Optic nerve glioma in mice requires astrocyte Nf1 gene inactivation and Nf1 brain heterozygosity

Whereas biallelic neurofibromatosis 1 (NF1) inactivation is observed in NF1-associated gliomas, astrocyte-restricted Nf1 conditional knockout mice do not develop gliomas. These observations suggest that NF1 glioma formation requires additional cellular or genetic conditions. To determine the effect...

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Veröffentlicht in:Cancer research (Chicago, Ill.) Ill.), 2003-12, Vol.63 (24), p.8573-8577
Hauptverfasser: Bajenaru, M Livia, Hernandez, M Rosario, Perry, Arie, Zhu, Yuan, Parada, Luis F, Garbow, Joel R, Gutmann, David H
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container_end_page 8577
container_issue 24
container_start_page 8573
container_title Cancer research (Chicago, Ill.)
container_volume 63
creator Bajenaru, M Livia
Hernandez, M Rosario
Perry, Arie
Zhu, Yuan
Parada, Luis F
Garbow, Joel R
Gutmann, David H
description Whereas biallelic neurofibromatosis 1 (NF1) inactivation is observed in NF1-associated gliomas, astrocyte-restricted Nf1 conditional knockout mice do not develop gliomas. These observations suggest that NF1 glioma formation requires additional cellular or genetic conditions. To determine the effect of an Nf1 heterozygous brain environment on NF1 glioma formation, we generated Nf1+/- mice lacking Nf1 expression in astrocytes. In contrast to astrocyte-restricted Nf1 conditional knockout mice, Nf1+/- mice lacking Nf1 in astrocytes develop optic nerve gliomas. This mouse model demonstrates that Nf1+/- cells contribute to the pathogenesis of gliomas in NF1 and provides a tool for the preclinical evaluation of potential therapeutic interventions for these tumors.
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source MEDLINE; American Association for Cancer Research; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals
subjects Animals
Astrocytes - pathology
Astrocytes - physiology
Brain - metabolism
Brain - physiology
Gene Expression Regulation, Neoplastic
Gene Silencing
Genes, Neurofibromatosis 1 - physiology
Heterozygote
Humans
Male
Mice
Mice, Inbred C57BL
Mice, Knockout
Mice, Transgenic
Neurofibromatosis 1 - complications
Neurofibromatosis 1 - genetics
Neurofibromatosis 1 - pathology
Nf1 gene
Optic Nerve Glioma - genetics
Optic Nerve Glioma - pathology
title Optic nerve glioma in mice requires astrocyte Nf1 gene inactivation and Nf1 brain heterozygosity
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