BRIP1 encodes a DNA helicase that plays a critical role in maintaining chr17 stability through homologous recombination DNA repair 1. The protein functions as a 5'-3' DNA helicase and DNA-dependent ATPase, acting late in the Fanconi anemia pathway after FANCD2 ubiquitination. BRIP1 is involved in repairing DNA double-strand breaks through homologous recombination in association with BRCA1, and can unwind G-quadruplex DNA structures requiring a 5'-single stranded tail 1. Germline pathogenic variants in BRIP1 confer moderate to high cancer susceptibility, particularly for ovarian cancer with an odds ratio of 4.94-11.22 23. BRIP1 mutations are also significantly associated with colorectal cancer risk (OR=3.6) in Japanese populations 4 and contribute to hereditary cancer predisposition 5. The gene is considered a moderate-penetrance cancer susceptibility gene, contributing 2-3% to familial breast cancer cases 6. Clinical testing reveals BRIP1 pathogenic variants are found in approximately 0.9% of ovarian cancer patients compared to 0.09% of controls 2, making it one of the most important ovarian cancer risk genes after BRCA1/2.