@article{oai:ir.kagoshima-u.ac.jp:00013106, author = {Kawabata, Naoya and 川畑, 直也 and Ijiri, Kosei and 井尻, 幸成 and Ishidou, Yasuhiro and 石堂, 康弘 and Yamamoto, Takuya and 山元, 拓哉 and Nagao, Hiroko and 永尾, 宗子 and Nagano, Satoshi and 永野, 聡 and Maeda, Shingo and 前田, 真吾 and Komiya, Setsuro and 小宮, 節郎 and Setoguchi, Takao and 瀬戸口, 啓夫}, issue = {4}, journal = {International Journal of Oncology}, month = {}, note = {The Hedgehog pathway functions as an organizer in embryonic development. Recent studies have shown that mutation of the PTCH1 gene involved in the Hedgehog path-way affects rhabdomyosarcoma development. However, the expression of Hedgehog pathway molecules in human rhabdomyosarcoma cells has not been well clarified. In addition, the effect of pharmacological inhibition of the Hedgehog pathway is not known. We investigated the expression of the genes involved in the Hedgehog pathway using human rhabdomyosarcoma cell lines and biopsy specimens. Further, we evaluated the effect of pharmacological inhibition of the Hedgehog pathway using cyclopamine or GANT61 by WST assay, cell proliferation assay and cell death detection assay. Real-time PCR revealed that human rhabdomyosarcoma cell lines and biopsy specimens overexpressed the following genes: Sonic hedgehog, Indian hedgehog, Desert hedgehog, PTCH1, SMO, GLI1, GLI2 and ULK3. Immunohistochemistry revealed that rhabdomyosarcoma cell lines and biopsy specimens expressed SMO and GLI2. Inhibition of SMO by cyclopamine slowed the growth of human rhabdomyosarcoma cell lines. Similarly, inhibition of GLI by GANT61 slowed the growth of human rhabdomyosarcoma cell lines. Inhibition of cell proliferation and apoptotic cell death together prevented the growth of rhabdomyosarcoma cells by cyclopamine and GANT61 treatment. Our findings suggest that pharmacological inhibition of the Hedgehog pathway may be a useful approach for treating rhabdomyosarcoma patients.}, pages = {899--906}, title = {Pharmacological inhibition of the Hedgehog pathway prevents human rhabdomyosarcoma cell growth}, volume = {39}, year = {2011}, yomi = {カワバタ, ナオヤ and イジリ, コウセイ and イシドウ, ヤスヒロ and ヤマモト, タクヤ and ナガオ, ヒロコ and ナガノ, サトシ and マエダ, シンゴ and コミヤ, セツロウ and セトグチ, タカオ} }