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Enzyme replacement therapy in two patients with classic Fabry disease from the same family tree:Two case reports

World Journal of Clinical Cases 2023年第15期11卷 3542-3551页

作者： Yuki Harigane Issei Morimoto O Suzuki Jumpei Temmoku Takayuki Sakamoto Kohichiro Nakamura Kazuo Machii masayuki Miyata Department of Urology Fukushima Medical UniversityFukushima 960-1295Japan Otsuki Sleep Clinic Fukushima 960-8044Japan Department of Diagnostic Pathology Fukushima Medical UniversityFukushima 960-1295Japan Center of Internal Medicine for Rheumatology and Collagen Disease Fukushima Red Cross HospitalFukushima 960-8530Japan Department of Cardiology Fukushima Red Cross HospitalFukushima 960-8530Japan Department of Neurology Fukushima Red Cross HospitalFukushima 960-8530Japan Machii Cardiology and Internal Medicine Clinic Fukushima 960-8530Japan

BACKGROUND The pathophysiology of Fabry disease(FD)-induced progressive vital organ damage is *** progression can be delayed using enzyme replacement therapy(ERT).In patients with classic FD,sporadic accumulation of globotriaosylceramide(GL-3)in the heart and kidney begins in utero;however,until childhood,GL-3 accumulation is mild and reversible and can be restored by *** current consensus is that ERT initiation during early childhood is ***,complete recovery of organs in patients with advanced FD is *** SUMMARY Two related male patients,an uncle(patient 1)and nephew(patient 2),presented with classic *** patients were treated by *** 1 was in his 50s,and ERT was initiated following end-organ damage;this was subsequently *** developed cerebral infarction and died of sudden cardiac *** 2 was in his mid-30s,and ERT was initiated when the patient was diagnosed with FD,during which the damage to vital organs was not overtly *** he had left ventricular hypertrophy at the beginning of this treatment,the degree of hypertrophy progression was limited to a minimal range after>18 years of *** We obtained discouraging ERT outcomes for older patients but encouraging outcomes for younger adults with classic FD.

关键词： Enzyme replacement therapy Fabry disease Pedigree Left ventricular hypertrophy α-galactosidase Case report

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A study of optimal switching problem in limited-cycle with multiple periods

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Journal of Zhejiang University-Science A(Applied Physics & Engineering) 2010年第12期11卷 933-937页

作者： Jing SUN Hisashi YAMAMOTO masayuki MATSUI Graduate School of Engineering Nagoya Institute of Technology Gokiso-cho Showa-ku Nagoya Aichi 466-8555 Japan Department of System Design Management Systems Engineering Tokyo Metropolitan University 6-6 Asahigaoka Hino Tokyo 191-0065 Japan Department of lnformatics Graduate School of lnformatics and Engineering The University of Electro-Communications 1-5-1 Chofugaoka Chofu-shi Tokyo 182-8585 Japan

In a social system or production line,the restrictions of the cost and the due-time exist in each ***,whether these restrictions are satisfied is dependent not only on the risks of this period,but also on the risks generated *** consider controlling the production process by switching the processing rate to a faster one at a given *** paper deals with the optimal switching period to minimize the total expected cost of the production *** first propose the optimal switching period model,and then the mathematic formulation of the total expectation is ***,the policy of optimal switching period is investigated in detail by numerical experiments.

关键词： Limited-cycle problem Multi-period Optimal switching period Production seat system Manufacturing line

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Neuroprotective Effects of 4-phenylbutyric Acid and Its Derivatives： Possible Therapeutics for Neurodegenerative Diseases

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Journal of Health Science 2017年第1期5卷 9-17页

作者： Seisuke Mimori Toru hosoi masayuki Kaneko Koichiro Ozawa Tetsuto Kanzaki Akinori Nishi Yasuyuki Nomura Department of Clinical Medicine Chiba Institute of Science Choshi 288-0025 Japan Department of Pharmacotherapy Graduate School of Biomedical and Health Sciences Hiroshima University Hiroshima734-8553 Japan Department of Biochemistry Institute of Biomedical & Health Sciences Hiroshima University Hiroshima 734-8553 Japan Department of Drug Informatics Graduate School and Faculty of Pharmaceutical Sciences Chiba University Chiba 260-8675Japan Department of Pharmacology Kurume University School of Medicine Kurume 830-0011 Japan

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Molecular design and application of luminescent materials composed of group 13 elements with an aggregation-induced emission property

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National Science Review 2021年第6期8卷 80-93页

