{"entity":{"id":"znrf3","kind":"target","name":"ZNRF3","aka":["zinc and ring finger 3","E3 ubiquitin-protein ligase ZNRF3","KIAA1133","BK747E2.3","FLJ22057","RNF203"],"tldr":"ZNRF3 (E3 ubiquitin-protein ligase ZNRF3) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as a tumour suppressor, and it is called a cancer driver by mutation analysis of patient cohorts. Tied to Adrenocortical carcinoma.","summary":"E3 ubiquitin-protein ligase that acts as a negative regulator of the Wnt signalling pathway by mediating the ubiquitination and subsequent degradation of Wnt receptor complex components Frizzled and LRP6. Acts on both canonical and non-canonical Wnt signalling pathway. Acts as a tumour suppressor in the intestinal stem cell zone by inhibiting the Wnt signalling pathway, thereby restricting the size of the intestinal stem cell zone.\n\nOpen Targets scores its association with cancer at 0.55 (direct and indirect evidence; datatypes affected pathway 0.61, literature 0.94, genetic association 0.56, somatic mutation 0.47, animal model 0.41). IntOGen calls it a driver in 1 cohort (0 activating, 1 loss-of-function), covering Adrenocortical Carcinoma.","asOf":"2026-09-23","links":[{"label":"HGNC HGNC:18126","url":"https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:18126"},{"label":"UniProt Q9ULT6","url":"https://www.uniprot.org/uniprotkb/Q9ULT6/entry"},{"label":"NCBI Gene 84133","url":"https://www.ncbi.nlm.nih.gov/gene/84133"},{"label":"Ensembl ENSG00000183579","url":"https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000183579"}],"tags":["cancer-genes-wave"],"related":["open-targets","intogen"],"cancers":["adrenocortical"],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"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 a loss-of-function (LoF) driver in 1 cohort. Evidence tier \"cohort-driver\" is the strongest of those signals.","Prevalence not recorded: none of the sources gives a positivity rate."],"provenance":{"editedBy":"scripts/fetch-cancer-genes.ts (CIViC, Open Targets, IntOGen, HGNC, UniProt)","editedOn":"2026-09-23"},"symbol":"ZNRF3","role":["tumour-suppressor"],"evidenceTier":"cohort-driver","sources":[{"label":"HGNC HGNC:18126","url":"https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:18126","note":"approved symbol, name, aliases, locus and cross-references (hgnc_complete_set.txt)"},{"label":"UniProt Q9ULT6","url":"https://www.uniprot.org/uniprotkb/Q9ULT6/entry","note":"protein name, function text, keywords and locations (REST API)"},{"label":"Open Targets ENSG00000183579","url":"https://platform.opentargets.org/target/ENSG00000183579/associations","note":"association with cancer (MONDO_0004992) 0.55;  (GraphQL API, CC0)"},{"label":"IntOGen ZNRF3","url":"https://www.intogen.org/search?gene=ZNRF3","note":"driver in 1 cohort (Act 0, LoF 1); Compendium_Cancer_Genes.tsv release 20240920, CC0 1.0"}],"hgnc":"HGNC:18126","ensembl":"ENSG00000183579","uniprot":"Q9ULT6","entrez":"84133","biology":"E3 ubiquitin-protein ligase that acts as a negative regulator of the Wnt signalling pathway by mediating the ubiquitination and subsequent degradation of Wnt receptor complex components Frizzled and LRP6. Acts on both canonical and non-canonical Wnt signalling pathway. Acts as a tumour suppressor in the intestinal stem cell zone by inhibiting the Wnt signalling pathway, thereby restricting the size of the intestinal stem cell zone. Along with RSPO2 and RNF43, constitutes a master switch that governs limb specification. Location: Cell membrane (UniProt). Locus 22q12.1 (HGNC).","whereFound":["Adrenocortical carcinoma: IntOGen driver in 1 cohort (ACC)"],"targetClass":"tumor-suppressor","prevalence":[]},"route":"/targets/znrf3/","neighbours":{"collection":[{"id":"intogen","kind":"collection","name":"IntOGen","route":"/collections/intogen/"},{"id":"open-targets","kind":"collection","name":"Open Targets Platform","route":"/collections/open-targets/"}],"cancer":[{"id":"adrenocortical","kind":"cancer","name":"Adrenocortical carcinoma","route":"/cancers/adrenocortical/"}]}}