# DTX1

Source: https://onco.cc/targets/dtx1/  
OnCo record `dtx1` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

DTX1 (E3 ubiquitin-protein ligase DTX1) 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 Diffuse large B-cell lymphoma and Chronic lymphocytic leukaemia.

## Summary

E3 ubiquitin-protein ligase that mediates ubiquitination and proteasomal degradation of MEKK1, thereby modulating signalling pathways involved in cell fate determination. Regulates the Notch signalling pathway, acting predominantly as a positive regulator but functioning as a context-dependent negative regulator in specific developmental or cellular settings. Mediates the antineural activity of Notch signalling, likely by inhibiting transcriptional activation mediated by MATCH1.

CIViC holds 1 clinical evidence item and 0 assertions across 1 variant. IntOGen calls it a driver in 1 cohort (0 activating, 1 loss-of-function), covering Chronic Lymphocytic Leukaemia/Small Lymphocytic Lymphoma.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: deltex E3 ubiquitin ligase 1; E3 ubiquitin-protein ligase DTX1; hDx-1; RNF140
- Tags: cancer-genes-wave
- Symbol: DTX1
- Class: tumor-suppressor
- Biology: E3 ubiquitin-protein ligase that mediates ubiquitination and proteasomal degradation of MEKK1, thereby modulating signalling pathways involved in cell fate determination. Regulates the Notch signalling pathway, acting predominantly as a positive regulator but functioning as a context-dependent negative regulator in specific developmental or cellular settings. Mediates the antineural activity of Notch signalling, likely by inhibiting transcriptional activation mediated by MATCH1. Negatively regulates Notch signalling by controlling the activity of PIP4K2C, a lipid kinase that promotes recycling of Notch receptors from RAB4A-positive endosomes to the cell surface. Participates in several developmental and immune processes, including neurogenesis, lymphogenesis, and myogenesis. Influences lymphocyte differentiation by promoting B-cell development at the expense of T-cell development, suggesting functional antagonism of NOTCH1 in this context. Location: Cytoplasm; Nucleus; Endosome (UniProt). Locus 12q24.13 (HGNC).
- Where found: Diffuse large B-cell lymphoma: CIViC evidence names this disease; Chronic lymphocytic leukaemia: IntOGen driver in 1 cohort (CLLSLL)

## 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:3060: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:3060
- UniProt Q86Y01: https://www.uniprot.org/uniprotkb/Q86Y01/entry
- NCBI Gene 1840: https://www.ncbi.nlm.nih.gov/gene/1840
- Ensembl ENSG00000135144: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000135144

## Connected records

- collections: [CIViC](https://onco.cc/collections/civic/), [IntOGen](https://onco.cc/collections/intogen/)
- cancers: [Chronic lymphocytic leukaemia](https://onco.cc/cancers/cll/), [Diffuse large B-cell lymphoma](https://onco.cc/cancers/dlbcl/)

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JSON: https://onco.cc/api/v1/entities/dtx1.json