# DROSHA

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

## TL;DR

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.

CIViC 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.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: drosha ribonuclease III; Ribonuclease 3; RNASE3L; Etohi2; HSA242976; RN3; RNASEN
- Tags: cancer-genes-wave
- Symbol: DROSHA
- Class: enzyme
- 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).
- Where found: 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)

## 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.

## Sources

- HGNC HGNC:17904: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:17904
- UniProt Q9NRR4: https://www.uniprot.org/uniprotkb/Q9NRR4/entry
- NCBI Gene 29102: https://www.ncbi.nlm.nih.gov/gene/29102
- Ensembl ENSG00000113360: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000113360

## Connected records

- collections: [CIViC](https://onco.cc/collections/civic/), [IntOGen](https://onco.cc/collections/intogen/), [Open Targets Platform](https://onco.cc/collections/open-targets/)
- cancers: [Brain and spinal cord tumours (all types)](https://onco.cc/cancers/brain-tumours/), [Breast cancer (all types)](https://onco.cc/cancers/breast-cancer/), [Gastric & gastro-oesophageal junction cancer](https://onco.cc/cancers/gastric/), [Lung cancer (all types)](https://onco.cc/cancers/lung-cancer/), [Non-small-cell lung cancer](https://onco.cc/cancers/nsclc/), [Ovarian cancer](https://onco.cc/cancers/ovarian/), [Pancreatic ductal adenocarcinoma](https://onco.cc/cancers/pancreatic/), [Skin cancer (all types)](https://onco.cc/cancers/skin-cancer/)

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