作者： Shunichiro Ito masayuki Gon Kazuo Tanaka Yoshiki Chujo Department of Polymer Chemistry Graduate School of EngineeringKyoto University

Complexation of π-conjugated ligands by metal or semimetal ions leads to the enhancement of the planarity and rigidity of π-conjugated systems. Boron, especially, has played a central role in the design of luminescent main-group complexes. However, these complexes still suffer the disadvantage of aggregation-caused quenching as well as typical organic fluorophores. It has recently been reported that some types of boron complexes exhibit the aggregation-induced emission(AIE) property. Moreover, AIE behavior from complexes and organometallic compounds composed of the other group 13 elements, such as aluminum and gallium, has emerged in this decade. These observations greatly encourage us to develop advanced functional materials based on the group 13 elements. Indeed, recent research has demonstrated that these classes of materials are potentially versatile scaffolds for constructing chromic luminophores,efficiently emissive π-conjugated polymers and so on. This review mainly describes AIE-active group 13 complexes with four-coordinate structures and their application as photo-functional materials. Proposed mechanisms of the origins of AIE behavior are briefly discussed.

关键词： aggregation-induced emission crystallization-induced emission chromism conjugated polymer group 13

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Modulation of valosin-containing protein by Kyoto University Substances(KUS) as a novel therapeutic strategy for ischemic neuronal diseases

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Neural Regeneration Research 2017年第8期12卷 1252-1255页

作者： masayuki Hata Hanako Ohashi Ikeda Department of Ophthalmology and Visual Sciences Kyoto University Graduate School of Medicine Neuroprotective Treatment Project for Ocular Diseases Institute for Advancement of Clinical and Translational Science Kyoto University Hospital

Retinal ischemia causes several vision-threatening diseases, including diabetic retinopathy, retinal artery occlusion, and retinal vein occlusion. Intracellular adenosine triphosphate（ATP） depletion and subsequent induced endoplasmic reticulum（ER） stress are proposed to be the underlying mechanisms of ischemic retinal cell death. Recently, we found that a naphthalene derivative can inhibit ATPase activity of valosin-containing protein, universally expressed within various types of cells, including retinal neural cells, with strong cytoprotective activity. Based on the chemical structure, we developed novel valosin-containing protein modulators, Kyoto University Substances（KUSs）, that not only inhibit intracellular ATP depletion, but also ameliorate ER stress. Suppressing ER stress by KUSs is associated with neural cell survival in animal models of several neurodegenerative diseases, such as glaucoma and retinal degeneration. Given that a major pathology of ischemic retinal diseases, other than intracellular ATP depletion, is ER stress-induced cell death, KUSs may provide a novel strategy for cell protection in ischemic conditions. Hence, we investigated the efficacy of KUS121 in a rat model of retinal ischemic injury. Intravitreal injections of KUS121, which is clinically preferable route of drug administration in retinal diseases, significantly suppressed inner retinal thinning and retinal cell death, and maintained visual functions. Valosin-containing protein modulation by KUS is a promising novel therapeutic strategy for ischemic retinal diseases.

关键词： adenosine triphosphatase C/EBP homologous protein central retinal artery occlusion endoplasmicreticulum stress neuroprotective therapy retinal ganglion cell

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Influence of Microstructures on Aerodynamic Characteristics for Dragonfly Wing in Gliding Flight

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Journal of Bionic Engineering 2019年第3期16卷 423-431页

作者： Sheng Zhang masayuki Ochiai Yuta Sunami Hiromu Hashimoto Micro/Nano Technology Center Tokai University 4-1-1 Kitakaname Hiratsuka-city Kanagawa 259-1292 Japan Department of Mechanical Engineering Tokai University 4-1-1 Kitakaname Hiratsuka-city Kanagawa 259-1292 Japan

In this paper, the functionalities of microstructures for dragonfly wing during gliding flight are investigated. Three dragonfly-mimic airfoil-shaped wings with hybrid structures were designed and fabricated as: flat wing, zigzag-edged wing and zigzag-edged wing with pillar structure. Based on the wind tunnel experiments, the zigzag-edged wing structure significantly reduces the drag force in the gliding flight. Moreover, the drag reduction is more effective on the combination of the surface pillar and zigzag-edged structure. In addition, the zigzag-edged wing structure has less influence of Karman vortex street, and the surface pillars reduce the frictional drag and stabilized the streamline in the lower vortex region. Overall, the microstructure of the dragonfly wing is an important element in the aerodynamic study. These findings can enhance the knowledge of insect-mimic wing structure and facilitate the application of Micro Air Vehicle (MAV) in the gliding flight.

