Neurofibromatosis type 1 - a model for nervous system tumour formation?
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 nervous system tumours recapitulate several of the unique clinical aspects of the disease. Moreover, these Nf1 GEM models allow for a direct examination of the earliest stages of tumour evolution, including the contributions that Nf1(+/-) cellular elements and cooperating genetic changes make to facilitate the transition from the pre-neoplastic to the neoplastic state and, in some cases, to promote malignant progression.
Rubin
Joshua B
JB
Department of Pediatrics, Washington University School of Medicine and St. Louis Children's Hospital, St. Louis, Missouri 63110, USA.
Gutmann
David H
DH
D000818:Animals; D016514:Genes, Neurofibromatosis 1 / Q000502:physiology; D006801:Humans; D051379:Mice; D023421:Models, Animal; D009423:Nervous System Neoplasms / Q000235:genetics*; D009456:Neurofibromatosis 1 / Q000235:genetics*