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研究领域

遗传代谢性疾病基因诊断、病理生理和治疗开发研究;肥胖、糖尿病等代谢异常和代谢综合症的中西医结合研究;慢性疲劳综合症的分子机理、中西医结合治疗法的开发研究

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1.李孟贤, Toshihiro Nakajima, Tomoko Fukushige, Keiko Kobayashi, Nikolaus Seiler, and Takeyori Saheki: 鸟氨酸氨甲酰基转移酶缺乏spf-ash小鼠的氨代谢紊乱及其尿素循环中间氨基酸和鸟氨酸氨基转移酶抑制剂治疗后的保护作用Biochim Biophys Acta 1455:1-11, 1999. 2.Takeyori Saheki, 李孟贤, and Keiko Kobayashi:核转录因子激活蛋白-1(AP-1)在尿素循环酶糖皮质激素诱导方面的拮抗作用:全身性肉碱缺乏JVS小鼠高氨血症的研究. Mol Genet Metab 71:545-551, 2000. 3.Tomotsugu Yasuda, Naoki Yamaguchi, Keiko Kobayashi, Ikumi Nishi, Hidehito Horinouchi, Md. Abdul Jalil, 李孟贤, Miharu Ushikai, Mikio Iijima, and Takeyori Saheki: SLC 25A 13基因两个新突变的同定及其102例成人发病II型瓜氨酸血症病人7种突变的检测. Hum Genet 107:537-545, 2000. 4.Mikio Iijima, Md. Abdul Jalil, Laila Begum, Tomotsugu Yasuda, Naoki Yamaguchi, 李孟贤, Norifumi Kawada, Hitoshi Endou, Keiko Kobayashi, and Takeyori Saheki. 由线粒体溶质载体蛋白缺乏引起的成人发病II型瓜氨酸血症的发病机制:希特林(citrin)蛋白的组织和亚细胞定位. Adv Enzyme Regul 41:325-342, 2001. 5.Yamauchi T, Oike Y, Kamon J, Waki H, Kameda K, Tsuchida A, Date Y, 李孟贤, Miki H, Akanuma Y, Nagai R, Kimura S, Saheki T, Nakazato M, Naitoh T, Yamamura K, and Kadowaki T: Creb结合蛋白缺乏杂合子小鼠中的胰岛素敏感性增加. Nature Genet 30: 221-226, 2002. 6.T. Saheki, K. Kobayashi, M. Iijima, I. Nishi, T. Yasuda, N. Yamaguchi, M.A. Jalil, L. Begum, and李孟贤. 希特林(一种线粒体天门冬氨酸-谷氨酸载体)缺乏的发病机制和病理生理. Metab Brain Dis 17: 335-346, 2002. 7.李孟贤, K. Kobayashi, M. Horiuchi, M.A. Jalil, G. Yoshida, and T. Sakeki: 肉碱缺乏JVS成年小鼠饥饿后所引起的高氨血症. Metab Brain Dis 17: 359-366, 2002. 8.Laila Begum, Md. Abdul Jalil, Keiko Kobayashi, Mikio Iijima, 李孟贤, Tomotsugu Yasuda, Masahisa Horiuchi, Araceli del Arco, Jorgina Satrustegui, and Takeyori Saheki: 希特林、阿那拉和鸟氨酸转运蛋白3种与尿素循环相关的线粒体溶质载体在小鼠体内的表达. Biochim Biophys Acta (Gene Structure and Expression), 1574: 283-292, 2002. 9.Keiko Kobayashi, Yao Bang Lu, 李孟贤, Ikumi Nishi, Kwang-Jen Hsiao, Kyuchul Choeh, Yangling Yang, Wuh-LIang Hwu, Juergen Reichardt, Ferdinando Palmieri, Yoshiyuki Okano, Takeyori Saheki: 东亚地区SLC 25A 13突变基因携带者的高频度发现. Molecular Genetics and Metabolism 80 (2003) 356-359. 10.David S. Sinasac, Mitsuaki Moriyama, M. Abdul Jalil, Laila Begum,李孟贤, Mikio Iijima, Masahisa Horiuchi, Brian H. Robinson, Keiko Kobayashi, Takeyori Saheki, and Lap-Chee Tsui: 成人发病II型瓜氨酸血症疾病模型- slc 25a 13 -基因敲除小鼠具有潜在的代谢异常. Mol Cell Biol 24 (2): 527 2004. 