# RNF213

Source: https://onco.cc/targets/rnf213/  
OnCo record `rnf213` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

RNF213 (E3 ubiquitin-protein ligase RNF213) is an enzyme. The public catalogues list it as an oncogene driver, a tumour suppressor and a fusion partner, and it is called a cancer driver by mutation analysis of patient cohorts. Tied to Prostate cancer, Bladder & urothelial cancer, Renal cell carcinoma and 5 more.

## Summary

Atypical E3 ubiquitin ligase that can catalyse ubiquitination of both proteins and lipids, and which is involved in various processes, such as lipid metabolism, angiogenesis and cell-autonomous immunity. Acts as a key immune sensor by catalysing ubiquitination of the lipid A moiety of bacterial lipopolysaccharide (LPS) via its RZ-type zinc-finger: restricts the proliferation of cytosolic bacteria, such as Salmonella, by generating the bacterial ubiquitin coat through the ubiquitination of LPS. Also acts indirectly by mediating the recruitment of the LUBAC complex, which conjugates linear polyubiquitin chains.

IntOGen calls it a driver in 11 cohorts (5 activating, 6 loss-of-function), covering Angiosarcoma, Bladder Urothelial Carcinoma, Renal Clear Cell Carcinoma, Cholangiocarcinoma, Oesophageal Adenocarcinoma, Glioblastoma Multiforme and others.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: ring finger protein 213; E3 ubiquitin-protein ligase RNF213; KIAA1554; NET57; ALO17; C17orf27; KIAA1618; MYMY2
- Tags: cancer-genes-wave
- Symbol: RNF213
- Class: enzyme
- Biology: Atypical E3 ubiquitin ligase that can catalyse ubiquitination of both proteins and lipids, and which is involved in various processes, such as lipid metabolism, angiogenesis and cell-autonomous immunity. Acts as a key immune sensor by catalysing ubiquitination of the lipid A moiety of bacterial lipopolysaccharide (LPS) via its RZ-type zinc-finger: restricts the proliferation of cytosolic bacteria, such as Salmonella, by generating the bacterial ubiquitin coat through the ubiquitination of LPS. Also acts indirectly by mediating the recruitment of the LUBAC complex, which conjugates linear polyubiquitin chains. Ubiquitination of LPS triggers cell-autonomous immunity, such as antibacterial autophagy, leading to degradation of the microbial invader. Also functions as an intracellular sensor for ISGylated proteins: upon type I interferon stimulation, undergoes ISGylation and oligomerises on lipid droplets to form a binding platform for ISG15-modified substrate proteins, thereby acting as a broad-spectrum antimicrobial effector. Restricts intracellular bacterial pathogens, such as Listeria monocytogenes, in an ISG15-dependent manner by decorating the bacterial surface with ubiquitin to promote autophagic clearance. Location: Cytoplasm, cytosol; Lipid droplet (UniProt). Locus 17q25.3 (HGNC).
- Where found: Prostate cancer: IntOGen driver in 2 cohorts (PRAD); Bladder & urothelial cancer: IntOGen driver in 1 cohort (BLCA); Renal cell carcinoma: IntOGen driver in 1 cohort (CCRCC); Oesophageal cancer: IntOGen driver in 1 cohort (ESCA); Endometrial cancer: IntOGen driver in 1 cohort (UCEC); Angiosarcoma: IntOGen driver in 1 cohort (ANGS)

## Notes

- Written by scripts/fetch-cancer-genes.ts from CIViC, Open Targets, IntOGen, HGNC and UniProt; the function text is UniProt's, condensed and in UK spelling. Roles: IntOGen calls it an activating (Act) driver in 5 cohorts; IntOGen calls it a loss-of-function (LoF) driver in 6 cohorts; UniProt disease notes describe a translocation or gene fusion involving the gene. Evidence tier "cohort-driver" is the strongest of those signals.
- Prevalence not recorded: none of the sources gives a positivity rate.

## Sources

- HGNC HGNC:14539: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:14539
- UniProt Q63HN8: https://www.uniprot.org/uniprotkb/Q63HN8/entry
- NCBI Gene 57674: https://www.ncbi.nlm.nih.gov/gene/57674
- Ensembl ENSG00000173821: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000173821

## Connected records

- collections: [IntOGen](https://onco.cc/collections/intogen/)
- cancers: [Angiosarcoma](https://onco.cc/cancers/angiosarcoma/), [Biliary tract cancer (cholangiocarcinoma)](https://onco.cc/cancers/cholangiocarcinoma/), [Bladder & urothelial cancer](https://onco.cc/cancers/urothelial/), [Clear cell renal cell carcinoma](https://onco.cc/cancers/clear-cell-rcc/), [Endometrial cancer](https://onco.cc/cancers/endometrial/), [Oesophageal cancer](https://onco.cc/cancers/esophageal/), [Prostate cancer](https://onco.cc/cancers/prostate/), [Renal cell carcinoma](https://onco.cc/cancers/rcc/)

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JSON: https://onco.cc/api/v1/entities/rnf213.json