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Beschreibung

RanBP-type and C3HC4-type zinc finger-containing protein 1 (UniProt Q9BYM8, also known as HOIL-1, HBV-associated factor 4, Heme-oxidized IRP2 ubiquitin ligase 1, Hepatitis B virus X-associated protein 4, RBCC protein interacting with PKC1, RING finger protein 54, Ubiquitin-conjugating enzyme 7-interacting protein 3) is encoded by the RBCK1 gene (also known as C20orf18, HOIL1, PBMEI, RBCK2, RNF54, UBCE7IP3, XAP3, XAP4, ZRANB4) (Gene ID 10616) in human. The linear ubiquitin assembly complex (LUBAC) is comprised of two RING-IBR-RING (RBR)-containing E3 ligases, HOIL-1L and HOIP. LUBAC conjugates a head-to-tail-linked linear polyubiquitin chain onto lysine residues on substrate proteins. Ovarian tumor deubiquitinating enzyme (DUB) with linear linkage specificity (OTULIN/Gumby/FAM105B) and cylindromatosis (CYLD) are two DUBs that catalyze the cleavage linear polyubiquitin chains. OTULIN and CYLD interact with the N-terminal PNGase/UBA or UBX (PUB) domain of HOIP and negatively regulate LUBAC-mediated cellular processes. LUBAC is reported to suppress IFN-dependent antiviral activity by mediating TRIM25 degradation and competing against TRIM25 for RIG-I interaction.