11.Takeyori Saheki, Keiko Kobayashi, Mikio Iijima, Masahisa Horiuchi, Laila Begum, Md.Abdul Jalil, 李孟贤, Yao Bang Lu, Miharu Ushikai, Ayako Tabata, Mitsuaki Moriyama, Kwang-Jen Hsiao and Yangling Yang: 希特林蛋白缺乏所致的成人发病II型瓜氨酸血症和特发性新生儿肝炎. Mol Genet Metab 81:S20-S26 (2004) Apr. 12.Yao Bang Lu, Keiko Kobayashi, Miharu Ushikai, Ayako Tabata, Mikio Iijima,李孟贤, Lei Lei, Kotaro Kawabe, Satoru Taura, Yanling Yang, Tze-Tze Liu, Szu-Hui Chiang, Kwang-Jen Hsiao, Yu-Lung Lau, Lap-Chee Tsui, Dong Hwan Lee, and Takeyori Saheki: 希特林蛋白缺乏症日本患者中同定的SLC 25A 13基因的12个突变在东亚地区的分布及其频度. J Hum Genet. 2005;50(7):338-46. Epub 2005 Jul 30. 13.Goichiro Yoshida 1, 3,李孟贤,Masahisa Horiuchi, Shiro Nakagawa,2 Mie Sakata, Satoshi Kuchiiwa, Toshiko Kushiiwa, Md. Abdul Jalil,1, 2 Laila Begum,1 Yao Bang Lu,1 Mikio Iijima,1 Takeshi Hanada,4 Masamitsu Nakazato,4 Zhi-Li Huang, Naomi Eguchi, Keiko Kobayashi,1 Takeyori Saheki1:禁食引起肉碱缺乏小鼠自主活动减少和食欲素神经元活动减弱. Neurosci Res. 55: 78-86, 2006. 14.李孟贤, Yoshida G, Horiuchi M, Kobayashi K, Saheki T: 单次肉碱治疗显著改善肉碱缺乏JVS小鼠禁食时的自主活动和能量代谢. Biochim Biophys Acta. 1761(10): 1191-9, 2006 Oct. 15.Mitsuaki Moriyama, 李孟贤, Keiko Kobayashi, David S. Sinasac, Yukiko Kannan, Mikio Iijima, Lap-Chee Tsui, Masashi Tanaka, Yoichi Nakamura, and Takeyori Saheki: 丙酮酸改善希特林蛋白缺陷症小鼠的尿素合成. J Hepatol. 44: 930-938,2006. 16.Md. Abdul Jalil, Laila Begum, Laura Contreras, Beatriz Pardo, Mikio Iijima,李孟贤, Milagros Ramos, Patricia Marmol, Masahisa Horiuchi, Kyoko Shimotsu, Shiro Nakagawa, Akiko Okubo, Munefumi Sameshima, Yasushi Isashiki, Araceli del Arco, Keiko Kobayashi, Jorgina Satrústegui, and Takeyori Saheki: 阿那拉(aralar一种脑和肌肉型线粒体天门冬氨酸-谷氨酸载体)缺乏小鼠体内N-乙酰天门冬氨酸水平降低. JBC 280 (35):31333-9 2005. 17.Md. Abdul Jalil, Masahisa Horiuchi, Michiko Wakamatsu, 李孟贤, Laila Begum, Keuko Suzuki, Keiko Kobayashi, Tatsuzo Oka and Takeyori Saheki: 低脂肪饲料可以减轻肉碱缺乏JVS小鼠的心脏肥大. J Biochem.139 (2):263-70, 2006. 18.Takeyori Saheki, Mikio Iijima, 李孟贤, Keiko Kobayashi, Masahisa Horiuchi, Miharu Ushikai, Fumihiko Okumura, Xiao Jian Meng, Ituro Inoue, Atsushi Tajima, Mitsuaki Moriyama, Kazuhiro Eto, Takashi Kadowaki, David S. Sinasac, Lap-Chee Tsui, Mihoko Tsuji, Akira Okano, Tsuyoshi Kobayashi: 希特林/线粒体甘油三磷酸脱氢酶双基因敲除鼠再现人类西特林蛋白缺乏症特征. J Biol Chem. 2007 Jun 25 . 19.杨艳玲,田上泰子,佐伯武赖,小林圭子,李孟贤,花井润师,藤田晃三,袁云,秦炯:成人瓜氨酸血症II一—家系分析。中华医学遗传杂志 20 (5): 380, 2003. 20.佐伯武赖,小林圭子,饭岛干雄,安田智嗣,山口直喜,Jalil MD Abdul, Begum Laila,李孟贤,西育美,崛内正久,今村也寸志:基于希特林(Citrin)蛋白缺乏的成人发病II型瓜氨酸血症的新治疗法的开发 日本临床分子医学会记录(日) 39:44,2002。

学术兼职

日本生化学会、日本先天代谢异常学会、日本人类遗传学学会会员,教育部博士点基金、博士后基金评审专家

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