Kobayashi Masato, Kuroki Shiori, Tanaka Yu, Moriya Yukari, Kozutumi Yukari, Uehara Yuji, Ono Kenichiro, Tamura Kyoichi, Washizu Tsukimi, Bonkobara Makoto
Department of Veterinary Clinical Pathology, Nippon Veterinary and Life Science University, Kyonan-cho, Musashino-shi, Tokyo, Japan.
Japan Animal Referral Medical Center, Kuji, Takatsu-ku, Kawasaki-shi, Kanagawa, Japan.
Eur J Haematol. 2015 Dec;95(6):524-31. doi: 10.1111/ejh.12526. Epub 2015 Mar 13.
Although imatinib has therapeutic activity for certain subsets of patients with mastocytosis, it is not always curative. Here, molecular mechanisms that confer imatinib resistance to neoplastic mast cells were investigated using an imatinib-sensitive canine neoplastic mast cell line VI-MC carrying a KIT c.1523A>T activating mutation. Two imatinib-resistant sublines were established by culturing VI-MC cells in increasing concentrations of imatinib (1 μM resistant, rVI-MC1; 10 μM resistant, rVI-MC10). Both sublines had a second KIT mutation c.2443G>C. Recombinant KIT with the second mutation was insensitive to 1 μM but sensitive to 10 μM imatinib. The effect of imatinib on the phosphorylation of KIT and its downstream signalling proteins was then examined using these sublines. KIT and ERK were constitutively phosphorylated in both sublines, and their phosphorylation was suppressed by 10 μM imatinib in rVI-MC1 cells. However, KIT but not ERK phosphorylation was suppressed in rVI-MC10 cells. The phosphorylation of ERK in rVI-MC10 cells was also not diminished by the Src family kinase (SFK) inhibitor dasatinib. This second mutation in KIT may play an important role in imatinib resistance in neoplastic mast cells. Furthermore, KIT/SFK-independent activation of ERK would be involved in imatinib resistance when the neoplastic cells are exposed to higher concentrations of imatinib.
尽管伊马替尼对某些肥大细胞增多症患者亚群具有治疗活性,但并不总是能治愈。在此,使用携带KIT c.1523A>T激活突变的伊马替尼敏感犬肿瘤性肥大细胞系VI-MC,研究了赋予肿瘤性肥大细胞伊马替尼抗性的分子机制。通过在浓度不断增加的伊马替尼(1 μM抗性,rVI-MC1;10 μM抗性,rVI-MC10)中培养VI-MC细胞,建立了两个伊马替尼抗性亚系。两个亚系都有第二个KIT突变c.2443G>C。具有第二个突变的重组KIT对1 μM伊马替尼不敏感,但对10 μM伊马替尼敏感。然后使用这些亚系研究伊马替尼对KIT及其下游信号蛋白磷酸化的影响。在两个亚系中,KIT和ERK均组成性磷酸化,并且在rVI-MC1细胞中,它们的磷酸化被10 μM伊马替尼抑制。然而,在rVI-MC10细胞中,KIT磷酸化被抑制,但ERK磷酸化未被抑制。rVI-MC10细胞中ERK的磷酸化也未被Src家族激酶(SFK)抑制剂达沙替尼减弱。KIT中的这第二个突变可能在肿瘤性肥大细胞的伊马替尼抗性中起重要作用。此外,当肿瘤细胞暴露于更高浓度的伊马替尼时,ERK的KIT/SFK非依赖性激活将参与伊马替尼抗性。