{"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"}],"specificity":"tumour-specific","distribution":"many-types","specificityNote":"Tumour-specific alteration: the catalogues call it an oncogene driver (IntOGen cohort analysis finds it activated more often than chance) and a tumour suppressor (IntOGen finds it knocked out more often than chance), so the direction differs between cohorts but the alteration is somatic either way; what a medicine would aim at or exploit is the altered form or its loss, absent from normal cells; no corpus medicine is aimed at it yet. HPA DROSHA: RNA low tissue specificity; no normal tissue stained high. Distribution: 7 cancer families in the corpus carry a prevalence row, label threshold or catalogue link for it (Breast cancer (all types), Brain and spinal cord tumours (all types), Pancreatic ductal adenocarcinoma, Ovarian cancer, Lung cancer (all types), Skin cancer (all types), Gastric & gastro-oesophageal junction cancer); Open Targets associates it with 0 specific cancer types at or above 0.5. (Rule 6 of scripts/fetch-target-specificity.ts.)","specificitySources":[{"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":"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"},{"label":"Human Protein Atlas DROSHA tissue","url":"https://www.proteinatlas.org/ENSG00000113360-DROSHA/tissue","note":"RNA tissue and blood lineage specificity, normal tissue antibody staining (version 25.1, CC BY-SA 3.0)"},{"label":"Open Targets ENSG00000113360 associations","url":"https://platform.opentargets.org/target/ENSG00000113360/associations","note":"cancer associations at or above 0.5 (CC0)"}],"hgnc":"HGNC:17904","ensembl":"ENSG00000113360","uniprot":"Q9NRR4","entrez":"29102","firstDescribed":1998,"firstDescribedBasis":"sequence","firstDescribedNote":"Earliest sequence paper UniProt cites for the protein: Wei Y.J. et al, 1998.","firstDescribedSource":"https://www.uniprot.org/uniprotkb/Q9NRR4/entry","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/"}]}}