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UnknownSOD1A4V aggregation alters ubiquitin homeostasis in a cell model of ALS.
A hallmark of amyotrophic lateral sclerosis (ALS) pathology is the accumulation of ubiquitylated protein inclusions within motor neurons. Recent studies suggest the sequestration of ubiquitin (Ub) into inclusions reduces the availability of free Ub, which is essential for cellular function and survi...
S-EPMC6919637 | biostudies-literature
UnknownClusterin interacts with Paclitaxel and confer Paclitaxel resistance in ovarian cancer.
Optimal debulking followed by chemotherapy is the standard treatment of managing late-stage ovarian cancer, but chemoresistance is still a major problem. In this study, we compared expression profiles of primary tumor tissue from five long-term (>8 years) and five short-term (<2 years) ovarian cance...
S-EPMC2517641 | biostudies-literature
UnknownA polytherapy approach demonstrates therapeutic efficacy for the treatment of SOD1 associated amyotrophic lateral sclerosis.

Background

SOD1 mutations are a significant contributor of familial amyotrophic lateral sclerosis (ALS) cases. SOD1 mutations increase the propensity for the protein to misfold and aggregate into insoluble proteinaceous deposits within motor neurons and neighbouring cells. The small molecule...
S-EPMC12018197 | biostudies-literature
UnknownThe Ubiquitin Proteasome System Is a Key Regulator of Pluripotent Stem Cell Survival and Motor Neuron Differentiation.
The ubiquitin proteasome system (UPS) plays an important role in regulating numerous cellular processes, and a dysfunctional UPS is thought to contribute to motor neuron disease. Consequently, we sought to map the changing ubiquitome in human iPSCs during their pluripotent stage and following differ...
S-EPMC6627164 | biostudies-literature
UnknownCuATSM improves motor function and extends survival but is not tolerated at a high dose in SOD1G93A mice with a C57BL/6 background.
The synthetic copper-containing compound, CuATSM, has emerged as one of the most promising drug candidates developed for the treatment of amyotrophic lateral sclerosis (ALS). Multiple studies have reported CuATSM treatment provides therapeutic efficacy in various mouse models of ALS without any obse...
S-EPMC8481268 | biostudies-literature
UnknownDevelopment of a targeted BioPROTAC degrader selective for misfolded SOD1.
The accumulation of misfolded proteins underlies a broad range of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). Due to their dynamic nature, these misfolded proteins have proven challenging to target therapeutically. Here, we specifically target misfolded disease variant...
S-EPMC12603324 | biostudies-literature
UnknownPathogenic mutation in the ALS/FTD gene, CCNF, causes elevated Lys48-linked ubiquitylation and defective autophagy.
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are fatal neurodegenerative disorders that have common molecular and pathogenic characteristics, such as aberrant accumulation and ubiquitylation of TDP-43; however, the mechanisms that drive this process remain poorly understood....
S-EPMC11105586 | biostudies-literature
ProteomicsMutations to the ALS/FTD gene CCNF reveals elevated Lys48-linked ubiquitination by the SCF(cyclin F) complex
Approximately 10% of Amyotrophic lateral sclerosis (ALS) cases have a positive family history (familial ALS) and appear clinically indistinguishable from sporadic cases. ALS and frontotemporal dementia (FTD) are fatal neurodegenerative disorders that have common molecular and pathogenic characterist...
ORGANISM(S): Mus musculus (Mouse) 
2017-12-01 | PXD002011 | Pride
Motor neurone disease Als Ccnf Ubiquitin 
ProteomicsEvaluating protein crosslinking as a therapeutic strategy to stabilize SOD1 variants in a mouse model of familial ALS
Mutations in the gene encoding Cu-Zn superoxide dismutase 1 (SOD1) cause a subset of familial amyotrophic lateral sclerosis (fALS) cases. A shared effect of these mutations is that SOD1, which is normally a stable dimer, dissociates into toxic monomers that seed toxic aggregates. Considerable resear...
ORGANISM(S): Mus musculus (Mouse) 
2023-12-17 | PXD047030 | Pride
Cross-linking Pharmacological chaperone Amyotrophic lateral sclerosis (als) Stabilization Sod1 
UnknownEvaluating protein cross-linking as a therapeutic strategy to stabilize SOD1 variants in a mouse model of familial ALS.
Mutations in the gene encoding Cu-Zn superoxide dismutase 1 (SOD1) cause a subset of familial amyotrophic lateral sclerosis (fALS) cases. A shared effect of these mutations is that SOD1, which is normally a stable dimer, dissociates into toxic monomers that seed toxic aggregates. Considerable resear...
S-EPMC10826971 | biostudies-literature
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