Linked Life Data

16180234

Source:http://linkedlifedata.com/resource/pubmed/id/16180234

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
2005-10-24
pubmed:abstractText
Neurofibromatosis type 1 (NF1) is one of the most common dominantly inherited genetic diseases associated with the nervous system. Functional loss of the NF1 tumor suppressor is frequently associated with the generation of benign neurofibromas that can progress to malignancy. Recent evidence in genetic mouse models indicates that the development of neurofibromas requires a loss of Nf1 in the cells destined to become neoplastic as well as heterozygosity in nonneoplastic cells. We tested this hypothesis in a newly developed syngraft mouse model in which Nf1-/- Schwann cells isolated from knockout embryos were grafted into the sciatic nerves of Nf1+/- mice, corresponding to the genetic background of NF1 patients. Furthermore, we also characterized in vitro growth of these cells. We found that embryonic mouse Nf1-/- Schwann cells exhibit increased proliferation and less growth factor-dependence in vitro compared with heterozygous and wild-type counterparts. Moreover, Nf1-/- Schwann cells showed tumorigenic growth when implanted into nerve of adult Nf1 heterozygous mice. These findings support the conclusion that loss of Nf1 in embryonic mouse Schwann cells is sufficient for tumor development in the heterozygous environment of adult mouse nerve. In addition, this syngraft model provides a practical means for the controlled induction of neurofibromas, greatly facilitating localized application of therapeutic agents and gene delivery.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Nov
pubmed:issn
0360-4012
pubmed:author
pubmed:copyrightInfo
Copyright (c) 2005 Wiley-Liss, Inc.
pubmed:issnType
Print
pubmed:day
1
pubmed:volume
82
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
357-67
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
2005
pubmed:articleTitle
Tumorigenic properties of neurofibromin-deficient Schwann cells in culture and as syngrafts in Nf1 knockout mice.
pubmed:affiliation
Department of Pediatrics, Division of Neurology, University of Florida College of Medicine, Gainesville, Florida 32610, USA.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, Non-P.H.S.