2) in amyotrophic lateral sclerosis [PDF]
Background Vascular endothelial growth factor (VEGF) has neurotrophic activity which is mediated by its main agonist receptor, VEGFR2. Dysregulation of VEGF causes motor neurone degeneration in a mouse model of amyotrophic lateral sclerosis (ALS), and
Brockington, A. +4 more
core +3 more sources
Nanozymes for Non‐Neoplastic Diseases: Catalytic Therapy Redefined
Non‐neoplastic diseases pose a significant health burden. Nanozymes, enzyme‐like nanomaterials, offer promising strategies for regulating oxidative stress and inflammation. This review explores their development, structure–function relationships, and therapeutic potential, with a focus on precision medicine through targeted delivery and multimodal ...
Ling Mei +8 more
wiley +1 more source
SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse [PDF]
Motor axon degeneration is a critical but poorly understood event leading to weakness and muscle atrophy in motor neuron diseases. Here, we investigated oxidative stress-mediated axonal degeneration in mice lacking the antioxidant enzyme, Cu,Zn superoxide dismutase (SOD1). We demonstrate a progressive motor axonopathy in these mice and show that Sod1(-/
Jason M. Hansen +6 more
openaire +3 more sources
Pathological Roles of Wild-Type Cu, Zn-Superoxide Dismutase in Amyotrophic Lateral Sclerosis
Dominant mutations in a Cu, Zn-superoxide dismutase (SOD1) gene cause a familial form of amyotrophic lateral sclerosis (ALS). While it remains controversial how SOD1 mutations lead to onset and progression of the disease, many in vitro and in vivo ...
Yoshiaki Furukawa
doaj +1 more source
Disruptions in metal ion homeostasis have been described in association with amyotrophic lateral sclerosis (ALS) for a number of years but the precise mechanism of involvement is poorly understood.
Megan W Bourassa +5 more
doaj +1 more source
Superoxide Radical Dismutation as New Therapeutic Strategy in Parkinson’s Disease [PDF]
open4siAging is the biggest risk factor for developing many neurodegenerative disorders, including idiopathic Parkinson's disease (PD). PD is still an incurable disorder and the available medications are mainly directed to the treatment of symptoms in ...
core +1 more source

