# LZTR1

Source: https://onco.cc/targets/lztr1/  
OnCo record `lztr1` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

LZTR1 (Leucine-zipper-like transcriptional regulator 1) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as an oncogene driver, a tumour suppressor and a biomarker, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Hepatocellular carcinoma, Prostate cancer, Endometrial cancer and 5 more.

## Summary

Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complex that mediates ubiquitination of Ras GTPases (M-Ras/MRAS, K-Ras/KRAS, N-Ras/NRAS and H-Ras/HRAS) and Ras-like protein RIT1. Acts as a negative regulator of RAS-MAPK signalling by controlling Ras levels and decreasing Ras association with membranes.

CIViC holds 2 clinical evidence items and 0 assertions across 2 variants. Open Targets scores its association with cancer at 0.78 (direct and indirect evidence; datatypes genetic literature 0.38, literature 0.90, genetic association 0.64, somatic mutation 0.94, animal model 0.37). IntOGen calls it a driver in 4 cohorts (1 activating, 3 loss-of-function), covering Glioblastoma Multiforme, Hepatocellular Carcinoma, Prostate Adenocarcinoma, Endometrial Carcinoma.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: leucine zipper like post translational regulator 1; Leucine-zipper-like transcriptional regulator 1; LZTR-1; BTBD29
- Tags: cancer-genes-wave
- Symbol: LZTR1
- Class: tumor-suppressor
- Biology: Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complex that mediates ubiquitination of Ras GTPases (M-Ras/MRAS, K-Ras/KRAS, N-Ras/NRAS and H-Ras/HRAS) and Ras-like protein RIT1. Acts as a negative regulator of RAS-MAPK signalling by controlling Ras levels and decreasing Ras association with membranes. Location: Endomembrane system; Recycling endosome; Golgi apparatus (UniProt). Locus 22q11.21 (HGNC).
- Where found: Hepatocellular carcinoma: IntOGen driver in 1 cohort (HCC); Prostate cancer: IntOGen driver in 1 cohort (PRAD); Endometrial cancer: IntOGen driver in 1 cohort (UCEC); Skin cancer: Open Targets association 0.57 with skin cancer (MONDO_0002898); Lung cancer: Open Targets association 0.52 with lung cancer (MONDO_0008903); Breast cancer: Open Targets association 0.51 with breast cancer (MONDO_0007254)

## 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 3 cohorts; CIViC holds 2 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.
- Diseases the sources name that have no OnCo cancer page yet, so they are not linked: Schwannomatosis.

## Sources

- HGNC HGNC:6742: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:6742
- UniProt Q8N653: https://www.uniprot.org/uniprotkb/Q8N653/entry
- NCBI Gene 8216: https://www.ncbi.nlm.nih.gov/gene/8216
- Ensembl ENSG00000099949: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000099949

## 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: [Breast cancer (all types)](https://onco.cc/cancers/breast-cancer/), [Endometrial cancer](https://onco.cc/cancers/endometrial/), [Glioma & glioblastoma](https://onco.cc/cancers/glioblastoma/), [Hepatocellular carcinoma](https://onco.cc/cancers/hcc/), [Lung cancer (all types)](https://onco.cc/cancers/lung-cancer/), [Melanoma](https://onco.cc/cancers/melanoma/), [Prostate cancer](https://onco.cc/cancers/prostate/), [Skin cancer (all types)](https://onco.cc/cancers/skin-cancer/)

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JSON: https://onco.cc/api/v1/entities/lztr1.json