# ZRSR2

Source: https://onco.cc/targets/zrsr2/  
OnCo record `zrsr2` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

ZRSR2 (U2 small nuclear ribonucleoprotein auxiliary factor 35 kDa subunit-related protein 2) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as a tumour suppressor and a biomarker, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Myeloproliferative neoplasms, Leukaemia, Skin cancer and 2 more.

## Summary

Pre-mRNA-binding protein required for splicing of both U2- and U12-type introns. Selectively interacts with the 3'-splice site of U2- and U12-type pre-mRNAs and promotes different steps in U2 and U12 intron splicing. Recruited to U12 pre-mRNAs in an ATP-dependent manner and is required for assembly of the pre-spliceosome, a precursor to other spliceosomal complexes.

CIViC holds 1 clinical evidence item and 0 assertions across 1 variant. Open Targets scores its association with cancer at 0.63 (direct and indirect evidence; datatypes literature 0.42, genetic association 0.00, somatic mutation 0.82). IntOGen calls it a driver in 1 cohort (0 activating, 1 loss-of-function), covering Acute Myeloid Leukaemia.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: zinc finger CCCH-type, RNA binding motif and serine/arginine rich 2; U2 small nuclear ribonucleoprotein auxiliary factor 35 kDa subunit-related protein 2; U2AF1-RS2; ZC3H22; U2AF1L2
- Tags: cancer-genes-wave
- Symbol: ZRSR2
- Class: tumor-suppressor
- Biology: Pre-mRNA-binding protein required for splicing of both U2- and U12-type introns. Selectively interacts with the 3'-splice site of U2- and U12-type pre-mRNAs and promotes different steps in U2 and U12 intron splicing. Recruited to U12 pre-mRNAs in an ATP-dependent manner and is required for assembly of the pre-spliceosome, a precursor to other spliceosomal complexes. For U2-type introns, it is selectively and specifically required for the second step of splicing. Location: Nucleus (UniProt). Locus Xp22.2 (HGNC).
- Where found: Myeloproliferative neoplasms: Open Targets association 0.59 with myeloproliferative neoplasm (MONDO_0020076); Leukaemia: Open Targets association 0.57 with leukaemia (MONDO_0005059); Skin cancer: Open Targets association 0.52 with skin cancer (MONDO_0002898); Non-Hodgkin lymphoma: Open Targets association 0.51 with non-Hodgkin lymphoma (MONDO_0018908); Acute myeloid leukaemia: CIViC evidence names this disease; IntOGen driver in 1 cohort (AML)

## 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 a loss-of-function (LoF) driver in 1 cohort; 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.

## Sources

- HGNC HGNC:23019: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:23019
- UniProt Q15696: https://www.uniprot.org/uniprotkb/Q15696/entry
- NCBI Gene 8233: https://www.ncbi.nlm.nih.gov/gene/8233
- Ensembl ENSG00000169249: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000169249

## 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: [Acute myeloid leukaemia](https://onco.cc/cancers/aml/), [Leukaemia (all types)](https://onco.cc/cancers/leukaemia/), [Myeloproliferative neoplasms (PV, ET, myelofibrosis)](https://onco.cc/cancers/myeloproliferative-neoplasms/), [Non-Hodgkin lymphoma (all types)](https://onco.cc/cancers/non-hodgkin-lymphoma/), [Skin cancer (all types)](https://onco.cc/cancers/skin-cancer/)

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JSON: https://onco.cc/api/v1/entities/zrsr2.json