| pubmed-article:14739366 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:14739366 | lifeskim:mentions | umls-concept:C0026339 | lld:lifeskim |
| pubmed-article:14739366 | lifeskim:mentions | umls-concept:C0025914 | lld:lifeskim |
| pubmed-article:14739366 | lifeskim:mentions | umls-concept:C0026809 | lld:lifeskim |
| pubmed-article:14739366 | lifeskim:mentions | umls-concept:C0026336 | lld:lifeskim |
| pubmed-article:14739366 | lifeskim:mentions | umls-concept:C0349639 | lld:lifeskim |
| pubmed-article:14739366 | lifeskim:mentions | umls-concept:C0027022 | lld:lifeskim |
| pubmed-article:14739366 | lifeskim:mentions | umls-concept:C1274040 | lld:lifeskim |
| pubmed-article:14739366 | lifeskim:mentions | umls-concept:C0085113 | lld:lifeskim |
| pubmed-article:14739366 | lifeskim:mentions | umls-concept:C0596988 | lld:lifeskim |
| pubmed-article:14739366 | lifeskim:mentions | umls-concept:C0002085 | lld:lifeskim |
| pubmed-article:14739366 | lifeskim:mentions | umls-concept:C1701901 | lld:lifeskim |
| pubmed-article:14739366 | pubmed:issue | 4 | lld:pubmed |
| pubmed-article:14739366 | pubmed:dateCreated | 2004-3-22 | lld:pubmed |
| pubmed-article:14739366 | pubmed:abstractText | Neurofibromatosis type one (NF1) is a common genetic disorder affecting 1:4000 births and is characterized by benign and malignant tumors. Children with NF1 are predisposed to juvenile myelomonocytic leukemia. The Nf1 gene encodes neurofibromin, which can function as a Ras GTPase-activating protein. Neurofibromin deficiency in mice leads to mid-gestation lethality due to cardiovascular defects. We have previously shown that conditional inactivation of Nf1 using Tie2-Cre recapitulates the heart defects seen in Nf1(-/-) embryos. Tie2-Cre transgenic mice express Cre recombinase in all endothelial cells. Here, we show that Tie2-Cre-mediated deletion of Nf1 also leads to excision of Nf1 in the hematopoietic lineage. Surviving mice exhibit a myeloproliferative disorder similar to juvenile myelomonocytic leukemia seen in NF1 patients. These mice provide a useful model to study neurofibromin deficiency in hematopoiesis. Furthermore, defects in Tie2-Cre-expressing progenitors that result in heart and blood defects suggest that related heart and blood disorders in NF1 and other syndromes represent disorders of the hemangioblast. | lld:pubmed |
| pubmed-article:14739366 | pubmed:language | eng | lld:pubmed |
| pubmed-article:14739366 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:14739366 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:14739366 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:14739366 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:14739366 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:14739366 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:14739366 | pubmed:month | Apr | lld:pubmed |
| pubmed-article:14739366 | pubmed:issn | 0031-3998 | lld:pubmed |
| pubmed-article:14739366 | pubmed:author | pubmed-author:EpsteinJonath... | lld:pubmed |
| pubmed-article:14739366 | pubmed:author | pubmed-author:ZhuYuanY | lld:pubmed |
| pubmed-article:14739366 | pubmed:author | pubmed-author:PearWarren... | lld:pubmed |
| pubmed-article:14739366 | pubmed:author | pubmed-author:ChoiJohn KJK | lld:pubmed |
| pubmed-article:14739366 | pubmed:author | pubmed-author:KongYiY | lld:pubmed |
| pubmed-article:14739366 | pubmed:author | pubmed-author:GitlerAaron... | lld:pubmed |
| pubmed-article:14739366 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:14739366 | pubmed:volume | 55 | lld:pubmed |
| pubmed-article:14739366 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:14739366 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:14739366 | pubmed:pagination | 581-4 | lld:pubmed |
| pubmed-article:14739366 | pubmed:dateRevised | 2009-11-19 | lld:pubmed |
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| pubmed-article:14739366 | pubmed:meshHeading | pubmed-meshheading:14739366... | lld:pubmed |
| pubmed-article:14739366 | pubmed:year | 2004 | lld:pubmed |
| pubmed-article:14739366 | pubmed:articleTitle | Tie2-Cre-induced inactivation of a conditional mutant Nf1 allele in mouse results in a myeloproliferative disorder that models juvenile myelomonocytic leukemia. | lld:pubmed |
| pubmed-article:14739366 | pubmed:affiliation | Department of Medicine, University of Pennsylvania Health System, Philadelphia, PA 19104, USA. | lld:pubmed |
| pubmed-article:14739366 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:14739366 | pubmed:publicationType | Research Support, U.S. Gov't, P.H.S. | lld:pubmed |
| pubmed-article:14739366 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| entrez-gene:18015 | entrezgene:pubmed | pubmed-article:14739366 | lld:entrezgene |
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