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Phenotypes and Chronic Organ Damage May Be Different among Siblings with Wilson's Disease

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Journal of Clinical and Translational Hepatology 2017年第1期5卷 27-30页

作者： shinsuke Yahata Seitetsu Yung Mari Mandai Takakazu Nagahara Daisaku Kuzume Hiroshi Sakaeda Shinya Wakusawa Ayako Kato Yasuaki Tatsumi Koichi Kato Hisao Hayashi Ryohei Isaji Yoji Sasaki Motoyoshi Yano Kazuhiko Hayashi Masatoshi Ishigami Hidemi Goto Division of Community Medicine and Career Development Kobe University Graduate School of Medicine2-1-5Arata-choHyogo-kuKobe 652-0032Japan Department of Gastroenterology Hyogo Prefectural Kakogawa Medical CenterKakogawaHyogoJapan Department of Gastroenterology and Hepatology Tottori Prefectural Kousei HospitalKurayoshiTottoriJapan Departments of Neurology and Gastroenterology Chikamori HospitalKochiJapan Medical Laboratory Sciences Nagoya University Graduate School of MedicineHigashi-kuNagoyaJapan Department of Medicine Aichi Gakuin University School of PharmacyChikusa-kuNagoyaJapan Department of Gastroenterology Konan Kosei HospitalTakaya-choKonanAichiJapan Department of Gastroenterology Yokkaichi Municipal HospitalYokkaichiMieJapan Department of Gastroenterology and Hepatology Nagoya University Graduate School of MedicineShowa-kuNagoyaJapan

Background and Aims:Cloning of ATP7B provided evidence that Wilson's disease is a hepatic copper toxicosis with a variety of extrahepatic *** siblings with the same genetic background and exposure to similar environmental factors may be a good model for the study of genotype-phenotype ***:Twenty-three affected siblings in 11 families were selected from a *** first phenotypes were determined according to the international *** final types of chronic organ damage were re-evaluated for life-long ***:Phenotypes were identical in 5 of the families and different in 6 of the *** acute hepatic phenotype H1 was found in 3 younger siblings and 1 older *** survived an acute episode of hemolysis with underlying chronic liver *** also presented complication with neurological *** neurological phenotype N1 with neuropsychiatric symptoms and hepatic disease was found in 2 aged siblings of 1 family,in an older sibling in 3 families and in the oldest sibling in 1 *** in siblings were mainly split by either H1 occurring in random order or age-dependent *** of chronic organ damage were identical in 8 of the families and different in 3 of the *** same combination of chronic liver disease was found in 6 families and chronic liver disease complicated with neurological disease in 2 *** organ damage in siblings was found when an older sibling was complicated by neurological *** was no reverse combination of a younger sibling being complicated by neurological disease in any of the ***:Phenotype combinations of siblings were mainly modified by externally-induced hemolytic episodes,while chronic organ damage in siblings was split by age-dependent neurological complications.

关键词： Hemolysis Liver disease Neurological disease Wilson's disease

Altered expression of gap junctions, desmosomes and adherens junctions in ischemic rat heart

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Altered expression of gap junctions, desmosomes and adherens...

Third Congress of the Asian-Pacific Organization for Cell Biology

作者： Tsutomu Matsushita Masahito Oyamada shinsuke Masuda Keiichi Yokoyama Kazushi Fujimoto Yuko Yasuda Setsuya Fujita Tetsuro Takamatsu Dept. of Path.and Cell Regul. Kyoto Pref. Univ. Med.Kamikyo-kuKyoto-shiKyoto 602 Japan Dept. of Anat. Kyoto Univ.Sakyo-kuKyoto-shiKyoto 606Japan L. Pasteur Ctr. Med. Res. Sakyo-kuKyoto-shiKyoto 606Japan

Altered distribution of gap junctions in ischemic hearts has been recognized as an important factor in arrhythmogenesis. Gap junctional intercellular communication is influenced by other cell junctions. To investigate

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流行就是“飘逸华丽”

国际服装技术 2007年第3期 111-117页

作者： Masami Yoshida Gong Yunzhe（翻译） Momoko Katsucka（摄影） shinsuke Kojima（摄影）

春装的焦点，时尚界中，飘逸的细节正在大地其道！只要在一个单品中使用，就能够大幅提升时尚感，同时还充满高贵的富贵气息，真是人气非常的高。就让我们来看看眼下最流行的“飘逸华丽”单品吧。

关键词：飘逸时尚春装

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远东的水质污染(上)

交通环保 1991年第5期13卷 52-56页

作者： DavidJ .H.Phillips shinsuke Tanade 鲍建国

本文概述了远东地区所面临的水质污染(重点是沿海水污染)问题。由于社会——经济因素的缘故,该地区的发展中国家和发达国家所面临的污染问题的类型有所不同。在发展中国家,适当的污水处理和排放仍然是一个特殊问题,许多国家工业和和农... 详细信息

本文概述了远东地区所面临的水质污染(重点是沿海水污染)问题。由于社会——经济因素的缘故,该地区的发展中国家和发达国家所面临的污染问题的类型有所不同。在发展中国家,适当的污水处理和排放仍然是一个特殊问题,许多国家工业和和农业废水对海洋资源影响明显。

关键词：水质污染沿海水污染藻花内陆水体工业污水有机废物远东地区有机物负荷发展中国家微生物污染

玩转时尚装扮组合：这就是High＆Low主义!

