Derlin-1 overexpression ameliorates mutant SOD1-induced endoplasmic reticulum stress by reducing mutant SOD1 accumulation

▶ Exogenous Derlin-1 reduced mutant SOD1-induced cell toxicity by suppressing ER stress. ▶ Derlin-1 resulted in a decrease in the expression of mutant SOD1 in cells. ▶ Reduced SOD1 protein expression was observed in the microsomal fraction. ▶ Derlin-1 regulates the turn over of SOD1 by promoting degradation of SOD1 protein.