Human Gene RBCK1 (ENST00000353660.7) Description and Page Index

Description: Homo sapiens RANBP2-type and C3HC4-type zinc finger containing 1 (RBCK1), transcript variant 1, mRNA. (from RefSeq NM_006462)
Gencode Transcript: ENST00000353660.7
Gencode Gene: ENSG00000125826.21
Transcript (Including UTRs)
Position: hg38 chr20:408,171-430,966 Size: 22,796 Total Exon Count: 11 Strand: +
Coding Region
Position: hg38 chr20:408,739-430,430 Size: 21,692 Coding Exon Count: 11

Page Index

Sequence and Links

UniProtKB Comments

Protein Structure

Other Names

Methods

Data last updated at UCSC: 2021-06-20 19:51:40

Comments and Description Text from UniProtKB


	ID: HOIL1_HUMAN DESCRIPTION: RecName: Full=RanBP-type and C3HC4-type zinc finger-containing protein 1; EC=6.3.2.-; AltName: Full=HBV-associated factor 4; AltName: Full=Heme-oxidized IRP2 ubiquitin ligase 1; Short=HOIL-1; AltName: Full=Hepatitis B virus X-associated protein 4; AltName: Full=RING finger protein 54; AltName: Full=Ubiquitin-conjugating enzyme 7-interacting protein 3; FUNCTION: 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 ('M-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 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. Binds polyubiquitin of different linkage types. SUBUNIT: Forms homodimers in vitro (By similarity). Component of the LUBAC complex (linear ubiquitin chain assembly complex) which consists of SHARPIN, RBCK1 and RNF31. LUBAC has a MW of approximative 600 kDa suggesting a heteromultimeric assembly of its subunits. Interacts with beta-I-type (PRKCB1) and zeta-type protein kinase C (PRKCZ) and with UBE2L3. Interacts with PRKCH. Interacts with the HBV pX/HBx protein, which is required to activate transcription of the viral genome. Isoform 1 and isoform 2 interact with IREB2 only in iron-rich conditions. Associates with the TNF-R1 signaling complex (TNF-RSC) in a stimulation- dependent manner. Interacts with EYA1, TAB2, TAB3, MAP3K7 TRAF6 and RIPK1. Interacts with IRF3. INTERACTION: Q4VA12:Traf1 (xeno); NbExp=2; IntAct=EBI-2340624, EBI-6116765; INDUCTION: By viral transfection. DOMAIN: The RanBP2-type zinc finger, also called Npl4 zinc finger (NZF), mediates binding to 'M-1'-linked polyubiquitins (By similarity). DOMAIN: The UBL domain mediates association with RNF31 via interaction with its UBA domain. PTM: Auto-ubiquitinated. Auto-ubiquitination leads to degradation by the proteasome (By similarity). PTM: Phosphorylated. In vitro, phosphorylation inhibits auto- ubiquitination activity (By similarity). SIMILARITY: Contains 1 IBR-type zinc finger. SIMILARITY: Contains 1 RanBP2-type zinc finger. SIMILARITY: Contains 2 RING-type zinc fingers. SIMILARITY: Contains 1 ubiquitin-like domain. SEQUENCE CAUTION: Sequence=AAH15219.2; Type=Erroneous initiation; Note=Translation N-terminally extended;