Schwann cell hyperplasia and tumors in transgenic mice expressing a naturally occurring mutant NF2 protein Journal Article
| Authors: | Giovannini, M.; Robanus-Maandag, E.; Niwa-Kawakita, M.; van der Valk, M.; Woodruff, J. M.; Goutebroze, L.; Mérel, P.; Berns, A.; Thomas, G. |
| Article Title: | Schwann cell hyperplasia and tumors in transgenic mice expressing a naturally occurring mutant NF2 protein |
| Abstract: | Specific mutations in some tumor suppressor genes such as p53 can act in a dominant fashion. We tested whether this mechanism may also apply for the neurofibromatosis type-2 gene (NF2) which, when mutated, leads to schwannoma development. Transgenic mice were generated that express, in Schwann cells, mutant NF2 proteins prototypic of natural mutants observed in humans. Mice expressing a NF2 protein with an interstitial deletion in the amino-terminal domain showed high prevalence of Schwann cell-derived tumors and Schwann cell hyperplasia, whereas those expressing a carboxy-terminally truncated protein were normal. Our results indicate that a subset of mutant NF2 alleles observed in patients may encode products with dominant properties when overexpressed in specific cell lineages. |
| Keywords: | protein expression; gene mutation; nonhuman; mouse; animals; mice; animal tissue; membrane proteins; transgenic mouse; animalia; mus musculus; mice, transgenic; tumor suppressor gene; hyperplasia; rats; rodentia; schwann cell; mutagenesis; transgenic mice; neurofibromin 2; rabbits; schwann cells; neurilemmoma; neurinoma; humans; priority journal; article; neurofibromatosis type 2; schwann cell tumors; schwannomin; dominant mutant |
| Journal Title: | Genes and Development |
| Volume: | 13 |
| Issue: | 8 |
| ISSN: | 0890-9369 |
| Publisher: | Cold Spring Harbor Laboratory Press |
| Date Published: | 1999-04-15 |
| Start Page: | 978 |
| End Page: | 986 |
| Language: | English |
| PUBMED: | 10215625 |
| PROVIDER: | scopus |
| PMCID: | PMC316642 |
| DOI/URL: | |
| Notes: | Article -- Export Date: 16 August 2016 -- Source: Scopus |
