Jun Kido, Johannes Häberle, Keishin Sugawara, Toju Tanaka, Masayoshi Nagao, Takaaki Sawada, Yoichi Wada, Chikahiko Numakura, Kei Murayama, Yoriko Watanabe, Kanako Kojima‐Ishii, Hideo Sasai, Kiyotaka Kosugiyama, Kimitoshi Nakamura,
Abstract Citrin deficiency is an autosomal recessive disorder caused by mutations in the SLC25A13 gene. The disease can present with age‐dependent clinical manifestations: neonatal intrahepatic cholestasis by citrin deficiency (NICCD), failure to thrive, and dyslipidemia by citrin deficiency (FTTDCD), and adult‐onset type II citrullinemia (CTLN2). As a nationwide study to investigate the clinical manifestations, medical therapy, and long‐term outcome in Japanese patients with citrin deficiency, we ...
Tópico(s): Pediatric Hepatobiliary Diseases and Treatments
2022 - Springer Science+Business Media | Journal of Inherited Metabolic Disease
Junichi Sugama, Shunsuke Uchida, Naoya YAMASHIRO, Yusuke Morishima, Tatsuya Hirose, Takahiro Miyazawa, Tomonori Satoh, Yoshiyuki Satoh, Koichi Iinuma, Yoichi Wada, Masahiko Tachibana,
A high temperature high pressure water loop, which can control H2O2 concentration with minimal oxygen (O2) coexistence, has been fabricated. In order to evaluate the effects of hydrogen peroxide (H2O2) on intergranular stress corrosion cracking. Not only static responses, i.e., electrochemical corrosion potential (ECP), of the stainless steel specimens exposed to H2O2 and O2 at elevated temperatures but also their dynamic responses, i.e., frequency dependent complex impedances (FDCI), were measured. ...
Tópico(s): Corrosion Behavior and Inhibition
2004 - Taylor & Francis | Journal of Nuclear Science and Technology
Yohei MURAYAMA, Tomonori Satoh, Shunsuke Uchida, Yoshiyuki Satoh, Shinji Nagata, Toshio SATOH, Yoichi Wada, Masahiko Tachibana,
Abstract The difference in electrochemical corrosion potential of stainless steel exposed to high temperature pure water containing hydrogen peroxide (H2O2) and oxygen (O2)is caused by differences in chemical form of oxide films. In order to identify differences in oxide film structures on stainless steel after exposure to H2O2 and O2 environments, characteristics of the oxide films have been examined by multilateral surface analyses, e.g., X-ray diffraction (XRD), Rutherford back scattering spectroscopy ( ...
Tópico(s): Concrete Corrosion and Durability
2002 - Taylor & Francis | Journal of Nuclear Science and Technology
Yoichi Wada, Atsushi Watanabe, Masahiko Tachibana, Kazushige Ishida, Naohito Uetake, Shunsuke Uchida, Kazuhiko AKAMINE, Mitsuru Sambongi, Shunichi Suzuki, Kenkichi Ishigure,
Abstract In order to determine the effects of hydrogen peroxide on electrochemical corrosion potential (ECP) of type 304 stainless steel (SUS304), ECPs were measured using a high temperature, high pressure water loop with polytetrafluoroethylene (PTFE) inner liner at controlled hydrogen peroxide concentration. It is observed that the ECP of SUS304 exposed to hydrogen peroxide is higher than that when exposed to oxygen at the same oxidant concentration. The ECP shows a hysteresis pattern for its concentration ...
Tópico(s): High Temperature Alloys and Creep
2001 - Taylor & Francis | Journal of Nuclear Science and Technology
Yoichi Wada, Atsushi Watanabe, Masahiko Tachibana, Naohito Uetake, Shunsuke Uchida, Kenkichi Ishigure,
Abstract The stress corrosion cracking (SCC) of structural materials used in boiling water reactors has been studied at relatively low hydrogen peroxide (H2O2) concentrations, around lOppb, which was assumed to be representative of the corrosion environment formed in hydrogen water chemistry (HWC). The 1/4T compact tension specimen was used for measurement of crack growth rates (CGRs) of sensitized type 304 stainless steel in high temperature and high purity water. Crack length was monitored by a reversing ...
Tópico(s): Fatigue and fracture mechanics
2000 - Taylor & Francis | Journal of Nuclear Science and Technology
Shunsuke Uchida, Masahiko Tachibana, Atsushi Watanabe, Yoichi Wada, Naoto Shigenaka, Kenkichi Ishigure,
In order to determine a crack propagation rate of less than 10-8 mm/s in a 24-hour integrated measurement, major parameters of a coupled system of a constant tension specimen and crack depth measurement, based on potential drop method, have been optimized. Influences of sensor geometry, location for detecting potential drop and data processing of the ratio of signal to noise (S/N) were optimized by applying Taguchi's Method. Then a suitable sensor geometry and data processing method were proposed ...
