InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: RBCK1

Summary

InnateDB Protein

IDBP-37280.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

RBCK1

Protein Name

RanBP-type and C3HC4-type zinc finger containing 1

Synonyms

C20orf18; HOIL-1; HOIL1; PBMEI; RBCK2; RNF54; UBCE7IP3; XAP3; XAP4; ZRANB4;

Species

Homo sapiens

Ensembl Protein

ENSP00000348632

InnateDB Gene

IDBG-37270 (RBCK1)

Protein Structure

UniProt Annotation

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 ('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 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. Binds polyubiquitin of different linkage types. {ECO:0000269PubMed:12629548, ECO:0000269PubMed:17006537, ECO:0000269PubMed:17449468, ECO:0000269PubMed:18711448, ECO:0000269PubMed:19136968, ECO:0000269PubMed:20005846, ECO:0000269PubMed:21455173, ECO:0000269PubMed:21455180, ECO:0000269PubMed:21455181}.

Subcellular Localization

Disease Associations

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 107 experimentally validated interaction(s) in this database.
They are also associated with 8 interaction(s) predicted by orthology.

Experimentally validated
Total	107 [view]
Protein-Protein	107 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	8 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004842	ubiquitin-protein transferase activity
GO:0005515	protein binding
GO:0008270	zinc ion binding
GO:0016874	ligase activity
GO:0043130	ubiquitin binding

Biological Process

GO:0000209	protein polyubiquitination
GO:0016032	viral process
GO:0032088	negative regulation of NF-kappaB transcription factor activity
GO:0042346	positive regulation of NF-kappaB import into nucleus
GO:0043065	positive regulation of apoptotic process
GO:0043123	positive regulation of I-kappaB kinase/NF-kappaB signaling
GO:0043161	proteasome-mediated ubiquitin-dependent protein catabolic process
GO:0045087	innate immune response (InnateDB)
GO:0050852	T cell receptor signaling pathway
GO:0051092	positive regulation of NF-kappaB transcription factor activity
GO:0060546	negative regulation of necroptotic process
GO:0097039	protein linear polyubiquitination
GO:2001238	positive regulation of extrinsic apoptotic signaling pathway

Cellular Component

GO:0071797

LUBAC complex

Protein Structure and Domains

PDB ID

InterPro

IPR000626 Ubiquitin-like
IPR001841 Zinc finger, RING-type
IPR001876 Zinc finger, RanBP2-type
IPR002867 Zinc finger, C6HC-type
IPR029071 Ubiquitin-related domain

PFAM

PF00240
PF14560
PF13639
PF14634
PF00641
PF01485

PRINTS

PIRSF

SMART

SM00213
SM00184
SM00547
SM00647

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q9BYM8