Molecular Analysis of Preleukemic and Leukemic Bone Marrow from Children withMonosomy V Syndrome and Juvenile Chronic Myelogenous Leukemia

摘要

The purpose of this study is to understand why children with neurofibromatosis,type 1 (NF1) are at increased risk of developing myeloid leukemia and, in a broader sense, to utilize these cancer cells to generate new knowledge about the role of the NF1 gene (NF1) in growth control. Genetic and biochemical data from our laboratory provide formal proof that NFl acts as a tumor suppressor gene in myeloid cells and regulates growth through its effects on the p21(ras) proteins. Boys with who inherit NF1 from their mothers are especially predisposed to leukemia, and children who receive chemotherapy and radiation for other cancers appear to be at increased risk of secondary leukemia. Finally, our data indicate that the leukemias of some children with no clinical evidence of NF1 carry NFl mutations.