Abstract:
:Previously, we have mimicked human neurofibromatosis type 2 (NF2) in conditional Nf2 mutant (P0Cre;Nf2flox2/flox2) mice. Schwannomas, characteristic for NF2, were found at low frequency in older mice. Here, we report that these mice, upon additional hemizygosity for p53, rapidly develop multiple tumours showing features consistent with malignant peripheral nerve sheath tumours. Thus, p53 hemizygosity promotes tumorigenesis of mutant Nf2 peripheral nerve cells. In contrast, young P0Cre;Nf2flox2/+;p53+/- cis mice mainly succumb to Nf2/p53-related osteogenic tumours. Therefore, Cre-mediated early biallelic loss of Nf2 function in neural crest-derived cells hemizygous for p53 results in resistance to osteogenic tumours and increased susceptibility to peripheral nerve sheath tumours.
journal_name
Oncogenejournal_title
Oncogeneauthors
Robanus-Maandag E,Giovannini M,van der Valk M,Niwa-Kawakita M,Abramowski V,Antonescu C,Thomas G,Berns Adoi
10.1038/sj.onc.1207858subject
Has Abstractpub_date
2004-08-26 00:00:00pages
6541-7issue
39eissn
0950-9232issn
1476-5594pii
1207858journal_volume
23pub_type
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