# DIS3

Source: https://onco.cc/targets/dis3/  
OnCo record `dis3` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

DIS3 (Exosome complex exonuclease RRP44) 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 Multiple myeloma.

## Summary

Putative catalytic component of the RNA exosome complex which has 3'->5' exoribonuclease activity and participates in a multitude of cellular RNA processing and degradation events. In the nucleus, the RNA exosome complex is involved in proper maturation of stable RNA species such as rRNA, snRNA and snoRNA, in the elimination of RNA processing by-products and non-coding 'pervasive' transcripts, such as antisense RNA species and promoter-upstream transcripts (PROMPTs), and of mRNAs with processing defects, thereby limiting or excluding their export to the cytoplasm. The RNA exosome may be involved in Ig class switch recombination (CSR) and/or Ig variable region somatic hypermutation (SHM) by targeting AICDA deamination activity to transcribed dsDNA substrates.

IntOGen calls it a driver in 1 cohort (1 activating, 0 loss-of-function), covering Plasma Cell Myeloma.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: DIS3 exosome endoribonuclease and 3'-5' exoribonuclease; Exosome complex exonuclease RRP44; dis3p; RRP44; EXOSC11; KIAA1008
- Tags: cancer-genes-wave
- Symbol: DIS3
- Class: oncogene
- Biology: Putative catalytic component of the RNA exosome complex which has 3'->5' exoribonuclease activity and participates in a multitude of cellular RNA processing and degradation events. In the nucleus, the RNA exosome complex is involved in proper maturation of stable RNA species such as rRNA, snRNA and snoRNA, in the elimination of RNA processing by-products and non-coding 'pervasive' transcripts, such as antisense RNA species and promoter-upstream transcripts (PROMPTs), and of mRNAs with processing defects, thereby limiting or excluding their export to the cytoplasm. The RNA exosome may be involved in Ig class switch recombination (CSR) and/or Ig variable region somatic hypermutation (SHM) by targeting AICDA deamination activity to transcribed dsDNA substrates. In the cytoplasm, the RNA exosome complex is involved in general mRNA turnover and specifically degrades inherently unstable mRNAs containing AU-rich elements (AREs) within their 3' untranslated regions, and in RNA surveillance pathways, preventing translation of aberrant mRNAs. It seems to be involved in degradation of histone mRNA. DIS3 has both 3'-5' exonuclease and endonuclease activities. Location: Cytoplasm; Nucleus, nucleolus; Nucleus, nucleoplasm; Nucleus (UniProt). Locus 13q21.33 (HGNC).
- Where found: Multiple myeloma: IntOGen driver in 1 cohort (PCM)

## 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.

## Sources

- HGNC HGNC:20604: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:20604
- UniProt Q9Y2L1: https://www.uniprot.org/uniprotkb/Q9Y2L1/entry
- NCBI Gene 22894: https://www.ncbi.nlm.nih.gov/gene/22894
- Ensembl ENSG00000083520: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000083520

## Connected records

- collections: [IntOGen](https://onco.cc/collections/intogen/)
- cancers: [Multiple myeloma](https://onco.cc/cancers/multiple-myeloma/)

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JSON: https://onco.cc/api/v1/entities/dis3.json