RBCK1

RBCK1
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	2CRC, 2LGY, 4DBG
Identifiers
Aliases	RBCK1, C20orf18, HOIL-1, HOIL1, PBMEI, RBCK2, RNF54, UBCE7IP3, XAP3, XAP4, ZRANB4, PGBM1, RANBP2-type and C3HC4-type zinc finger containing 1
External IDs	OMIM: 610924; MGI: 1344372; HomoloGene: 32448; GeneCards: RBCK1; OMA:RBCK1 - orthologs
Gene location (Human)
Chr.	Chromosome 20 (human)
End	432,139 bp
Gene location (Mouse)
Chr.	Chromosome 2 (mouse)
End	152,174,573 bp
RNA expression pattern
	Top expressed in
	right hemisphere of cerebellum; ; anterior pituitary; ; right uterine tube; ; right lobe of liver; ; granulocyte; ; right adrenal gland; ; left adrenal cortex; ; right adrenal cortex; ; right lobe of thyroid gland; ; mucosa of transverse colon;
	Top expressed in
	neural layer of retina; ; superior frontal gyrus; ; ankle joint; ; thymus; ; lip; ; dentate gyrus of hippocampal formation granule cell; ; primary visual cortex; ; granulocyte; ; mesenteric lymph nodes; ; genital tubercle;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	DNA-binding transcription factor activity; metal ion binding; ubiquitin binding; protein binding; ubiquitin-protein transferase activity; transferase activity; identical protein binding;
Cellular component	cytosol; LUBAC complex;
Biological process	negative regulation of necroptotic process; regulation of tumor necrosis factor-mediated signaling pathway; positive regulation of NF-kappaB transcription factor activity; positive regulation of apoptotic process; positive regulation of I-kappaB kinase/NF-kappaB signaling; positive regulation of extrinsic apoptotic signaling pathway; viral process; I-kappaB kinase/NF-kappaB signaling; negative regulation of NF-kappaB transcription factor activity; T cell receptor signaling pathway; protein linear polyubiquitination; proteasome-mediated ubiquitin-dependent protein catabolic process; protein polyubiquitination; positive regulation of NIK/NF-kappaB signaling; regulation of transcription, DNA-templated;
	Sources:Amigo / QuickGO
Orthologs
	10616
	24105
	ENSG00000125826
	ENSMUSG00000027466
	Q9BYM8
	Q9WUB0
NM_006462; NM_031227; NM_031228; NM_031229; NM_001323956;
	NM_001323958; NM_001323960
	NM_001083921; NM_019705; NM_001355282
	NP_001310885; NP_001310887; NP_001310889; NP_006453; NP_112506
	NP_001077390; NP_062679; NP_001342211
	Wikidata
View/Edit Human	View/Edit Mouse

RanBP-type and C3HC4-type zinc finger-containing protein 1 (also known as HOIL-1) is a protein that in humans is encoded by the RBCK1 gene.

The protein encoded by this gene is similar to mouse UIP28/UbcM4 interacting protein. Alternative splicing has been observed at this locus, resulting in distinct isoforms.

HOIL-1 is an E3 ubiquitin ligase and a part of the linear ubiquitin chain assembly complex (LUBAC), the only known ubiquitin ligase generating linear (Met1) polyubiquitin linkages. Although HOIL-1 isn’t responsible for the linear ubiquitin generation, it is a necessary component of LUBAC and ensures its proper assembly and function. Unlike most E3 ubiquitin ligases, HOIL-1 is able to catalyze oxyester bond formation between the C-terminus of a ubiquitin monomer and Ser/Thr of a different protein. This recently discovered function of HOIL-1 has so far been described in the context of MyD88 signaling. Additionally, a catalytically inactive mutant of HOIL-1 (HOIL-1^C458S) led to prolonged IKK activation and increase of inflammatory cytokine production by cytotoxic T cells. The proposed mechanism is that the ester-linked ubiquitin chains limit the size of isopeptide-linked (K63 and/or M1) ubiquitin chains.