Neurofibromatosis type 1 - a model for nervous system tumour formation?

作者: Joshua B. Rubin , David H. Gutmann

DOI: 10.1038/NRC1653

关键词: Genetically Engineered MouseGenetic ConditionCancer researchNervous systemDiseaseNeurofibromatosisBiologyExtramuralTumor formationMalignant progression

摘要: Neurofibromatosis type 1 (NF1) is a common genetic condition in which affected individuals develop benign and malignant nervous system tumours. Genetically engineered mouse (GEM) models of these NF1-associated tumours recapitulate several the unique clinical aspects disease. Moreover, Nf1 GEM allow for direct examination earliest stages tumour evolution, including contributions that Nf1(+/-) cellular elements cooperating changes make to facilitate transition from pre-neoplastic neoplastic state and, some cases, promote progression.

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