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The Effects of Rufinamide on <i>in Vitro</i>Spinal Muscular Atrophy Model

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Pharmacology & Pharmacy 2019年第4期10卷 159-168页

作者： Shiori Ando Arisu Sato Michinori Funato Kazuki Ohuchi Satoshi Inagaki shinsuke nakamura Masamitsu Shimazawa Hideo Kaneko Hideaki Hideaki Hara Molecular Pharmacology Department of Biofunctional Evaluation Gifu Pharmaceutical University Gifu Japan Department of Clinical Research National Hospital Organization Nagara Medical Center Gifu Japan

Spinal muscular atrophy (SMA) is devastating genetic disease characterized by progressive loss of motor neuron and skeletal muscle weakness. SMA is the most common lethal genetic disease in infancy. SMA is caused by deletion or mutation of SMN1 gene and subsequent lack of SMN protein. Our purpose in this study was to evaluate the therapeutic potential of rufinamide, an antiepileptic drug. In this study, SMA patient-derived fibroblasts and differentiated spinal motor neurons (MNs) using SMA patient-derived iPSCs were used as in vitro SMA model. SMN mRNA was significantly increased by addition of rufinamide in type III SMA patient-derived fibroblasts. Furthermore, rufinamide stimulated neurite elongation in type III SMA patient derived-iPSCs-MNs. In contrast of the result using type III SMA patient-derived fibroblasts, the expression level of SMN mRNA was not changed after rufinamide treatment in type I SMA patient-derived fibroblasts, and rufinamide did not affect neurite outgrowth in type I SMA patients derived-iPSCs-MNs. These findings indicate that rufinamide may be one of the potential candidate drugs for mild type of SMA.

关键词： Spinal Muscular Atrophy Rufinamide iPSCs

Identification of the Stress Which Causes Optineurin Aggregation

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Neuroscience & Medicine 2019年第2期10卷 150-161页

作者： Satoshi Inagaki Michinori Funato Junko Seki Chizuru Kawase Kazuki Ohuchi Shiori Ando shinsuke nakamura Masamitsu Shimazawa Hideo Kaneko Hideaki Hara Molecular Pharmacology Department of Biofunctional Evaluation Gifu Pharmaceutical University Gifu Japan Department of Clinical Research National Hospital Organization Nagara Medical Center Gifu Japan

Glaucoma is a common neurodegenerative disease that can cause blindness and occurs worldwide. Currently, lowering intraocular pressure is the only therapy available to protect retinal ganglion cells (RGCs). However, this therapy does not prevent RGC death in all patients. Therefore, new therapeutic approaches for glaucoma are urgently required, and neuroprotection of RGCs is a focus for many researchers. Optineurin (OPTN) is one of the normal tension glaucoma (NTG) relative genes, while mutant OPTN can form a characteristic aggregation, causing RGC death. Hence, elucidation of the mechanism of OPTN aggregation might provide a clue to help understand RGC death. To examine whether non-mutant OPTN could also aggregate, we pharmacologically induced some glaucoma-related stresses, such as endoplasmic reticulum (ER) stress, glutamate toxicity, activation of TNF-α signaling, mitochondrial dysfunction, and autophagic flux impairment. Our results showed that ER stress, TNF-α signaling, and autophagic flux are involved in OPTN aggregation. Furthermore, our data indicated that increased ER stress, activation of TNF-α signaling, and impaired autophagic flux induce OPTN aggregation, suggesting that OPTN aggregation might be an important therapeutic target not only for familial NTG with mutated OPTN but also for patients with glaucoma more generally.

关键词： Glaucoma Induced Pluripotent Stem Cells Retinal Ganglion Cells Optineurin Protein Aggregation

Present Status of the Nd:YAG Thomson Scattering System Development for Time Evolution Measurement of Plasma Profile on Heliotron J

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Plasma Science and Technology 2013年第3期15卷 240-243页

作者： Takashi MINAMI Shohei ARAI Naoki KENMOCHI Hiroaki YASHIRO Chihiro TAKAHASHI Shinji KOBAYASHI Tohru MIZUUCHI shinsuke OHSHIMA Satoshi YAMAMOTO Hiroyuki OKADA Kazunobu NAGASAKI Yuji nakamura Kiyoshi HANATANI Shigeru KONOSHIMA Fumimichi SANO Institute of Advanced Energy Kyoto University Graduate School of Energy Science Kyoto University Pioneering Research Unit for Next Generation Kyoto University

A new high repetition rate Nd：YAG Thomson scattering system is developed for the Heliotron J helical device. A main purpose of installing the new system is the temporal evolution measurement of a plasma profile for improved confinement physics such as the edge transport barrier （H-mode） or the internal transport barrier of the helical plasma. The system has 25 spatial points with -10 mm resolution. Two high repetition Nd：YAG lasers （〉 550 m J@ 50 Hz） realize the measurement of the time evolution of the plasma profile with ~10 ms time intervals. Scattered light is collected by a large concave mirror （D----800 mm, f/2.25） with a solid angle of -100 mstr and transferred to interference filter polychromators by optical fiber bundles in a staircase form. The signal is amplified by newly designed fast preamplifiers with DC and AC output, which reduces the low frequency background noise. The signals are digitized with a multi-event QDC, fast gated integrators. The data acquisition is performed by a VME-based system operated by the CINOS.

关键词： plasma diagnostic Nd:YAG laser Thomson scattering measurement plasma improved confinement

Determination of Telomere Length by the Quantitative Fluorescence <i>in Situ</i>Hybridization (Q-FISH) Method

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American Journal of Analytical Chemistry 2014年第12期5卷 775-783页

作者： Junko Aida Naotaka Izumiyama-Shimomura Ken-ichi nakamura Naoshi Ishikawa Masanori Terai Yoko Matsuda shinsuke Aida Tomio Arai Kaiyo Takubo Department of Pathology International University of Health and Welfare Mita Hospital Tokyo Japan Department of Pathology Tokyo Metropolitan Geriatric Hospital Tokyo Japan Research Team for Geriatric Pathology Tokyo Metropolitan Institute of Gerontology Tokyo Japan

Telomeres are nucleoprotein complexes located at the ends of eukaryotic chromosomes and are shorten with aging or various causes. Shortened telomere plays an important role for chromosomal instability in carcinogenesis, or a number of diseases in relation to aging. FISH using peptide-nucleic acid (PNA) probe is suitable for analyzing telomeres, because telomere is a part of DNA molecule and localized in chromosomal end in loop shape structure (T-loop). We introduce our telomere analysis for tissue sections and cultured cells in this paper. On the tissue sections, we analyze telomere length with telomere intensity to centromere intensity ratio (TCR), centromere as an inner control. In the cultured cells, we analyze telomere lengths on each chromosome with karyotyping. Those Q-FISH methods by PNA probe are an accurate and reliable tool for research on telomere lengths in various conditions in biology.

关键词： Telomere Telomere Length Q-FISH Tissue Section Chromosome

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