IU1 is widely used in biochemical and pharmaceutical research as a selective inhibitor of the ubiquitin-specific protease 14 (USP14), which is a deubiquitinating enzyme associated with the proteasome. By inhibiting USP14, IU1 enhances the degradation of ubiquitin-tagged proteins, thereby promoting the clearance of misfolded or damaged proteins within cells. This property makes IU1 a valuable tool in studying proteasome function and protein homeostasis. It has shown potential in research related to neurodegenerative diseases, where accumulation of abnormal proteins is a key feature, such as Alzheimer’s, Parkinson’s, and Huntington’s disease. IU1 has been reported to increase the turnover of disease-relevant proteins in cellular and animal models, suggesting its utility in probing therapeutic strategies aimed at boosting proteasomal activity. Additionally, IU1 is used to investigate the role of deubiquitinating enzymes in cancer, as modulating protein stability can influence tumor cell survival and proliferation. Its application remains primarily in preclinical research, where it serves as a chemical probe to understand ubiquitin-proteasome system dynamics and to validate USP14 as a drug target.