Tópico(s): Fatigue and fracture mechanics
2000 - Taylor & Francis | Journal of Nuclear Science and Technology
Jun Kido, Shirou Matsumoto, Johannes Häberle, Yoko Nakajima, Yoichi Wada, Narutaka Mochizuki, Kei Murayama, Tomoko Lee, Hiroshi Mochizuki, Yoriko Watanabe, Reiko Horikawa, Mureo Kasahara, Kimitoshi Nakamura,
Urea cycle disorders (UCDs) are inherited metabolic disorders with impaired nitrogen detoxification caused by defects in urea cycle enzymes. They often manifest with hyperammonemic attacks resulting in significant morbidity or death. We performed a nationwide questionnaire-based study between January 2000 and March 2018 to document all UCDs in Japan, including diagnoses, treatments, and outcomes. A total of 229 patients with UCDs were enrolled in this study: 73 males and 53 females with ornithine ...
Tópico(s): Neonatal Health and Biochemistry
2021 - Springer Science+Business Media | Journal of Inherited Metabolic Disease
Yoichi Wada, Atsuo Kikuchi, Akimune Kaga, Naoki Shimizu, Junya Ito, Ryo Onuma, Fumiyoshi Fujishima, Eriko Totsune, Ryo Sato, Tetsuya Niihori, Matsuyuki Shirota, Ryo Funayama, Kota Sato, Toru Nakazawa, Keiko Nakayama, Yoko Aoki, Setsuya Aiba, Kiyotaka Nakagawa, Shigeo Kure,
Skin lesions, cataracts, and congenital anomalies have been frequently associated with inherited deficiencies in enzymes that synthesize cholesterol. Lanosterol synthase (LSS) converts (S)-2,3-epoxysqualene to lanosterol in the cholesterol biosynthesis pathway. Biallelic mutations in LSS have been reported in families with congenital cataracts and, very recently, have been reported in cases of hypotrichosis. However, it remains to be clarified whether these phenotypes are caused by LSS enzymatic deficiencies ...
Tópico(s): Pharmacology and Obesity Treatment
2020 - Public Library of Science | PLoS Genetics
Shinya Iwasawa, Atsuo Kikuchi, Yoichi Wada, Natsuko Arai‐Ichinoi, Osamu Sakamoto, Gen Tamiya, Shigeo Kure,
Galactosemia is a metabolic disorder that affects the appropriate metabolism of β-D-galactose. Deficiencies in three of the enzymes of the Leloir pathway, namely, GALT, GALK1, or GALE, are characterized as type I, II, and III galactosemia, respectively. Recently, we reported a novel type of galactosemia (type IV galactosemia) due to biallelic GALM mutations. Genetic diagnosis is indispensable for diagnosing GALM deficiency because no biochemical diagnosis method has been established. Given that apparently ...
Tópico(s): Genomics and Rare Diseases
2019 - Elsevier BV | Molecular Genetics and Metabolism
Yoichi Wada, Atsuo Kikuchi, Natsuko Arai‐Ichinoi, Osamu Sakamoto, Yusuke Takezawa, Shinya Iwasawa, Tetsuya Niihori, Hiromi Nyuzuki, Yoko Nakajima, Erika Ogawa, Mika Ishige, Hiroki Hirai, Hideo Sasai, Ryoji Fujiki, Matsuyuki Shirota, Ryo Funayama, Masayuki Yamamoto, Tetsuya Ito, Osamu Ohara, Keiko Nakayama, Yoko Aoki, S. Koshiba, Toshiyuki Fukao, Shigeo Kure,
ABSTRACT Purpose Galactosemia is caused by metabolic disturbances at various stages of galactose metabolism, including deficiencies in enzymes involved in the Leloir pathway (GALT, GALK1, and GALE). Nevertheless, the etiology of galactosemia has not been identified in a subset of patients. This study aimed to explore the causes of unexplained galactosemia. Methods Trio-based exome sequencing and/or Sanger sequencing was performed in eight patients with unexplained congenital galactosemia. In vitro enzymatic ...
