{"entity":{"id":"drosha","kind":"target","name":"DROSHA","aka":["drosha ribonuclease III","Ribonuclease 3","RNASE3L","Etohi2","HSA242976","RN3","RNASEN"],"tldr":"DROSHA (Ribonuclease 3) is an enzyme. The public catalogues list it as an oncogene driver, a tumour suppressor and a biomarker, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Breast cancer, Brain and spinal cord tumours, Pancreatic ductal adenocarcinoma and 5 more.","summary":"Ribonuclease III double-stranded (ds) RNA-specific endoribonuclease that is involved in the initial step of microRNA (miRNA) biogenesis. Component of the microprocessor complex that is required to process primary miRNA transcripts (pri-miRNAs) to release precursor miRNA (pre-miRNA) in the nucleus. Within the microprocessor complex, DROSHA cleaves the 3' and 5' strands of a stem-loop in pri-miRNAs (processing centre 11 bp from the dsRNA-ssRNA junction) to release hairpin-shaped pre-miRNAs that are subsequently cut by the cytoplasmic DICER to generate mature miRNAs.\n\nCIViC holds 1 clinical evidence item and 0 assertions across 1 variant. Open Targets scores its association with cancer at 0.78 (direct and indirect evidence; datatypes literature 0.95, genetic association 0.83, somatic mutation 0.92). IntOGen calls it a driver in 3 cohorts (1 activating, 2 loss-of-function), covering Lung Squamous Cell Carcinoma, Pancreatic Adenocarcinoma, Wilms' Tumour.","asOf":"2026-09-23","links":[{"label":"HGNC HGNC:17904","url":"https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:17904"},{"label":"UniProt Q9NRR4","url":"https://www.uniprot.org/uniprotkb/Q9NRR4/entry"},{"label":"NCBI Gene 29102","url":"https://www.ncbi.nlm.nih.gov/gene/29102"},{"label":"Ensembl ENSG00000113360","url":"https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000113360"}],"tags":["cancer-genes-wave"],"related":["civic","open-targets","intogen"],"cancers":["breast-cancer","brain-tumours","pancreatic","ovarian","lung-cancer","skin-cancer","gastric","nsclc"],"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; IntOGen calls it a loss-of-function (LoF) driver in 2 cohorts; CIViC holds 1 clinical evidence items on its variants. Evidence tier \"clinical-evidence\" is the strongest of those signals.","Prevalence not recorded: none of the sources gives a positivity rate.","Diseases the sources name that have no OnCo cancer page yet, so they are not linked: Renal Wilms' Tumour."],"provenance":{"editedBy":"scripts/fetch-cancer-genes.ts (CIViC, Open Targets, IntOGen, HGNC, UniProt)","editedOn":"2026-09-23"},"symbol":"DROSHA","role":["oncogene-driver","tumour-suppressor","biomarker"],"evidenceTier":"clinical-evidence","sources":[{"label":"HGNC HGNC:17904","url":"https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:17904","note":"approved symbol, name, aliases, locus and cross-references (hgnc_complete_set.txt)"},{"label":"UniProt Q9NRR4","url":"https://www.uniprot.org/uniprotkb/Q9NRR4/entry","note":"protein name, function text, keywords and locations (REST API)"},{"label":"CIViC gene DROSHA","url":"https://civicdb.org/features/11314","note":"1 evidence items, 0 assertions, 1 variants; diseases: Renal Wilms' Tumour (GraphQL API, CC0)"},{"label":"Open Targets ENSG00000113360","url":"https://platform.opentargets.org/target/ENSG00000113360/associations","note":"association with cancer (MONDO_0004992) 0.78; per-cancer scores at or above 0.5: gastric cancer 0.51, ovarian cancer 0.56, skin cancer 0.55, brain cancer 0.57, breast cancer 0.63, lung cancer 0.56 (GraphQL API, CC0)"},{"label":"IntOGen DROSHA","url":"https://www.intogen.org/search?gene=DROSHA","note":"driver in 3 cohorts (Act 1, LoF 2); Compendium_Cancer_Genes.tsv release 20240920, CC0 1.0"}],"hgnc":"HGNC:17904","ensembl":"ENSG00000113360","uniprot":"Q9NRR4","entrez":"29102","biology":"Ribonuclease III double-stranded (ds) RNA-specific endoribonuclease that is involved in the initial step of microRNA (miRNA) biogenesis. Component of the microprocessor complex that is required to process primary miRNA transcripts (pri-miRNAs) to release precursor miRNA (pre-miRNA) in the nucleus. Within the microprocessor complex, DROSHA cleaves the 3' and 5' strands of a stem-loop in pri-miRNAs (processing centre 11 bp from the dsRNA-ssRNA junction) to release hairpin-shaped pre-miRNAs that are subsequently cut by the cytoplasmic DICER to generate mature miRNAs. Involved also in pre-rRNA processing. Cleaves double-strand RNA and does not cleave single-strand RNA. Involved in the formation of GW bodies. Location: Nucleus; Nucleus, nucleolus; Cytoplasm (UniProt). Locus 5p13.3 (HGNC).","whereFound":["Breast cancer: Open Targets association 0.63 with breast cancer (MONDO_0007254)","Brain and spinal cord tumours: Open Targets association 0.57 with brain cancer (MONDO_0001657)","Pancreatic ductal adenocarcinoma: IntOGen driver in 1 cohort (PAAD)","Ovarian cancer: Open Targets association 0.56 with ovarian cancer (MONDO_0008170)","Lung cancer: Open Targets association 0.56 with lung cancer (MONDO_0008903)","Skin cancer: Open Targets association 0.55 with skin cancer (MONDO_0002898)"],"targetClass":"enzyme","prevalence":[]},"route":"/targets/drosha/","neighbours":{"collection":[{"id":"civic","kind":"collection","name":"CIViC","route":"/collections/civic/"},{"id":"intogen","kind":"collection","name":"IntOGen","route":"/collections/intogen/"},{"id":"open-targets","kind":"collection","name":"Open Targets Platform","route":"/collections/open-targets/"}],"cancer":[{"id":"brain-tumours","kind":"cancer","name":"Brain and spinal cord tumours (all types)","route":"/cancers/brain-tumours/"},{"id":"breast-cancer","kind":"cancer","name":"Breast cancer (all types)","route":"/cancers/breast-cancer/"},{"id":"gastric","kind":"cancer","name":"Gastric & gastro-oesophageal junction cancer","route":"/cancers/gastric/"},{"id":"lung-cancer","kind":"cancer","name":"Lung cancer (all types)","route":"/cancers/lung-cancer/"},{"id":"nsclc","kind":"cancer","name":"Non-small-cell lung cancer","route":"/cancers/nsclc/"},{"id":"ovarian","kind":"cancer","name":"Ovarian cancer","route":"/cancers/ovarian/"},{"id":"pancreatic","kind":"cancer","name":"Pancreatic ductal adenocarcinoma","route":"/cancers/pancreatic/"},{"id":"skin-cancer","kind":"cancer","name":"Skin cancer (all types)","route":"/cancers/skin-cancer/"}]}}