{"entity":{"id":"lztr1","kind":"target","name":"LZTR1","aka":["leucine zipper like post translational regulator 1","Leucine-zipper-like transcriptional regulator 1","LZTR-1","BTBD29"],"tldr":"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.\n\nCIViC 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.","asOf":"2026-09-23","links":[{"label":"HGNC HGNC:6742","url":"https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:6742"},{"label":"UniProt Q8N653","url":"https://www.uniprot.org/uniprotkb/Q8N653/entry"},{"label":"NCBI Gene 8216","url":"https://www.ncbi.nlm.nih.gov/gene/8216"},{"label":"Ensembl ENSG00000099949","url":"https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000099949"}],"tags":["cancer-genes-wave"],"related":["civic","open-targets","intogen"],"cancers":["hcc","prostate","endometrial","skin-cancer","lung-cancer","breast-cancer","glioblastoma","melanoma"],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"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."],"provenance":{"editedBy":"scripts/fetch-cancer-genes.ts (CIViC, Open Targets, IntOGen, HGNC, UniProt)","editedOn":"2026-09-23"},"symbol":"LZTR1","role":["oncogene-driver","tumour-suppressor","biomarker"],"evidenceTier":"clinical-evidence","sources":[{"label":"HGNC HGNC:6742","url":"https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:6742","note":"approved symbol, name, aliases, locus and cross-references (hgnc_complete_set.txt)"},{"label":"UniProt Q8N653","url":"https://www.uniprot.org/uniprotkb/Q8N653/entry","note":"protein name, function text, keywords and locations (REST API)"},{"label":"CIViC gene LZTR1","url":"https://civicdb.org/features/6523","note":"2 evidence items, 0 assertions, 2 variants; diseases: Schwannomatosis (GraphQL API, CC0)"},{"label":"Open Targets ENSG00000099949","url":"https://platform.opentargets.org/target/ENSG00000099949/associations","note":"association with cancer (MONDO_0004992) 0.78; per-cancer scores at or above 0.5: melanoma 0.55, skin cancer 0.57, breast cancer 0.51, lung cancer 0.52 (GraphQL API, CC0)"},{"label":"IntOGen LZTR1","url":"https://www.intogen.org/search?gene=LZTR1","note":"driver in 4 cohorts (Act 1, LoF 3); Compendium_Cancer_Genes.tsv release 20240920, CC0 1.0"}],"hgnc":"HGNC:6742","ensembl":"ENSG00000099949","uniprot":"Q8N653","entrez":"8216","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).","whereFound":["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)"],"targetClass":"tumor-suppressor","prevalence":[]},"route":"/targets/lztr1/","neighbours":{"collection":[{"id":"civic","kind":"collection","name":"CIViC","route":"/collections/civic/"},{"id":"intogen","kind":"collection","name":"IntOGen","route":"/collections/intogen/"},{"id":"open-targets","kind":"collection","name":"Open Targets Platform","route":"/collections/open-targets/"}],"cancer":[{"id":"breast-cancer","kind":"cancer","name":"Breast cancer (all types)","route":"/cancers/breast-cancer/"},{"id":"endometrial","kind":"cancer","name":"Endometrial cancer","route":"/cancers/endometrial/"},{"id":"glioblastoma","kind":"cancer","name":"Glioma & glioblastoma","route":"/cancers/glioblastoma/"},{"id":"hcc","kind":"cancer","name":"Hepatocellular carcinoma","route":"/cancers/hcc/"},{"id":"lung-cancer","kind":"cancer","name":"Lung cancer (all types)","route":"/cancers/lung-cancer/"},{"id":"melanoma","kind":"cancer","name":"Melanoma","route":"/cancers/melanoma/"},{"id":"prostate","kind":"cancer","name":"Prostate cancer","route":"/cancers/prostate/"},{"id":"skin-cancer","kind":"cancer","name":"Skin cancer (all types)","route":"/cancers/skin-cancer/"}]}}