Analysis of abnormal signaling through KIT and PDGFRA in development of gastrointestinal stromal tumors

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We made a model mouse of human family with multiple gastrointestinal stromal tumors and germline exon 17-type c-kit gene mutation. The heterozygous and homozygous model mice had a cecal GIST-like tumor and hyperplasia of interstitial cells of Cajal at stomach and colon. The phenotypes were similar with those observed in previously reported model mice with exon 11 or exon 13 c-kit gene mutation. A molecular targeted drug, imatinib, which inhibits constitutively activated mutant KIT was given to the model mice, and we observed inhibition of both tumor growth and KIT activation. We also examined an effect of another molecular targeted drug, nilotinib, which inhibits constitutively activated mutant KIT on tumor growth and KIT activation, and now the manuscript is in preparation.