Fig. 12

Loss-of-function mutation in linear ubiquitin chain assembly complex (LUBAC) is associated with autoinflammation. Loss-of-function mutations in the HOIP (e.g., L72P) affects its interaction with OTU deubiquitinase with linear linkage specificity (OTULIN) and lysine 63 deubiquitinase (CYLD), leading to prolonged NF-κB activation