TGFBR3
Overview
TGFBR3 (Transforming Growth Factor Beta Receptor 3), also known as betaglycan, is a co-receptor that binds TGF-β ligands and presents them to the signaling receptors TGFBR1 and TGFBR2. It modulates TGF-β signaling and can act as a tumor suppressor by sequestering ligand. In adenoid cystic carcinoma (ACC), TGFBR3 is a novel structural rearrangement partner for MYB, where super-enhancers downstream of TGFBR3 are translocated to activate MYB expression.
Alterations observed in the corpus
- TGFBR3 identified as a novel MYB rearrangement partner locus in 2/20 adenoid cystic carcinomas (ACC); the downstream region of TGFBR3 contains super-enhancers that translocate to drive MYB overexpression via enhancer hijacking PMID:26829750
Cancer types (linked)
- Adenoid cystic carcinoma (ACC): TGFBR3 structural rearrangement as a non-MYB-NFIB MYB fusion partner in 10% of the discovery cohort; confirms that MYB regulatory activation extends beyond the canonical MYB-NFIB fusion PMID:26829750
Co-occurrence and mutual exclusivity
- MYB-TGFBR3 rearrangement is mutually exclusive with MYB-NFIB fusions within individual tumors (alternative enhancer hijacking mechanisms) PMID:26829750
Therapeutic relevance
- The unifying mechanism in ACC is enhancer hijacking; diagnostic strategies should detect MYB-TGFBR3 rearrangements alongside MYB-NFIB fusions; BET bromodomain inhibition with JQ1 targets the shared MYB-driven enhancer circuit in low-grade ACC PMID:26829750
Open questions
- Whether MYB-TGFBR3 rearrangement tumors respond identically to BET inhibition compared with MYB-NFIB tumors has not been tested PMID:26829750
Sources
This page was processed by crosslinker on 2026-05-14.