title: Modeling neurofibromatosis type 1 tumors in the mouse for therapeutic intervention.
abstract: Von Recklinghausen's neurofibromatosis is a dominantly inherited cancer syndrome. Its gene encodes neurofibromin, a protein with ras GTPase-activating function (rasGAP) and, therefore, all NF1-associated pathology is thought to originate from selective deregulation of the ras pathway. We have constructed a variety of mouse models for NF1 that permit recapitulation of the most common tumors seen in patients. In addition, these mouse models offer insights into tumor origin and into paracrine interactions. Given the molecular and pathological fidelity of the mouse tumors to the human counterparts, it is hoped that these mouse strains will serve as effective tools for therapeutic discovery.
authors.affiliation: Center for Developmental Biology, University of Texas Southwestern Medical Center, Dallas, 75390-9133, USA.
authors.forename: L F
authors.initials: LF
authors.lastname: Parada
authors.forename: C-H
authors.initials: CH
authors.lastname: Kwon
authors.forename: Y
authors.initials: Y
authors.lastname: Zhu
mesh_terms: D000818:Animals; D001254:Astrocytoma / Q000209:etiology / Q000235:genetics; D016543:Central Nervous System Neoplasms / Q000209:etiology / Q000235:genetics; D004195:Disease Models, Animal; D016514:Genes, Neurofibromatosis 1; D006579:Heterozygote; D006801:Humans; D019656:Loss of Heterozygosity; D008407:Mast Cells / Q000502:physiology; D051379:Mice; D008817:Mice, Mutant Strains; D009456:Neurofibromatosis 1 / Q000209:etiology / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D012583:Schwann Cells / Q000502:physiology; D015398:Signal Transduction; D018631:ras Proteins / Q000502:physiology
chemical_list: D018631:ras Proteins