IU1-248 is a selective inhibitor of USP14, a deubiquitinating enzyme associated with the proteasome. It is primarily used in research settings to study protein degradation pathways, particularly the ubiquitin-proteasome system. By inhibiting USP14, IU1-248 enhances the degradation of ubiquitinated proteins, making it a valuable tool for investigating cellular protein homeostasis. It has been applied in studies related to neurodegenerative diseases, where accumulation of misfolded proteins is a key feature. Researchers use IU1-248 to explore how boosting proteasomal activity can clear toxic protein aggregates, such as tau or alpha-synuclein. It is also used in cancer research to examine how modulating proteasome function affects tumor cell survival and response to other therapies. Due to its ability to fine-tune proteasome activity without fully blocking it, IU1-248 offers a more targeted approach compared to broad proteasome inhibitors, allowing for precise mechanistic studies in cell and animal models.