Tópico(s): Biomedical Research and Pathophysiology
2018 - Elsevier BV | Genetics in Medicine
S. Donald Holdsworth, Fabio Scenini, M.G. Burke, G. Bertali, Tsuyoshi Ito, Yoichi Wada, Hideyuki Hosokawa, Nobuyuki Ota, Makoto Nagase,
Oxidation tests on Type 316 stainless steel were performed under hydrogen water chemistry and normal water chemistry for 500 h with continuous injection of a 59Co solution with and without 5 ppb of Zn injection. The present paper identifies the resultant oxides, analysed using analytical electron microscopy and complementary surface microstructural characterisation techniques. Zn injection has been shown to reduce Co incorporation in the inner oxide layer under both water chemistry conditions. The ...
Tópico(s): Electronic and Structural Properties of Oxides
2018 - Elsevier BV | Corrosion Science
Masahiro Kobayashi, Michiko Johnson, Yoichi Wada, Tsuboi Hiromasa, Hideaki Takada, Togo Kenji, Takafumi Kishi, Hidekazu Takahashi, Takeshi Ichikawa, Shunsuke Inoue,
In this paper, we describe a device structure and optical design for a CMOS image sensor with phase-difference detection photodiodes (PD) for autofocus (AF) function. The individual pixel of this image sensor is composed of two horizontally displaced PDs separated by a PN junction. All the effective pixels function as both the imaging and the phase-difference detection AF (PDAF). We have realized a low dark random noise (1.8e- at 1PD, 2.5e- at 1pixel) and high sensitivity (78Ke-/lx.sec at 1green pixel) ...
Tópico(s): Advanced Fluorescence Microscopy Techniques
2016 - | ITE Transactions on Media Technology and Applications
Masahiko Tachibana, Kazushige Ishida, Yoichi Wada, Ryōsuke Shimizu, Nobuyuki Ota, Nobuyoshi Hara,
In order to examine the anodic polarization characteristics of typical structural materials of boiling water reactors (BWRs), the anodic polarization curves of type 316L stainless steel (316L SS) and Alloy 182 were measured in deaerated high purity water at 553 K using the previously reported measurement method which was confirmed suitable for high temperature – high purity water. In order to specify which constituent element determines the dissolution characteristics of these materials, the anodic ...
Tópico(s): Non-Destructive Testing Techniques
2012 - Taylor & Francis | Journal of Nuclear Science and Technology
Kenji Sakai, Norizumi Asano, Yoichi Wada, Shinzo Yoshikado,
The frequency dependences of the relative complex permeability μr*, the relative complex permittivity εr* and the absorption characteristics for composite electromagnetic wave absorbers made of polystyrene resin and sendust particles or permalloy particles were investigated in the frequency range from 1 to 40 GHz. The size of sendust particles was varied between approximately 5 and 20 μm and the particle size dependence of μr* was observed. The value of μr* was shown to be controlled by adjusting ...
Tópico(s): Metamaterials and Metasurfaces Applications
2009 - Elsevier BV | Journal of the European Ceramic Society
Masahiko Tachibana, Kazushige Ishida, Yoichi Wada, Motohiro Aizawa, Motomasa FUSE,
In order to improve the accuracy of electrochemical corrosion potential (ECP) calculations, information about the effects of the flow velocity, the type of oxidization species, the oxide film thickness, and the oxide film classification on the polarization curve is needed. Polarization curve measurements were studied here with that information goal in mind. A wire-shaped working electrode was employed to remove the IR drop caused by high solution resistance, which makes it difficult to apply a proper ...
Tópico(s): Corrosion Behavior and Inhibition
2009 - Taylor & Francis | Journal of Nuclear Science and Technology
Mei‐Ling Qi, Kazuhiko Tagawa, Yasushi Enokido, Natsue Yoshimura, Yoichi Wada, Kei Watase, Shoichi Ishiura, Ichiro Kanazawa, Juan Botas, Minoru Saitoe, Erich E. Wanker, Hitoshi Okazawa,
Tópico(s): Ubiquitin and proteasome pathways
2007 - Nature Portfolio | Nature Cell Biology
Takahiro Miyazawa, Takumi Terachi, Shunsuke Uchida, Tomonori Satoh, Takashi Tsukada, Yoshiyuki Satoh, Yoichi Wada, Hideyuki Hosokawa,
(2006). Effects of Hydrogen Peroxide on Corrosion of Stainless Steel, (V) Characterization of Oxide Film with Multilateral Surface Analyses. Journal of Nuclear Science and Technology: Vol. 43, No. 8, pp. 884-895.
