| First Author | Hart A | Year | 2000 |
| Journal | Immunity | Volume | 13 |
| Issue | 2 | Pages | 167-77 |
| PubMed ID | 10981960 | Mgi Jnum | J:64178 |
| Mgi Id | MGI:1888830 | Doi | 10.1016/s1074-7613(00)00017-0 |
| Citation | Hart A, et al. (2000) Fli-1 is required for murine vascular and megakaryocytic development and is hemizygously deleted in patients with thrombocytopenia. Immunity 13(2):167-77 |
| abstractText | The ETS gene Fli-1 is involved in the induction of erythroleukemia in mice by Friend murine leukemia virus and Ewings sarcoma in children. Mice with a targeted null mutation in the Fli-1 locus die at day 11.5 of embryogenesis with loss of vascular integrity leading to bleeding within the vascular plexus of the cerebral meninges and specific downregulation of Tek/Tie-2, the receptor for angiopoietin-1. We also show that dysmegakaryopoiesis in Fli-1 null embryos resembles that frequently seen in patients with terminal deletions of 11q (Jacobsen or Paris-Trousseau Syndrome). We map the megakaryocytic defects in 14 Jacobsen patients to a minimal region on 11q that includes the Fli-1 gene and suggest that dysmegakaryopoiesis in these patients may be caused by hemizygous loss of Fli-1. |
