We focused on the effects of CXCR4 antagonists, AMD11070 and KRH3955, on the invasiveness and tumorigenicity of GEM-R PaCa cells and determined whether these agents might represent a second line of chemotherapy for GEM-R PaCa cells. The gene discussed is CXCR4; the disease is permanent neonatal diabetes mellitus-pancreatic and cerebellar agenesis syndrome.