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Two novel mutations in theGAN gene causing giant axonal neuropathy

World Journal of Pediatrics 2018年第3期14卷 298-304页

作者： Monica Irad Normendez-Martínez Lucero Monterde-Cruz Roberto Martínez Magdalena Marquez-Harper Nayelli Esquitin-Garduno Margarita Valdes-Flores Leonora Casas-Avila Valeria Ponce de Leon-Suarez viktor Javier Romero-Díaz Alberto Hidalgo-Bravo Medical Specialties Hospital Boulevard Milenio 130San Carlos la Roncha37660 LeonGuanajuatoMexico Department of Gene tics National Institute of R ehabilitationAvenue Mexico-Xochimilco 28914389 Me xico CityMexico Department ofHis tology School ofMedicineAutonomous University oft heS tate ofN uevo LeonMonterreyMexico

Background Giant axonal neuropathy (GAN) is a rare neurodegenerative disease transmitted in an autosomal recessive mode. This disorder presents motor and sensitive symptoms with an onset in early childhood. Progressive neurodegeneration makes the patients wheelchair dependent by the end of the second decade of life. Affected individuals do not survive beyond the third decade of life. Molecular analysis has identified mutations in the gene GAN in patients with this disorder. This gene produces a protein called gigaxonin which is presumably involved in protein degradation via the ubiquitin-proteasome system. However, the underlying molecular mechanism is not clearly understood yet. Methods Here we present the fi rst patient from Mexico with clinical data suggesting GAN. Sequencing of the GAN gene was carried out. Changes in the nucleotide sequence were investigated for their possible impact on protein function and structure using the publicly available prediction tools PolyPhen-2 and PANTHER. Results The patient is a compound heterozygous carrying two novel mutations in the GAN gene. The sequence analy-sis revealed two missense mutations in the kelch repeats domain. In one allele, a C>T transition was found in exon 9 at the nucleotide position 55393 (g.55393C>T). In the other allele, a transversion G>T in exon 11 at the nucleotide position 67471 (g.67471G>T) was observed. Both of the bioinformatic tools predicted that these amino acid substitutions would have a negative impact on gigaxonin's function. Conclusion This work provides useful information for health professionals and expands the spectrum of disease-causing mutations in the GAN gene and it is the first documented case in Mexican population.

关键词： GAN Genetics Giant axonal neuropathy Incapacity Neurodegenerative

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Passively Q-switched femtosecond-laser-written thulium waveguide laser based on evanescent field interaction with carbon nanotubes

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Photonics Research 2018年第10期6卷 971-980页

作者： ESROM kIFLE PAVEL LOIkO JAVIER RODRíGUEZ VáZQUEZ DE ALDANA CAROLINA ROMERO AIRáN RóDENAS SUN YUNG CHOI JI EUN BAE FABIAN ROTERMUND viktor ZAkHAROV ANDREY VENIAMINOV MAGDALENA AGUILó FRANCESC DíAZ UWE GRIEBNER VALENTIN PETROV XAVIER MATEOS Universitat Rovira i Virgili Departament Quimica Fisica i Inorgànica Fisica i Cristal·lografia de Materials i Nanomaterials (FiCMA-FiCNA)-EMaS Campus Sescelades E-43007 Tarragona Spain ITMO University 49 Kronverkskiy pr. 197101 St. Petersburg Russia Aplicaciones del Láser y Fotónica University of Salamanca 37008 Salamanca Spain Istituto di Fotonica e Nanotecnologie Consiglio Nazionale delle Ricerche (IFN-CNR) Piazza Leonardo da Vinci 32 20133 Milano Italy Department of Physics kAIST 291 Daehak-ro Yuseong-gu 34141 Daejeon South Korea Max Born Institute for Nonlinear Optics and Short Pulse Spectroscopy Max-Bom-Str. 2a D-12489 Berlin Germany