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国际服装技术 2006年第12期 75-83页

作者： Masako Kondo Jacky Yu（翻译） Yoshihiro Sasaki（摄影） shinsuke Kojima（摄影）

不同于全身平价的简约装扮，也不同于全身高档品的奢华装扮，真正的时尚搭配技巧在于High＆Low价位的完美平衡组合！

关键词：搭配技巧时尚

Recent Progress in Plasma Control Studies on the Improvement of Plasma Performance in Heliotron J

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Plasma Science and Technology 2011年第1期13卷 21-25页

作者： Tohru MIZUUCHI Kazunobu NAGASAKI Hiroyuki OKADA Shinji KOBAYASHI Satoshi YAMAMOTO Takashi MINAMI shinsuke OHSHIMA Masaki TAKEUCHI Kiyofumi MUKAI Hyunyong LEE Linge ZANG Kohta NOMURA Masashige SUWA Kento YAMAMOTO Hiroaki YASHIRO Hayao YOSHINO Shouhei ARAI Tasuku KAGAWA Takayuki MINAMI Kouji MIZUNO Yoshinobu WADA Hiroto WATADA Nobuhiro NISHINO Yosuke NAKASHIMA Kiyoshi HANATANI Yuji NAKAMURA Shigeru KONOSHIMA Fumimichi SANO Institute of Advanced Energy Kyoto Univ. Kyoto University Pioneering Research Unit for Next Generation Graduate School of Energy Science Kyoto University Graduate School of Engineering Hiroshima University Plasma Research Center University of Tsukuba

Recent progress in plasma control studies on the improvement of plasma performance in Heliotron J is reviewed. The supersonic molecular beam injection （SMBI） fueling is successfully applied to Heliotron J plasma. A supersonic H2-beam is effectively injected to increase fueling efficiency and generate a peaked density profile. Local fueling with a short-pulsed SMBI can increase the core plasma density and avoid the degradation arising from edge cooling. Second harmonic electron cyclotron current drive （ECCD） experiments were conducted by launching a focused Gaussian beam with a parallel refractive index of -0.05 ≤ Nil 〈 0.6. Results show that the electron cyclotron （EC） driven current is determined not only by Nil but also by local magnetic field （B） structure where the EC power is deposited. Detailed analysis of the observed NI and B dependences is in progress with a ray-tracing simulation using the TRAVIS code. Fast ion velocity distribution was investigated using fast protons generated by ion cyclotron resonant frequency （ICRF） minority heating. For the standard configuration in Heliotron J, charge ex- change neutral particle analysis （CX-NPA） measurements show higher effective temperature of fast minority protons in the on-axis resonance case compared to that in the HFS （high field side） off-axis resonance case. However, the increase in bulk ion temperature in the HFS resonance case is larger than that in the on-axis resonance.

关键词： plasma control. Heliotron J improved confinement ECH/ECCD NBI ICRF. minority heating fueling control

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最热门连衣裙

国际服装技术 2007年第5期 118-121页

作者： Hitomi Takahashi Hideyo Fukuda（模特）（摄影） shinsuke Kojima（FLAME）（静物）（摄影）

“每一款连衣裙，都好想要啊！”在这个春季，发出如此感慨不足为奇。琳琅满目的各款连衣裙让人目不暇接。本期将倾情介绍其中最受欢迎的几种款式。

关键词：连衣裙款式流行风潮风格

鱼肝油中的有机氯杀虫剂和多氯联苯:北大西洋、挪威海、北海与波罗的海

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AMBIO-人类环境杂志 1994年第Z1期23卷 288-293页

作者： Jerzy Falandysz Kurunthachalam Kannan shinsuke Tanabe Ryo Tatssukawa 周光义波兰格但斯克大学化学系印度泰米尔纳德农业大学日本爱媛大学

选取80年代产自冰岛北大西洋和北大西洋东部海域的鱼肝油,对其中含有的耐分解有机氯化合物浓度进行了测定,以了解有机氯分布的空间变化,跟踪其可能来源和传送到挪威北极的途径。测定的化合物有多氯联苯、滴滴涕、六六六、六氯苯、氯丹... 详细信息

选取80年代产自冰岛北大西洋和北大西洋东部海域的鱼肝油,对其中含有的耐分解有机氯化合物浓度进行了测定,以了解有机氯分布的空间变化,跟踪其可能来源和传送到挪威北极的途径。测定的化合物有多氯联苯、滴滴涕、六六六、六氯苯、氯丹、环氧七氯以及狄氏剂等。产自上述海域的鱼肝油含有机氯浓度很高,尤以波罗的海产品含量最高。各类有机氯含量以多氯联苯和滴滴涕居首位,六氯苯、氯丹、六六六以及狄氏剂的浓度相对较低,并因地而异。空间变化最大的是滴滴涕类,其次是多氯联苯类、六氯苯、狄氏剂和氯丹类。除六六六类以外的所有有机氯,其在不同海域间的变化从统计上看是显著的(5%水平)。尽管由南向北、由近海到远洋有机氯浓度趋于降低,但其滴滴涕、氯丹和六六六的体/代谢物比率在不同取样海区有各自的组成特征。较易传送的滴滴涕、六六六和氯丹体/代谢物,在产自北大西洋遥远海域的鱼肝油中占较大比重,这说明挪威北极和北大西洋温水海域都受到可能来自大西洋东部和南部相同有机氯污染源或污染途径的影响。

关键词：波罗的海有机氯多氯联苯鱼肝油水产动物油挪威海

鱼肝油中的有机氯杀虫剂和多氯联苯:北大西洋、挪威海、北海与波罗的海

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人类环境杂志 1994年第4期23卷 283-293页

作者： Jerzy Falandysz Kurunthachalam Kannan shinsuke Tanabe Ryo Tatssukawa 周光义波兰格但斯克大学化学系印度泰米尔纳德农业大学日本爱媛大学

关键词：鱼肝油农药残留有机氯杀虫剂多氯联苯