XRCC4

Overview

XRCC4 (X-Ray Repair Cross Complementing 4) encodes a component of the non-homologous end-joining (NHEJ) DNA double-strand break repair complex. In salivary gland adenoid cystic carcinoma (ACC), XRCC4 has been identified as a novel 5’-NFIB fusion partner, implicating NHEJ machinery in the chromosomal rearrangements that drive ACC oncogenesis.

Alterations observed in the corpus

  • Novel 5’-NFIB fusion partner in t(6;9)-positive/MYB-NFIB-negative ACC tumors; XRCC4 fuses its 5’ end (exons 1–2) with NFIB in salivary adenoid cystic carcinoma PMID:26631609

Cancer types (linked)

  • ACYC: Novel gene fusion (XRCC4-NFIB) identified in MYB-NFIB-negative ACC; part of a panel of rare alternative NFIB fusion partners PMID:26631609

Co-occurrence and mutual exclusivity

  • XRCC4-NFIB fusion occurs in t(6;9)-positive tumors that lack the canonical MYB-NFIB fusion, placing it within the MYB-driven ACC molecular subclass PMID:26631609

Therapeutic relevance

Open questions

  • Whether XRCC4-NFIB fusion functions equivalently to MYB-NFIB in driving ACC oncogenesis is uncharacterized; functional studies of this novel fusion have not been reported.

Sources

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