Surface channel waveguides(WGs) were fabricated in a monoclinic Tm^(3+):kLu(WO_4)_2 crystal by femtosecond direct laser writing(fs-DLW). The WGs consisted of a half-ring cladding with diameters of 50 and 60 μm located just beneath the crystal surface. They were characterized by confocal laser microscopy and μ-Raman spectroscopy,indicating a reduced crystallinity and stress-induced birefringence of the WG cladding. In continuous-wave(CW)mode, under Ti:sapphire laser pumping at 802 nm, the maximum output power reached 171.1 mW at 1847.4 nm,corresponding to a slope efficiency η of 37.8% for the 60 μm diameter WG. The WG propagation loss was0.7 0.3 d B∕cm. The top surface of the WGs was spin-coated by a polymethyl methacrylate film containing randomly oriented(spaghetti-like) arc-discharge single-walled carbon nanotubes serving as a saturable absorber based on evanescent field coupling. Stable passively Q-switched(PQS) operation was achieved. The PQS60 μm diameter WG laser generated a record output power of 150 m W at 1846.8 nm with η = 34.6%. The conversion efficiency with respect to the CW mode was 87.6%. The best pulse characteristics(energy/duration)were 105.6 nJ/98 ns at a repetition rate of 1.42 MHz.

关键词：激光技术波导技术发展现状通讯技术

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Decision Support System for Water-User Associations in the Aral Sea Basin

Decision Support System for Water-User Associations in the A...

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国际农业工程大会

作者： Dukhovny viktor A. Tuchin Alexander I. Sokolov Vadim I. 11 Massiv karasu 4 700187 Tashkent Uzbekistan Prof.Director SIC ICWC Chief Division SIC ICWC Manager of project “IWRM-Fergana” PhD

＜正＞ DSS for WUA in Aral Sea basin. Transition to the market economy in Central Asia states accompanied by change of irrigation institutional structure - instead of big collective or state farms a lot of private landowners keep most part of irrigated lands. It requested organization of Water users Associations. Members and staff of WUAs have certain requirements in tools for proper water management. SIC ICWC together with its partners and Lisbon University

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Study of Caspian Goby Neogobius sp. karyotype Flexibility from Several Biotops

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Journal of Life Sciences 2014年第5期8卷 442-446页

作者： Aithazha Bigaliev Saidina kobegenova viktor Vasil'ev Elena Vasil'eva Aiman Imentai Ashan Shametov Department of Molecular Biology and Genetics Al-Farabi Kazakh National University Almaty BOX 050040 Kazakhstan IPEE (Institute of Ecology and Evolution) Russian Academy of Sciences Russia Zoological Museum Moscow State University Moscow Russia

The karyotype of Caspian goby was studied, which has been identified as Caspian bighead goby-Neogobius gorlap before. The results of cytogenetic analyses have shown that the diploid set of goby varies from 38 to 40 chromosomes, which is different from bighead goby （2n = 43-46）. We assumed that Caspian goby has independence as a species.

关键词： Caspian goby diploid number of chromosomes karyotype cytogenetic chromosomal mutation evolution.

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全俄食品生物技术科学研究所在酒精生产领域的科研工作

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酿酒 2003年第1期30卷 27-28页

作者：伍松陵 viktor POLYAkOV Vladimir LEDENEV 黄宇彤国家粮食局科学研究院北京100037 全俄食品生物技术科学研究所莫斯科独联体109033 中国食品发酵工业研究所北京100027

俄国酒精工业某些方面的技术水平处于世界领先的地位。全俄食品生物技术科学研究所主要技术科研方向是 :提高粮食利用率 ,同时开发更加节约原料的工艺和无废物排放生产工艺 ,进一步提高产品质量并改善环境污染。该所在高温、高产酿酒酵... 详细信息

俄国酒精工业某些方面的技术水平处于世界领先的地位。全俄食品生物技术科学研究所主要技术科研方向是 :提高粮食利用率 ,同时开发更加节约原料的工艺和无废物排放生产工艺 ,进一步提高产品质量并改善环境污染。该所在高温、高产酿酒酵母菌种 ,涡流均质 ,快速浓醪发酵 ,霉变粮食脱毒 ,黑麦制酒精 ,蒸馏废水回用。

关键词：食品生物技术科学研究所酒精生产科研工作俄罗斯

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Electromagnetic-Acoustic Transducers for In-Service Manual Testing of Heated Objects

Electromagnetic-Acoustic Transducers for In-Service Manual T...

