title: Knock-in murine models of familial gastrointestinal stromal tumours.
abstract: In recent years, gastrointestinal stromal tumour (GIST) has emerged from a poorly understood group of mesenchymal tumours to a distinct pathological entity that has become a leading model for new therapies targeting kinases. GIST pathogenesis is driven by receptor tyrosine kinase-activating mutations most often in KIT or PDGFRA that may be sporadic or result in familial GIST syndromes. In a recent issue of The Journal of Pathology (J Pathol 2008;214:302-311), Nakai and colleagues report that mutation of the previously un-modelled tyrosine kinase II domain of KIT generates a spectrum of features very similar to that in two human GIST families. Knock-in mouse models of GIST may prove extremely useful in pre-clinical evaluation of kinase-targeted compounds and of the mechanism of drug resistance, but intriguingly it now seems clearer that the distribution of GIST in mouse models and humans is different.
authors.affiliation: Institute of Pathology, University of Göttingen, Germany. bastian.gunawan@med.uni-goettingen.de
authors.forename: B
authors.initials: B
authors.lastname: Gunawan
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D046152:Gastrointestinal Stromal Tumors / Q000235:genetics*; D018095:Germ-Line Mutation; D006801:Humans; D051379:Mice; D008817:Mice, Mutant Strains; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics*; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D015398:Signal Transduction / Q000235:genetics
chemical_list: D019009:Proto-Oncogene Proteins c-kit