关键词： dragonfly wing vein structure aerodynamics gliding biomimetics

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A new approach to retrieve leaf normal distribution using terrestrial laser scanners

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Journal of Forestry Research 2016年第3期27卷 631-638页

作者： Shengye Jin masayuki Tamura Junichi Susaki Graduate School of Global Environmental Studies Kyoto University C1-1-209 Kyoto Daigaku Katsura Nishikyo-kuKyoto 615-8540 Japan Department of Civil and Earth Resources Engineering Kyoto University C1-1-209 Kyoto Daigaku Katsura Nishikyo-kuKyoto 615-8540 Japan

Leaf normal distribution is an important structural characteristic of the forest canopy. Although terrestrial laser scanners（TLS） have potential for estimating canopy structural parameters, distinguishing between leaves and nonphotosynthetic structures to retrieve the leaf normal has been challenging. We used here an approach to accurately retrieve the leaf normals of camphorwood（Cinnamomum camphora） using TLS point cloud ***, nonphotosynthetic structures were filtered by using the curvature threshold of each point. Then, the point cloud data were segmented by a voxel method and clustered by a Gaussian mixture model in each voxel. Finally, the normal vector of each cluster was computed by principal component analysis to obtain the leaf normal distribution. We collected leaf inclination angles and estimated the distribution, which we compared with the retrieved leaf normal distribution. The correlation coefficient between measurements and obtained results was 0.96, indicating a good coincidence.

关键词： Leaf normal distribution Leaf inclinationangle Terrestrial laser scanner Point cloud data Curvature - Clustering

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Rapid growth of nanostructure on tungsten thin film by exposure to helium plasma

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Plasma Science and Technology 2023年第4期25卷 1-4页

作者： Shuangyuan FENG Shin KAJITA masayuki TOKITANI Daisuke NAGATA Noriyasu OHNO National Institute for Fusion Science National Institutes of Natural SciencesTokiGifu 509-5292Japan Graduate School of Frontier Sciences The University of TokyoKashiwaChiba 277-8561Japan Graduate School of Engineering Nagoya UniversityNagoya 464-8603Japan

A fiberform nanostructure was synthesized by exposing high-density helium plasma to a 100 nm thick tungsten thin film in the linear plasma device *** helium plasma exposure,the cross-section of samples was observed by a scanning electron microscope,transmission electron microscope,and focused ion beam scanning electron *** is shown that the thickness of the nanostructured layer increases significantly for only a short irradiation *** optical absorptivity remains high,even though it is exposed to helium plasma for a short *** usage of the thin film can shorten the processing time for nanostructure growth,which will be beneficial for commercial production.

关键词： helium plasma fiberform nanostructure rapid growth

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Biomarkers of graft-vs-host disease:Understanding and applications for the future

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World Journal of Transplantation 2021年第8期11卷 335-343页

作者： masayuki Nagasawa Department of Pediatrics Musashino Red Cross HospitalMusashino City 180-8610TokyoJapan

Hematopoietic stem cell transplantation(HSCT)is widely performed as a treatment for malignant blood disorders,such as *** achieve good clinical outcomes in HSCT,it is necessary to minimize the unfavorable effects of acute graft-vs-host disease(GVHD)and induce the more tolerable,chronic form of the *** better management of GVHD,sensitive and specific biomarkers that predict the severity and prognosis of the disease have been intensively investigated using proteomics,transcriptomics,genomics,cytomics,and tandem mass spectrometry ***,I will briefly review the current understanding of GVHD biomarkers and future prospects.

关键词： Graft-vs-host disease Hematopoietic stem cell transplantation Biomarker Cytokine Graft-vs-host reaction Organ damage

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Synthetic Study on Polyhydroxyestrogens Modeled for Inagami-Tamura Endogenous Digitalis-like Factor and Their Activity

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复旦学报（自然科学版） 2007年第5期46卷 603-604页

作者： masayuki Sakakibara Lab of Pharmaceutical Resource Discovery Dalian Institute of Chemistry and Physics

1 Results Mammalian possess receptors for digitalis,cardiac glycosides,derived from *** one of the results from studies on a real endogenous ligand for the receptors,so called an endogenous digitalis-like factor (EDF) or substance (EDS),Inagami and Tamura isolated a promising candidate possessing a molecular weight,*** elucidated several possible structures (Fig.1) for the factor and synthesized several modeled compounds,some of which exhibited remarkable constriction at higher concentrations t...

关键词： inagami-tamura endogenous digitalis-like factor (EDF) activity

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