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PMID:10973261

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Citation

Reilly, KM, Loisel, DA, Bronson, RT, McLaughlin, ME and Jacks, T (2000) Nf1;Trp53 mutant mice develop glioblastoma with evidence of strain-specific effects. Nat. Genet. 26:109-13

Abstract

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

Links

PubMed Online version:10.1038/79075

Keywords

Age Factors; Alleles; Animals; Astrocytoma/genetics; Astrocytoma/pathology; Brain Neoplasms/genetics; Brain Neoplasms/pathology; Brain Neoplasms/secondary; Cerebellum/pathology; Culture Techniques; Female; Genes, Neurofibromatosis 1/genetics; Genes, p53/genetics; Genotype; Glioblastoma/genetics; Glioblastoma/pathology; Glioblastoma/secondary; Humans; Immunohistochemistry; Male; Mice; Mice, Mutant Strains; Mice, Nude; Mutation; Necrosis; Neoplasm Transplantation; Nerve Tissue Proteins/biosynthesis; Neurofibromin 1; Pituitary Gland/pathology; Polymerase Chain Reaction; Species Specificity; Tumor Suppressor Protein p53/biosynthesis

Significance

Annotations

Gene product Qualifier GO ID GO term name Evidence Code with/from Aspect Notes Status


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References

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