# TFE3

Source: https://onco.cc/targets/tfe3/  
OnCo record `tfe3` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

TFE3 (Transcription factor E3) is a protein that switches other genes on and off. The public catalogues list it as a drug target and a fusion partner, and the evidence so far is association rather than a proven role. Tied to Renal cell carcinoma, Sarcomas, Colorectal cancer and 1 more.

## Summary

Transcription factor that acts as a master regulator of lysosomal biogenesis and immune response. Specifically recognises and binds E-box sequences (5'-CANNTG-3'); efficient DNA-binding requires dimerisation with itself or with another MiT/TFE family member such as TFEB or MITF. Involved in the cellular response to amino acid availability by acting downstream of MTOR: in the presence of nutrients, TFE3 phosphorylation by MTOR promotes its inactivation.

Open Targets scores its association with cancer at 0.76 (direct and indirect evidence; datatypes literature 0.99, genetic association 0.64, somatic mutation 0.98, genetic literature 0.76). In OnCo, 1 product record names it (Cediranib).

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: transcription factor binding to IGHM enhancer 3; Transcription factor E3; bHLHe33
- Tags: cancer-genes-wave
- Symbol: TFE3
- Class: transcription
- Biology: Transcription factor that acts as a master regulator of lysosomal biogenesis and immune response. Specifically recognises and binds E-box sequences (5'-CANNTG-3'); efficient DNA-binding requires dimerisation with itself or with another MiT/TFE family member such as TFEB or MITF. Involved in the cellular response to amino acid availability by acting downstream of MTOR: in the presence of nutrients, TFE3 phosphorylation by MTOR promotes its inactivation. Upon starvation or lysosomal stress, inhibition of MTOR induces TFE3 dephosphorylation, resulting in transcription factor activity. Specifically recognises and binds the CLEAR-box sequence (5'-GTCACGTGAC-3') present in the regulatory region of many lysosomal genes, leading to activate their expression, thereby playing a central role in expression of lysosomal genes. Maintains the pluripotent state of embryonic stem cells by promoting the expression of genes such as ESRRB; mTOR-dependent TFE3 cytosolic retention and inactivation promotes exit from pluripotency. Location: Cytoplasm, cytosol; Nucleus; Lysosome membrane (UniProt). Locus Xp11.23 (HGNC).
- Where found: Renal cell carcinoma: Open Targets association 0.66 with renal cell carcinoma (MONDO_0005086); Sarcomas: Open Targets association 0.57 with sarcoma (MONDO_0005089); Colorectal cancer: Open Targets association 0.50 with colorectal cancer (MONDO_0005575); Melanoma: Open Targets association 0.52 with melanoma (MONDO_0005105)

## 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: 1 OnCo product record names it; UniProt disease notes describe a translocation or gene fusion involving the gene. Evidence tier "association-only" is the strongest of those signals.
- Prevalence not recorded: none of the sources gives a positivity rate.

## Sources

- HGNC HGNC:11752: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:11752
- UniProt P19532: https://www.uniprot.org/uniprotkb/P19532/entry
- NCBI Gene 7030: https://www.ncbi.nlm.nih.gov/gene/7030
- Ensembl ENSG00000068323: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000068323

## Connected records

- collections: [Open Targets Platform](https://onco.cc/collections/open-targets/)
- cancers: [Colorectal cancer](https://onco.cc/cancers/colorectal/), [Melanoma](https://onco.cc/cancers/melanoma/), [Renal cell carcinoma](https://onco.cc/cancers/rcc/), [Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/)
- drugs: [Cediranib](https://onco.cc/drugs/cediranib/)

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JSON: https://onco.cc/api/v1/entities/tfe3.json