Nf1 ; Trp53 mutant mice develop glioblastoma with evidence of strain-specific effects

摘要: Astrocytomas are the leading cause of brain cancer in humans. Because these tumours highly infiltrative, current treatments that rely on targeting tumour mass often ineffective. A mouse model for astrocytoma would be a powerful tool dissecting progression and testing therapeutics. Mouse models have been designed to express oncogenic proteins astrocytes, but had limited success due low penetrance or progression. We present here astrocytomas involving mutation two tumour-suppressor genes, Nf1 Trp53. Humans with mutations NF1 develop neurofibromatosis type I (NF1) increased risk optic gliomas, glioblastomas. The TP53 suppressor is mutated subset at young age progress slowly glioblastoma (termed secondary glioblastomas, contrast primary glioblastomas rapidly de novo). This shows range stages, from low-grade multiforme, may accurately human loss. first reported initiated by loss suppressors, rather than overexpression transgenic oncogenes.