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Ubiquitin-specific protease 30 (USP30) is a deubiquitinating enzyme (DUB) primarily localized to the mitochondrial outer membrane and peroxisomes. It regulates mitochondrial quality control, cellular metabolism, and various signaling pathways by removing ubiquitin from specific protein substrates. USP30 negatively regulates PINK1/Parkin-mediated mitophagy and is involved in apoptosis, lipid metabolism, tumorigenesis, and NLRP3 inflammasome activation. It is a therapeutic target for diseases such as Parkinson's disease and cancer, with several small-molecule inhibitors in development.
Inhibition of deubiquitination activity, leading to increased ubiquitination of target proteins and subsequent modulation of downstream pathways such as mitophagy and inflammasome activation.
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