{"entity":{"id":"esrra","kind":"target","name":"ESRRA","aka":["estrogen related receptor alpha","Steroid hormone receptor ERR1","ERR1","ERRalpha","NR3B1","ERRa","ESRL1"],"tldr":"ESRRA (Steroid hormone receptor ERR1) is a gene that drives cell growth when it is altered. The public catalogues list it as an oncogene driver, and it is called a cancer driver by mutation analysis of patient cohorts. Tied to Salivary gland cancers and Adenoid cystic carcinoma.","summary":"Binds to an ERR-alpha response element (ERRE) containing a single consensus half-site, 5'-TNAAGGTCA-3'. Can bind to the medium-chain acyl coenzyme A dehydrogenase (MCAD) response element NRRE-1 and may act as an important regulator of MCAD promoter. Binds to the C1 region of the lactoferrin gene promoter.\n\nIntOGen calls it a driver in 1 cohort (1 activating, 0 loss-of-function), covering Adenoid Cystic Carcinoma.","asOf":"2026-09-23","links":[{"label":"HGNC HGNC:3471","url":"https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:3471"},{"label":"UniProt P11474","url":"https://www.uniprot.org/uniprotkb/P11474/entry"},{"label":"NCBI Gene 2101","url":"https://www.ncbi.nlm.nih.gov/gene/2101"},{"label":"Ensembl ENSG00000173153","url":"https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000173153"}],"tags":["cancer-genes-wave"],"related":["intogen"],"cancers":["salivary-gland","adenoid-cystic-carcinoma"],"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 an activating (Act) 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":"ESRRA","role":["oncogene-driver"],"evidenceTier":"cohort-driver","sources":[{"label":"HGNC HGNC:3471","url":"https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:3471","note":"approved symbol, name, aliases, locus and cross-references (hgnc_complete_set.txt)"},{"label":"UniProt P11474","url":"https://www.uniprot.org/uniprotkb/P11474/entry","note":"protein name, function text, keywords and locations (REST API)"},{"label":"IntOGen ESRRA","url":"https://www.intogen.org/search?gene=ESRRA","note":"driver in 1 cohort (Act 1, LoF 0); Compendium_Cancer_Genes.tsv release 20240920, CC0 1.0"}],"hgnc":"HGNC:3471","ensembl":"ENSG00000173153","uniprot":"P11474","entrez":"2101","biology":"Binds to an ERR-alpha response element (ERRE) containing a single consensus half-site, 5'-TNAAGGTCA-3'. Can bind to the medium-chain acyl coenzyme A dehydrogenase (MCAD) response element NRRE-1 and may act as an important regulator of MCAD promoter. Binds to the C1 region of the lactoferrin gene promoter. Requires dimerisation and the coactivator, PGC-1A, for full activity. The ERRalpha/PGC1alpha complex is a regulator of energy metabolism. Induces the expression of PERM1 in the skeletal muscle. Location: Nucleus; Cytoplasm (UniProt). Locus 11q13.1 (HGNC).","whereFound":["Salivary gland cancers: IntOGen driver in 1 cohort (ACYC)","Adenoid cystic carcinoma: IntOGen driver in 1 cohort (ACYC)"],"targetClass":"oncogene","prevalence":[]},"route":"/targets/esrra/","neighbours":{"collection":[{"id":"intogen","kind":"collection","name":"IntOGen","route":"/collections/intogen/"}],"cancer":[{"id":"adenoid-cystic-carcinoma","kind":"cancer","name":"Adenoid cystic carcinoma","route":"/cancers/adenoid-cystic-carcinoma/"},{"id":"salivary-gland","kind":"cancer","name":"Salivary gland cancers","route":"/cancers/salivary-gland/"}]}}