rdf:type |
|
lifeskim:mentions |
umls-concept:C0030705,
umls-concept:C0040034,
umls-concept:C0521432,
umls-concept:C0591833,
umls-concept:C1514873,
umls-concept:C1527148,
umls-concept:C1546857,
umls-concept:C1553877,
umls-concept:C1556066,
umls-concept:C1608885,
umls-concept:C1619636,
umls-concept:C1801960,
umls-concept:C1880274
|
pubmed:issue |
2
|
pubmed:dateCreated |
2000-9-25
|
pubmed: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.
|
pubmed:language |
eng
|
pubmed:journal |
|
pubmed:citationSubset |
IM
|
pubmed:chemical |
|
pubmed:status |
MEDLINE
|
pubmed:month |
Aug
|
pubmed:issn |
1074-7613
|
pubmed:author |
|
pubmed:issnType |
Print
|
pubmed:volume |
13
|
pubmed:owner |
NLM
|
pubmed:authorsComplete |
Y
|
pubmed:pagination |
167-77
|
pubmed:dateRevised |
2006-11-15
|
pubmed:meshHeading |
pubmed-meshheading:10981960-Animals,
pubmed-meshheading:10981960-Blood Vessels,
pubmed-meshheading:10981960-Cell Differentiation,
pubmed-meshheading:10981960-DNA-Binding Proteins,
pubmed-meshheading:10981960-Embryonic and Fetal Development,
pubmed-meshheading:10981960-Gene Deletion,
pubmed-meshheading:10981960-Gene Expression Regulation, Developmental,
pubmed-meshheading:10981960-Humans,
pubmed-meshheading:10981960-Megakaryocytes,
pubmed-meshheading:10981960-Mice,
pubmed-meshheading:10981960-Proto-Oncogene Protein c-fli-1,
pubmed-meshheading:10981960-Proto-Oncogene Proteins,
pubmed-meshheading:10981960-Thrombocytopenia,
pubmed-meshheading:10981960-Trans-Activators
|
pubmed:year |
2000
|
pubmed:articleTitle |
Fli-1 is required for murine vascular and megakaryocytic development and is hemizygously deleted in patients with thrombocytopenia.
|
pubmed:affiliation |
Program in Molecular Biology and Cancer, Samuel Lunenfeld Research Institute, Mount Sinai Hospital, Toronto, Ontario, Canada. hart@mshri.on.ca
|
pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
|