Tópico(s): High-Temperature Coating Behaviors
2006 - Taylor & Francis | Journal of Nuclear Science and Technology
Kazushige Ishida, Yoichi Wada, Masahiko Tachibana, Motohiro Aizawa, Motomasa FUSE, Eiichi Kadoi,
Abstract Hydrazine and hydrogen co-injection into reactor water is considered a new mitigation method of stress corrosion cracking in BWRs. Fundamental data such as the thermal decomposition of hydrazine, the reaction of hydrazine with oxygen and with hydrogen peroxide at temperatures ranging from 150 to 280°C are needed to evaluate suitability of this method. Reactions in bulk water were studied in a polytetrafluoroethylene pipe to separate surface reaction effects. The results were as follows. ( ...
Tópico(s): Hydrogen embrittlement and corrosion behaviors in metals
2006 - Taylor & Francis | Journal of Nuclear Science and Technology
Yunglong Qi, Masataka Hoshino, Yoichi Wada, Shigeki Marubuchi, Natsue Yoshimura, Ichiro Kanazawa, Kenichi Shinomiya, Hitoshi Okazawa,
Summary Mutations of PQBP‐1 (polyglutamine binding protein‐1) have been shown recently to cause human mental retardation accompanied by microcephaly at a high frequency. As a first step towards understanding the molecular basis of this developmental anomaly, we analysed developmental expression of PQBP‐1 by in situ hybridization, immunohistochemsitry and Western blot analysis. Although it had been shown by Northern blot analysis that PQBP‐1 mRNA is expressed in multiple organs in adult mice, our ...
Tópico(s): Mitochondrial Function and Pathology
2005 - Wiley | European Journal of Neuroscience
Takahiro Miyazawa, Shunsuke Uchida, Tomonori Satoh, Yusuke Morishima, Tatsuya Hirose, Yoshiyuki Satoh, Koichi Iinuma, Yoichi Wada, Hideyuki Hosokawa, Naoshi Usui,
Corrosive conditions in BWRs are determined mainly by hydrogen peroxide (H2O2). Then, a high temperature, high-pressure H2O2 water loop was fabricated to identify the effects of H2O2 on corrosion and stress corrosion cracking of stainless steel. By changing concentrations of H2O2 and O2, in situ measurements of electrochemical corrosion potential (ECP) and frequency dependent complex impedance of test specimens were carried out and then characteristics of oxide film on the specimens were determined ...
Tópico(s): Non-Destructive Testing Techniques
2005 - Taylor & Francis | Journal of Nuclear Science and Technology
Shigeki Marubuchi, Yoichi Wada, Tomohiro Okuda, Yukiko Hara, Mei‐Ling Qi, Masataka Hoshino, Masaya Nakagawa, Ichiro Kanazawa, Hitoshi Okazawa,
Abstract Polyglutamine tract‐binding protein‐1 (PQBP‐1) is a nuclear protein that interacts and colocalizes with mutant polyglutamine proteins. We previously reported that PQBP‐1 transgenic mice show a late‐onset motor neuron disease‐like phenotype and cell death of motor neurons analogous to human neurodegeneration. To investigate the molecular mechanisms underlying the motor neuron death, we performed microarray analyses using the anterior horn tissues of the spinal cord and compared gene expression ...
Tópico(s): Metabolism and Genetic Disorders
2005 - Wiley | Journal of Neurochemistry
Hideki Takiguchi, Masahiko Sekiguchi, Ayumi Abe, Kazuhiko AKAMINE, Masanori Sakai, Yoichi Wada, Shunsuke Uchida,
Short period tests of hydrogen water chemistry (HWC) were carried out at Tsuruga-1 and Tokai-2 Nuclear Power Plants. The effects of HWC at Tsuruga-1, a non-jet pump plant, are larger than those at Tokai-2, a jet pump plant; this is caused by the much higher recombination rate of hydrogen and oxygen at the downcomer region under suitable gamma ray irradiation for the former. The effective oxygen concentrations ([O2]eff=[O2] + 1/2[H2O2]) in water measured at the reactor pressure vessel bottom are suppressed ...
Tópico(s): Nuclear Materials and Properties
1999 - Taylor & Francis | Journal of Nuclear Science and Technology
Yoichi Wada, Shunsuke Uchida, Masato Nakamura, Kazuhiko AKAMINE,
Dependency of hydrogen water chemistry (HWC) effectiveness on plant specifications and operational conditions has been studied for an empirical interpretation. The HWC effectiveness was calculated exactly by employing a water radiolysis model requiring results of complex flow analysis and dose distribution analysis as input, in addition to the plant specifications. It was found that decrease of oxygen concentration in the reactor water in the reactor recirculation system could be taken as an exponential ...
