FRS2
Overview
FRS2 (Fibroblast Growth Factor Receptor Substrate 2) is an adaptor protein that mediates downstream signaling from FGFR. In cancer genomics, FRS2 is located within the 12q15 amplicon recurrently amplified in rhabdomyosarcoma.
Alterations observed in the corpus
- FRS2 located within the 12q15 amplicon amplified in 9% of rhabdomyosarcoma cases, skewed toward fusion-negative (PFN) tumors; co-amplified with MDM2 within this locus PMID:24436047
- FRS2 amplification (96%) is part of the defining 12q13-15 amplicon in dedifferentiated liposarcoma (DDLPS), alongside MDM2 (100%), CDK4 (92%), HMGA2 (76%), and NAV3 (60%) PMID:29100075
Cancer types (linked)
- RMS: 12q15 focal amplification (9%, PFN-skewed) co-amplifying FRS2 and MDM2 PMID:24436047
Co-occurrence and mutual exclusivity
- Co-amplified with MDM2 within the 12q15 amplicon in fusion-negative rhabdomyosarcoma PMID:24436047
Therapeutic relevance
- FRS2 amplification positions the FGF signaling axis as a potential therapeutic target in fusion-negative RMS; FGFR4 is separately recurrently mutated in the same cohort PMID:24436047
Open questions
- Whether FRS2 amplification independently drives RMS or is a co-passenger within the 12q15 MDM2 amplicon requires functional validation PMID:24436047
Sources
This page was processed by crosslinker on 2026-05-09. - PMID:29100075
This page was processed by wiki-cli on 2026-05-15.