Disruption of the Cbfa2 gene causes necrosis and hemorrhaging in the central nervous system and blocks definitive hematopoiesis.
Journal:
Proc. Natl. Acad. Sci. U.S.A. 1996 AprAuthors:
Wang Q, Stacy T, Binder M, Marin-Padilla M, Sharpe AH, Speck NA
Abstract
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
...[more] 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.[less]
Mesh Headings:
Animals, Base Sequence, Central Nervous System Diseases, Core Binding Factor Alpha 2 Subunit, DNA Primers, DNA, Complementary, DNA-Binding Proteins, Erythropoiesis, Female, Fetal Death, Gene Targeting, Hematopoiesis, Hemorrhage, Humans, Mice, Mice, Inbred BALB C, Mice, Inbred C57BL, Molecular Sequence Data, Mutation, Necrosis, Neoplasm Proteins, Pregnancy, Proto-Oncogene Proteins, Proto-Oncogenes, Transcription Factors