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MERIT40 also known as BRISC, BABAM1 plays a critical role in DNA damage response and cell cycle regulation, making MERIT40 essential for maintaining genomic integrity. Located in cytoplasm and nucleus, MERIT40 is part of BRISC complex, which specifically hydrolyzes ′Lys-63′-linked polyubiquitin chains, a function distinct from other ubiquitin-processing proteases that target ′Lys-48′-linked chains for protein degradation. This unique enzymatic activity is vital for various cellular processes, including signal transduction, DNA repair, and immune responses, as BABAM1 regulates localization, activity, and stability of ubiquitinated proteins without leading them to degradation. BABAM1 interacts with BRCC36 within BRISC complex, enhancing specificity for ′Lys-63′-linked ubiquitin chains, which is particularly important in DNA damage response. BRISC complex, through MERIT40, negatively regulates NF-κB signaling pathway, thereby modulating immune responses.