ZNF350

Overview

ZNF350 (also known as ZBRK1) encodes a KRAB-domain zinc finger protein that interacts with BRCA1 and functions as a transcriptional repressor. It has been implicated in DNA damage response pathways through its BRCA1 interaction. Recurrent expressed mutations in rhabdomyosarcoma suggest a candidate role in pediatric sarcoma pathogenesis.

Alterations observed in the corpus

  • Recurrently expressed mutations (2 cases) in a comprehensive rhabdomyosarcoma (RMS) genomic analysis; interacts with BRCA1; flagged as a candidate target requiring functional validation PMID:24436047

Cancer types (linked)

  • RMS: recurrent expressed mutations in pediatric RMS genomic characterization PMID:24436047

Co-occurrence and mutual exclusivity

Therapeutic relevance

  • BRCA1-interacting partner; loss of ZNF350 function may affect homologous recombination DNA repair, with potential relevance to PARP inhibitor sensitivity (not directly established in this corpus).

Open questions

  • Functional role of ZNF350 mutations in RMS is unvalidated; relationship to BRCA1-mediated DNA repair in this tumor type is hypothetical.

Sources

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