Aliases for SHARPIN Gene
External Ids for SHARPIN Gene
Previous GeneCards Identifiers for SHARPIN Gene
GeneCards Summary for SHARPIN Gene
SHARPIN (SHANK Associated RH Domain Interactor) is a Protein Coding gene. Diseases associated with SHARPIN include Incontinentia Pigmenti and Autoinflammation, Panniculitis, And Dermatosis Syndrome. Among its related pathways are TNF signaling (REACTOME) and Signaling by GPCR. Gene Ontology (GO) annotations related to this gene include identical protein binding and polyubiquitin modification-dependent protein binding. An important paralog of this gene is RBCK1.
UniProtKB/Swiss-Prot Summary for SHARPIN Gene
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 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 OTULIN, the LUBAC complex regulates the canonical Wnt signaling during angiogenesis.