Tópico(s): Molten salt chemistry and electrochemical processes
1999 - Taylor & Francis | Journal of Nuclear Science and Technology
Nobuyuki Sodeyama, Kazuyuki Ishida, Kurt A. Jaeckle, L. Zhang, Arata Azuma, Masahito Yamada, Hidehiro Mizusawa, Yoichi Wada,
Previous study has shown that the anti-Hu antibody titre of serum samples from patients with paraneoplastic encephalomyelitis/paraneoplastic sensory neuronopathy (PEM/PSN) was significantly higher than that from patients with small cell lung cancer without neurological disturbances (non-PEM/PSN). The aims of this study were (1) to identify the fine epitopes on HuD recognised by the anti-Hu antibody, (2) to determine if the pattern of epitopic reactivity differed between antibodies from patients with ...
Tópico(s): Cancer, Hypoxia, and Metabolism
1999 - BMJ | Journal of Neurology Neurosurgery & Psychiatry
Shunsuke Uchida, Naoto Shigenaka, Masahiko Tachibana, Yoichi Wada, Masanori Sakai, Kazuhiko AKAMINE, Katsumi Ohsumi,
In order to determine effects of hydrogen peroxide on stress corrosion cracking of structural materials in the primary cooling systems of boiling water reactors, a high temperature high pressure water loop with controlled hydrogen peroxide concentrations and lower possible oxygen concentrations has been fabricated. Test specimens are installed in a stainless steel autoclave which has poly tetra-fluoro-ethylene (PTFE) inner liner to prevent decomposition of hydrogen peroxide on the autoclave surfaces. ...
Tópico(s): Heat Transfer and Boiling Studies
1998 - Taylor & Francis | Journal of Nuclear Science and Technology
Shunsuke Uchida, Naoto Shigenaka, Masahiko Tachibana, Yoichi Wada, Masanori Sakai, Kazuhiko AKAMINE, Katsumi Ohsumi,
In order to determine effects of hydrogen peroxide on stress corrosion cracking of structural materials in the primary cooling systems of boiling water reactors, a high temperature high pressure water loop with controlled hydrogen peroxide concentrations and lower possible oxygen concentrations has been fabricated. Test specimens are installed in a stainless steel autoclave which has poly tetra-fluoro-ethylene (PTFE) inner liner to prevent decomposition of hydrogen peroxide on the autoclave surfaces. ...
Tópico(s): Heat Transfer and Boiling Studies
1998 - Taylor & Francis | Journal of Nuclear Science and Technology
Makoto Michikawa, Yoichi Wada, M. Sano, Toshiki Uchihara, T Furukawa, Hitoshi Shibuya, Hiroshi Tsukagoshi,
Tópico(s): Brain Metastases and Treatment
1991 - Springer Science+Business Media | Neuroradiology
M. Yamada, Shuzo Shintani, K. Mitani, Hideki Kametani, Yoichi Wada, T Furukawa, Hiroshi Tsukagoshi, Kokoro Ozaki, Yoshinobu Eishi, S. Hatakeyama,
Tópico(s): Brain Metastases and Treatment
1988 - Springer Science+Business Media | Journal of Neurology
Takehiko Inui, Yoichi Wada, Moriei Shibuya, Natsuko Arai‐Ichinoi, Yukimune Okubo, Wakaba Endo, Toshihiko Uchida, Noriko Togashi, Etsuo Naito, Kazuhiro Haginoya,
Pyruvate dehydrogenase complex (PDHC) deficiency is an inborn error of metabolism that causes lactic acidosis and neurodevelopmental changes. Five causative genes have been identified: PDHA1, PDHB, DLAT, DLD, and PDHX. Four neurological phenotypes have been reported: neonatal encephalopathy with lactic acidosis, non-progressive infantile encephalopathy, Leigh syndrome, and relapsing ataxia. Of these, neonatal encephalopathy has the worst mortality and morbidity and there is no effective treatment. ...
Tópico(s): Ion Transport and Channel Regulation
2021 - Elsevier BV | Brain and Development
Masahiko Tachibana, Kazushige Ishida, Yoichi Wada, Ryōsuke Shimizu, Nobuyuki Ota, Nobuyoshi Hara,
Cathodic polarization curves of the O2 reduction reaction were measured by using electrodes made from typical structural materials of boiling water reactors (BWRs) to evaluate the effects of kind of material on the electrochemical corrosion potential (ECP) calculation. To estimate ECPs at any region in the BWRs on the basis of the BWR environmental conditions, anodic and cathodic polarization curves should be obtained in advance under relevant conditions. The concentration of oxidants such as O2 and ...
Tópico(s): Corrosion Behavior and Inhibition
2012 - Taylor & Francis | Journal of Nuclear Science and Technology