Lithium facilitates removal of misfolded proteins and attenuated faulty interaction between mutant SOD1 and p-CREB (Ser133) through enhanced autophagy in mutant hSOD1G93A transfected neuronal cell lines
Sign Up to like & getrecommendations! Published in 2019 at "Molecular Biology Reports"
DOI: 10.1007/s11033-019-05071-4
Abstract: Abnormally protein aggregation and deposition are key pathological features of ALS, which may related with dysfunctional cellular autophagy. In the current study, we found that, compared with wtSOD1 cells, serum starvation treatment resulted in significant… read more here.
Keywords: protein; sod1; treatment; mutant sod1 ... See more keywords
Single chain variable fragment antibodies directed against SOD1 ameliorate disease in mutant SOD1 transgenic mice
Sign Up to like & getrecommendations! Published in 2019 at "Neurobiology of Disease"
DOI: 10.1016/j.nbd.2018.08.021
Abstract: Mutations in Cu/Zn superoxide dismutase (SOD1) are the cause of ~20% of cases of familial ALS (FALS), which comprise ~10% of the overall total number of cases of ALS. Mutant (mt) SOD1 is thought to… read more here.
Keywords: sod1; directed sod1; mutant sod1; chain variable ... See more keywords
The UPR-PERK pathway is not a promising therapeutic target for mutant SOD1-induced ALS
Sign Up to like & getrecommendations! Published in 2019 at "Neurobiology of Disease"
DOI: 10.1016/j.nbd.2019.03.024
Abstract: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease, characterized by motor neuron death in the brain and spinal cord. Mutations in the Cu/Zn superoxide dismutase (SOD1) gene account for ~20% of all familial ALS… read more here.
Keywords: mtsod1; upr perk; perk pathway; disease ... See more keywords
The deletion of mutant SOD1 via CRISPR/Cas9/sgRNA prolongs survival in an amyotrophic lateral sclerosis mouse model
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DOI: 10.1038/s41434-019-0116-1
Abstract: The superoxide dismutase 1 (SOD1) mutation is one of the most notable causes of amyotrophic lateral sclerosis (ALS), and modifying the mutant SOD1 gene is the best approach for the treatment of patients with ALS… read more here.
Keywords: crispr; sod1; amyotrophic lateral; mutant sod1 ... See more keywords
Protein disulfide isomerase ERp57 protects early muscle denervation in experimental ALS
Sign Up to like & getrecommendations! Published in 2021 at "Acta Neuropathologica Communications"
DOI: 10.1186/s40478-020-01116-z
Abstract: Amyotrophic lateral sclerosis (ALS) is a progressive fatal neurodegenerative disease that affects motoneurons. Mutations in superoxide dismutase 1 (SOD1) have been described as a causative genetic factor for ALS. Mice overexpressing ALS-linked mutant SOD1 develop… read more here.
Keywords: sod1; disulfide isomerase; protein disulfide; mutant sod1 ... See more keywords
Anti-SOD1 Nanobodies That Stabilize Misfolded SOD1 Proteins Also Promote Neurite Outgrowth in Mutant SOD1 Human Neurons
Sign Up to like & getrecommendations! Published in 2022 at "International Journal of Molecular Sciences"
DOI: 10.3390/ijms232416013
Abstract: ALS-linked mutations induce aberrant conformations within the SOD1 protein that are thought to underlie the pathogenic mechanism of SOD1-mediated ALS. Although clinical trials are underway for gene silencing of SOD1, these approaches reduce both wild-type… read more here.
Keywords: neurite outgrowth; sod1 nanobodies; anti sod1; sod1 ... See more keywords