Abstract: Disclosed are a pharmaceutical composition for treatment of neurodegenerative diseases or diseases caused by abnormality of RNA binding protein and applications thereof, in particular the application in the treatment of ALS. The pharmaceutical composition can significantly enhance the dynamic performance of stress particles containing RNA binding proteins such as hnRNP A1 and TDP-43 proteins; influences the interaction between the RNA binding proteins and other poly ADP ribosylation modified proteins or other PAR binding proteins; influences the subcellular localization and stress response of RNA binding proteins; influences the liquid-liquid phase separation and aggregation tendency of RNA binding proteins; influences the co-phase separation between RNA binding proteins; influences the interaction of RNA binding proteins in cells; and has a significant inhibitory effect on neurotoxicity caused by RNA binding proteins.