UFD1 plays a crucial role in the ubiquitin-proteasome system, which is essential for maintaining cellular homeostasis by regulating protein degradation. UFD1 is primarily located in the cytoplasm and nucleus, where UFD1 participates in the recognition and processing of ubiquitin-conjugated substrates, thereby influencing the degradation of misfolded or damaged proteins. Proper functioning of UFD1 is vital, as dysregulation can lead to the accumulation of harmful proteins, contributing to various diseases, including neurodegenerative disorders. Notably, mutations in the UFD1 gene have been linked to CATCH22 syndrome, underscoring UFD1′s importance in developmental processes and potential implications in human health.