SOD1-Derlin-1 inhibitor-2 (compound 56-59) is an inhibitor of SOD1-Derlin-1 interaction. SOD1-Derlin-1 inhibitor-2 attenuates the interactions between Derlin-1 and SOD1mut. SOD1-Derlin-1 inhibitor-2 can be used for the research of amyotrophic lateral sclerosis (ALS).In Vitro:SOD1-Derlin-1 inhibitor-2 (compound 56-59; 0.1-100 μM) inhibits the SOD1-Derlin-1 interaction in a dose-dependent manner.In Vivo:SOD1-Derlin-1 inhibitor-2 (compound 56-59; i.c.v.; 3?mM, flow rate 0.15?μL/h) ameliorates ALS pathology in ALS model mice.