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Title: Conditional inactivation of Pten with EGFR overexpression in Schwann cells models sporadic MPNST
Authors: Keng, WKV 
Watson, AL
Rahrmann, EP
Li, H
Tschida, BR
Moriarity, BS
Choi, K
Rizvi, TA
Collins, MH
Wallace, MR
Ratner, N
Largaespada, DA
Keywords: Animal cell
Animal experiment
Animal model
Gene overexpression
Cell proliferation
Colony formation
Controlled study
Enzyme inactivation
Nerve sheath tumor
Peripheral nerve sheath tumor
Protein depletion
Schwann cell
Issue Date: 2012
Publisher: Hindawi Publishing Corporation
Source: Sarcoma, v. 2012 (620834), p. 1-12 How to cite?
Journal: Sarcoma 
Abstract: The genetic mechanisms involved in the transformation from a benign neurofibroma to a malignant sarcoma in patients with neurofibromatosis-type-1- (NF1-)associated or sporadic malignant peripheral nerve sheath tumors (MPNSTs) remain unclear. It is hypothesized that many genetic changes are involved in transformation. Recently, it has been shown that both phosphatase and tensin homolog (PTEN) and epidermal growth factor receptor (EGFR) play important roles in the initiation of peripheral nerve sheath tumors (PNSTs). In human MPNSTs, PTEN expression is often reduced, while EGFR expression is often induced. We tested if these two genes cooperate in the evolution of PNSTs. Transgenic mice were generated carrying conditional floxed alleles of Pten, and EGFR was expressed under the control of the 2′,3′-cyclic nucleotide 3′phosphodiesterase (Cnp) promoter and a desert hedgehog (Dhh) regulatory element driving Cre recombinase transgenic mice (Dhh-Cre). Complete loss of Pten and EGFR overexpression in Schwann cells led to the development of high-grade PNSTs. In vitro experiments using immortalized human Schwann cells demonstrated that loss of PTEN and overexpression of EGFR cooperate to increase cellular proliferation and anchorage-independent colony formation. This mouse model can rapidly recapitulate PNST onset and progression to high-grade PNSTs, as seen in sporadic MPNST patients.
ISSN: 1357-714X
DOI: 10.1155/2012/620834
Rights: Copyright © 2012 Vincent W. Keng et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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