Ranbp-Type And C3Hc4-Type Zinc Finger-Containing Protein 1 (RBCK1) Reagents

RanBP-type and C3HC4-type zinc finger-containing protein 1

E3 ubiquitin-protein ligase, which accepts ubiquitin from specific E2 ubiquitin-conjugating enzymes, such as UBE2L3/UBCM4, and then transfers it to substrates. Functions as an E3 ligase for oxidized IREB2 and both heme and oxygen are necessary for IREB2 ubiquitination. Promotes ubiquitination of TAB2 and IRF3 and their degradation by the proteasome. Component of the LUBAC complex which conjugates linear ('Met-1'-linked) polyubiquitin chains 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. Binds polyubiquitin of different linkage types.

Polyglucosan body myopathy 1 with or without immunodeficiency (PGBM1)

C20orf18; Chromosome 20 open reading frame 18; HBV associated factor 4 ; HBV-associated factor 4; Heme-oxidized IRP2 ubiquitin ligase 1; Hepatitis B virus X associated protein 4; Hepatitis B virus X-associated protein 4; HOIL 1L; HOIL-1; HOIL-1L; HOIL1; HOIL1L; RanBP type and C3HC4 type zinc finger containing 1; RanBP-type and C3HC4-type zinc finger-containing protein 1; RBCC protein interacting with PKC1; Rbck1; RBCK2; RING finger protein 54; RNF54 ; UB7I3_HUMAN; UBCE7IP3 ; Ubiquitin conjugating enzyme 7 interacting protein 3; Ubiquitin-conjugating enzyme 7-interacting protein 3; XAP3; XAP4 ; ZRANB4

Enables several functions, including identical protein binding activity; protein sequestering activity; and ubiquitin binding activity. Involved in several processes, including T cell receptor signaling pathway; cellular protein metabolic process; and regulation of DNA-binding transcription factor activity. Part of LUBAC complex. Implicated in glycogen storage disease.