ZNF280A is a zinc finger transcription factor located on chromosome 22 that functions as a DNA-binding transcription factor with RNA polymerase II-specific activity 1. Its primary cellular role involves regulating DNA double-strand break (DSB) repair through homologous recombination. Mechanistically, ZNF280A is recruited to DSB sites where it facilitates long-range DNA-end resection by promoting BLM-DNA2 helicase-nuclease complex recruitment, enhancing repair efficiency 1. Additionally, ZNF280A functions as a transcriptional regulator in cancer contexts, promoting oncogenic pathways through interaction with co-factors like CUX2 to activate downstream genes such as ACRV1 and EIF3C 23. Disease relevance is substantial: hemizygous deletion of ZNF280A causes 22q11.2 distal deletion syndrome, characterized by congenital heart disease, immune deficiency, developmental delay, and increased genomic instability 1. Clinically, ZNF280A is significantly upregulated across multiple cancer types (ovarian, lung, bladder) and correlates with poor prognosis, advanced stage, and reduced patient survival 243. In ovarian cancer specifically, ZNF280A drives metabolic reprogramming and aerobic glycolysis via the PI3K/AKT pathway 2. ZNF280A shows promise as a prognostic biomarker and potential therapeutic target 5.