# USP9X

Source: https://onco.cc/targets/usp9x/  
OnCo record `usp9x` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

USP9X (Ubiquitin carboxyl-terminal hydrolase 9X) is an enzyme. The public catalogues list it as an oncogene driver and a tumour suppressor, and it is called a cancer driver by mutation analysis of patient cohorts. Tied to Thyroid cancer, Hepatocellular carcinoma, Sarcomas and 5 more.

## Summary

Deubiquitinase involved both in the processing of ubiquitin precursors and of ubiquitinated proteins. May therefore play an important regulatory role at the level of protein turnover by preventing degradation of proteins through the removal of conjugated ubiquitin. Specifically hydrolyses 'Lys-11'-, followed by 'Lys-63'-, 'Lys-48'- and 'Lys-6'-linked polyubiquitins chains.

IntOGen calls it a driver in 7 cohorts (1 activating, 6 loss-of-function), covering Cholangiocarcinoma, Hepatocellular Carcinoma, Leiomyosarcoma, Nasopharyngeal Carcinoma, Prostate Adenocarcinoma, Well-Differentiated Thyroid Cancer.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: ubiquitin specific peptidase 9 X-linked; Ubiquitin carboxyl-terminal hydrolase 9X; DFFRX; FAF-X; MRX99
- Tags: cancer-genes-wave
- Symbol: USP9X
- Class: enzyme
- Biology: Deubiquitinase involved both in the processing of ubiquitin precursors and of ubiquitinated proteins. May therefore play an important regulatory role at the level of protein turnover by preventing degradation of proteins through the removal of conjugated ubiquitin. Specifically hydrolyses 'Lys-11'-, followed by 'Lys-63'-, 'Lys-48'- and 'Lys-6'-linked polyubiquitins chains. Essential component of TGF-beta/BMP signalling cascade. Specifically deubiquitinates monoubiquitinated SMAD4, opposing the activity of E3 ubiquitin-protein ligase TRIM33. Deubiquitinates alkylation repair enzyme ALKBH3. Location: Cytoplasm, cytosol; Cell projection, growth cone; Cytoplasm, cytoskeleton, cilium axoneme (UniProt). Locus Xp11.4 (HGNC).
- Where found: Thyroid cancer: IntOGen driver in 2 cohorts (WDTC); Hepatocellular carcinoma: IntOGen driver in 1 cohort (HCC); Sarcomas: IntOGen driver in 1 cohort (LMS); Nasopharyngeal carcinoma: IntOGen driver in 1 cohort (NPC); Prostate cancer: IntOGen driver in 1 cohort (PRAD); Papillary thyroid cancer: IntOGen driver in 2 cohorts (WDTC)

## 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 6 cohorts. Evidence tier "cohort-driver" is the strongest of those signals.
- Prevalence not recorded: none of the sources gives a positivity rate.

## Sources

- HGNC HGNC:12632: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:12632
- UniProt Q93008: https://www.uniprot.org/uniprotkb/Q93008/entry
- NCBI Gene 8239: https://www.ncbi.nlm.nih.gov/gene/8239
- Ensembl ENSG00000124486: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000124486

## Connected records

- collections: [IntOGen](https://onco.cc/collections/intogen/)
- cancers: [Biliary tract cancer (cholangiocarcinoma)](https://onco.cc/cancers/cholangiocarcinoma/), [Hepatocellular carcinoma](https://onco.cc/cancers/hcc/), [Leiomyosarcoma](https://onco.cc/cancers/leiomyosarcoma/), [Nasopharyngeal carcinoma](https://onco.cc/cancers/nasopharyngeal/), [Papillary thyroid cancer](https://onco.cc/cancers/papillary-thyroid-cancer/), [Prostate cancer](https://onco.cc/cancers/prostate/), [Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/), [Thyroid cancer](https://onco.cc/cancers/thyroid/)

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JSON: https://onco.cc/api/v1/entities/usp9x.json