TY - JOUR T1 - Disruption of the Cbfa2 gene causes necrosis and hemorrhaging in the central nervous system and blocks definitive hematopoiesis JF - Proceedings of the National Academy of Sciences JO - Proc Natl Acad Sci USA SP - 3444 LP - 3449 DO - 10.1073/pnas.93.8.3444 VL - 93 IS - 8 AU - Wang, Q AU - Stacy, T AU - Binder, M AU - Marin-Padilla, M AU - Sharpe, A H AU - Speck, N A Y1 - 1996/04/16 UR - http://www.pnas.org/content/93/8/3444.abstract N2 - The CBFA2 (AML1) gene encodes a DNA-binding subunit of the heterodimeric core-binding factor. The CBFA2 gene is disrupted by the (8;21), (3;21), and (12;21) chromosomal translocations associated with leukemias and myelodysplasias in humans. Mice lacking a CBF alpha 2 protein capable of binding DNA die between embryonic days 11.5 and 12.5 due to hemorrhaging in the central nervous system (CNS), at the nerve/CNS interfaces of cranial and spinal nerves, and in somitic/intersomitic regions along the presumptive spinal cord. Hemorrhaging is preceded by symmetric, bilateral necrosis in these regions. Definitive erythropoiesis and myelopoiesis do not occur in Cbfa2-deficient embryos, and disruption of one copy of the Cbfa2 gene significantly reduces the number of progenitors for erythroid and myeloid cells. ER -