Component of the LUBAC complex which conjugates linear polyubiquitin chains in a head-to-tail manner to substrates and plays a key role in NF-kappa-B activation and regulation of inflammation. LUBAC conjugates linear polyubiquitin to IKBKG and RIPK1 and is involved in activation of the canonical NF-kappa-B and the JNK signaling pathways. Linear ubiquitination mediated by the LUBAC complex interferes with TNF-induced cell death and thereby prevents inflammation. LUBAC is proposed to be recruited to the TNF-R1 signaling complex (TNF-RSC) following polyubiquitination of TNF-RSC components by BIRC2 and/or BIRC3 and to conjugate linear polyubiquitin to IKBKG and possibly other components contributing to the stability of the complex. Together with FAM105B/otulin, the LUBAC complex regulates the canonical Wnt signaling during angiogenesis.
Protein Aliases: hSIPL1; mSIPL1; protein kinase C-interacting protein RBCC like 1; SHANK-associated RH domain interacting protein; SHANK-associated RH domain interactor; Shank-associated RH domain-interacting protein; shank-interacting protein; Shank-interacting protein-like 1; Sharpin
Gene Aliases: 0610041B22Rik; AW121341; Conneck1; Cpdm; PSEC0216; RBCKL1; SHARPIN; SIPL1