TLX1
Overview
TLX1 (T-Cell Leukemia Homeobox 1, also known as HOX11) is a developmental master regulator transcription factor normally repressed by PRC2 (Polycomb Repressive Complex 2). In malignant peripheral nerve sheath tumors (MPNST) with PRC2 loss, TLX1 is aberrantly upregulated due to loss of H3K27me3 at its promoter.
Alterations observed in the corpus
- TLX1 identified as a PRC2-repressed developmental master regulator aberrantly upregulated in PRC2-loss MPNSTs (EED or SUZ12 mutation/deletion); promoter H3K27me3 restored after SUZ12 reintroduction, confirming direct PRC2-mediated repression PMID:25240281
Cancer types (linked)
- MPNST (malignant peripheral nerve sheath tumor) — aberrant upregulation due to PRC2 inactivation (EED/SUZ12 loss) in >90% of sporadic and radiotherapy-associated MPNSTs PMID:25240281
Co-occurrence and mutual exclusivity
- TLX1 upregulation co-occurs with EED or SUZ12 loss and NF1 inactivation in MPNST PMID:25240281
Therapeutic relevance
- TLX1 upregulation is a downstream consequence of PRC2 loss; PRC2 inhibitors are unlikely to benefit MPNST patients since PRC2 acts as a tumor suppressor in this context PMID:25240281
Open questions
Sources
This page was processed by entity-page-writer on 2026-05-11.