Abstract:
: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.
journal_name
Immunityjournal_title
Immunityauthors
Hart A,Melet F,Grossfeld P,Chien K,Jones C,Tunnacliffe A,Favier R,Bernstein Adoi
10.1016/s1074-7613(00)00017-0keywords:
subject
Has Abstractpub_date
2000-08-01 00:00:00pages
167-77issue
2eissn
1074-7613issn
1097-4180pii
S1074-7613(00)00017-0journal_volume
13pub_type
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