The important role of OTULIN in restraining inflammation was also suggested by another study in patients suffering from otulipenia due to OTULIN missense mutations that were associated with excessive linear ubiquiylation of target proteins such as NEMO, RIPK1, and TNFR1, resulting in severe inflammation and further confirming OTULIN as a gatekeeper of innate immunity145. Here, OTULIN is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.