LZTR1

Overview

LZTR1 (Leucine-Zipper-Like Transcription Regulator 1) is a putative transcriptional regulator located at 22q11.21. It was identified as a novel significantly mutated gene in glioblastoma by the TCGA 2013 multi-platform analysis, with hemizygous deletion observed in the majority of cases with copy-number alteration data. LZTR1 is also known to be involved in RAS regulation and is a germline predisposition gene for schwannomatosis.

Alterations observed in the corpus

  • Novel significantly mutated gene (SMG) identified by MutSig/InVEx in GBM (n=291 WES); putative transcriptional regulator at 22q; hemizygous deletion in 5/6 samples with CNA data PMID:24120142
  • Single-case missense p.Gly404Arg mutation detected in the sequenced AdCC cohort; observed among the recurrently mutated minor genes in sinonasal adenoid cystic carcinoma PMID:24418857
  • Recurrent germline pathogenic/likely-pathogenic variant in the Indian familial NSCLC cohort (Rastogi et al.), alongside ATM, CHEK2, BAP1, FANCA, FANCI, FANCM, and XRCC3 PMID:27346245

Cancer types (linked)

Co-occurrence and mutual exclusivity

  • No co-occurrence or mutual exclusivity data currently in corpus.

Therapeutic relevance

  • No direct therapeutic data currently in corpus.

Open questions

  • Functional mechanism of LZTR1 loss in GBM pathogenesis is uncharacterized.
  • Relationship between somatic LZTR1 mutations in GBM and germline LZTR1 mutations in schwannomatosis is unexplored.

Sources

This page was processed by entity-page-writer on 2026-05-15. - PMID:24418857

This page was processed by entity-page-writer on 2026-05-15. - PMID:27346245

This page was processed by entity-page-writer on 2026-05-15.