... in a cell model of ALS induced by expressing mutant SOD1 (SOD1 A4V ). By monitoring the distribution of Ub in cells expressing SOD1 A4V , we show that Ub is present at the earliest stages of SOD1 A4V aggregation, and that cells containing SOD1 A4V aggregates have greater ubiquitin-proteasome system (UPS...

... disease amyotrophic lateral sclerosis (ALS). Over 150 mutations within the gene encoding superoxide dismutase 1 ( SOD1 ) have been implicated in ALS, but why such mutations lead to ALS-associated cellular dysfunction is unclear. In this study, we identify how ALS-linked SOD1 mutations lead to changes...

... of proteins activate autophagy ( Tyedmers et al., 2010 ). The autophagy-lysosomal degradation machinery can also, to some extent, clear protein aggregates ( Korolchuk et al., 2010 ). Amyotrophic lateral sclerosis Huntington disease Huntingtin Optineurin Phosphorylation SOD1 TBK1 Ubiquitin...