title: Nf2 gene inactivation in arachnoidal cells is rate-limiting for meningioma development in the mouse.
abstract: Biallelic NF2 gene inactivation is common in sporadic and in neurofibromatosis type 2 (NF2)-related meningiomas. We show that, beginning at four months of age, thirty percent of mice with arachnoidal cell Cre-mediated excision of Nf2 exon 2 developed a range of meningioma subtypes histologically similar to the human tumors. Additional hemizygosity for p53 did not modify meningioma frequency or progression suggesting that Nf2 and p53 mutations do not synergize in meningeal tumorigenesis. This first mouse model initiated with a genetic lesion found in human meningiomas provides a powerful tool for investigating tumor progression and for the preclinical evaluation of therapeutic interventions.
authors: INSERM U434, Fondation Jean Dausset-Centre d'Etude du Polymorphisme Humain, 75010 Paris, France.
authors: Michel
authors: M
authors: Kalamarides
authors: Michiko
authors: M
authors: Niwa-Kawakita
authors: Hélène
authors: H
authors: Leblois
authors: Vincent
authors: V
authors: Abramowski
authors: Michel
authors: M
authors: Perricaudet
authors: Anne
authors: A
authors: Janin
authors: Gilles
authors: G
authors: Thomas
authors: David H
authors: DH
authors: Gutmann
authors: Marco
authors: M
authors: Giovannini
mesh_terms: D000256:Adenoviridae / Q000235:genetics; D000818:Animals; D001099:Arachnoid / Q000473:pathology / Q000502:physiology*; D020868:Gene Silencing; D005822:Genetic Vectors; D006720:Homozygote; D008577:Meningeal Neoplasms / Q000235:genetics* / Q000473:pathology; D008579:Meningioma / Q000235:genetics* / Q000473:pathology; D051379:Mice; D008817:Mice, Mutant Strains; D009374:Neoplasms, Experimental / Q000235:genetics / Q000473:pathology; D025581:Neurofibromin 2 / Q000235:genetics* / Q000378:metabolism; D016159:Tumor Suppressor Protein p53 / Q000235:genetics
chemical_list: D025581:Neurofibromin 2; D016159:Tumor Suppressor Protein p53