GNB1L (G protein subunit beta 1 like) is a critical regulator of DNA damage response (DDR) signaling pathways located on chromosome 22.2. Its primary function is to act as a co-chaperone that specifically regulates phosphatidylinositol 3-kinase-related protein kinase (PIKK) family proteins, including ATR and ATM 12. GNB1L promotes the biogenesis of these essential DNA damage checkpoint kinases, which coordinate cellular responses to genotoxic stress and maintain genome stability 2. The gene is highly expressed in the heart 3 and operates in both cytoplasmic and nuclear compartments to regulate DNA damage checkpoint signaling 1. Clinically, GNB1L dysfunction is implicated in 22q11.2 deletion syndrome, where haploinsufficiency associates with increased schizophrenia risk—the third-highest genetic risk factor for this condition 4. Reduced GNB1L expression has been documented in schizophrenia patient prefrontal cortex 5, and missense variants affecting conserved WD40 repeat domains are associated with autism spectrum disorders 6. Additionally, GNB1L exhibits frequent copy number variations in hepatocellular carcinomas, potentially affecting cancer-associated growth signaling pathways 7. These findings establish GNB1L as central to both genome maintenance and neurodevelopmental disease pathogenesis.