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17th World Conference on Non-Destructive Testing

作者： Vladimir T.BOBROV Andrey A.SAMOkRUTOV viktor G.SHEVALDYkIN JSC"Spectrum-RII" MoscowRussia

＜正＞The problems of Ultrasonic Testing（UT）of metal products and constructions with the surface being heated up to 300-800C are *** is noted that the process of UT of objects at chemical plants of heat-power, metallurgy industry etc complicates the need to protect Ultrasonic transducers from heated surfaces,hot air and heat radiation from objects,with the operational conditions becoming dangerous. We offer to use electromagnetic-acoustic（EMA）excitation and signal reception technique that does not require direct mechanical contact of the transducer with a heated surface of the object under *** EMA transducers （EMAT）are protected from thermal influence by a thin shield made of heat- resisting material with low thermal conductivity;they do not require the use of couplants and are intended to be used as a part of a serial EMA gauge A1270 for testing of products heated up to 300℃with no additional *** designed time mode follows the *** enables the transducer to work reliably for 8 *** a successful design of the EMAT and the use of a composite protector made of alumina ceramics provide reliable test parameters. The same EMAT can be used to test objects heated up to 700℃.There is a developed carrying-in and -out system for the *** system is provided with a heat-resisting shield that protects the EMAT from thermal influence of the object under test and significantly reduces the influence of it onto an operator. The designed time modes for testing of objects at different temperatures prevents the operating part of the EMAT from overheating and,at the same time provides multiple measurement of heated object *** there is compensation of the thermal influence from an object onto the US wave velocity.

关键词： EMAT Heated products Heat-resistant material US velocity

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Pt Supported on Plasma-Chemical Titanium Nitride for Efficient Room-Temperature CO Oxidation

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Modern Research in Catalysis 2022年第1期11卷 1-11页

作者： David Correa Coyac Eugene kabachkov Eugene kurkin Nikolay Vershinin Igor Balikhin viktor Berestenko Alexandre Michtchenko Yury Shulga Instituto Politécnico Nacional SEPI-ESIME-Zacatenco Av. IPN S/N Ed.5 3-r piso Ciudad de Mexico Mexico Federal Research Center of Problem of Chemical Physics and Medicinal Chemistry Russian Academy of Sciences Chernogolovka Russian Federation Institute of Solid State Physics Russian Academy of Sciences Chernogolovka Russian Federation

Catalysts of carbon monoxide oxidation were synthesized by deposition of platinum on titanium nitride (TiN). Two substrates with an average particle size of 18 and 36 nm were obtained by hydrogen reduction of titanium tetrachloride in a stream of microwave plasma of nitrogen. The surface of the catalysts was studied by X-ray photoelectron spectroscopy (XPS). The data obtained by us in the present work indicate the presence of oxynitride as a transition layer between nitride and oxide. It was found that the CO oxidation rate on the 9 - 15 wt.% Pt loaded TiN catalysts is 120 times higher than that on the platinum black with a specific surface of 30 m2/g. Increase in the reaction rate of CO oxidation on Pt/TiN catalysts as compared to platinum black can be associated with both an increase in the concentration of CO molecules adsorbed and a decrease in the activation energy of the reaction. Catalysts are promising for use in catalytic air purification systems.

关键词： CO Oxidation Titanium Nitride Pt/TiN Catalysts Carbon Monoxide Microwave Plasma Platinum Black X-Ray Photoelectron Spectroscopy

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NPC三电平逆变器中点电压平衡的研究

NPC三电平逆变器中点电压平衡的研究

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作者：维克多（Plotnikov viktor）哈尔滨工业大学

学位级别：硕士

在近几年以来,三电平NPC逆变器被认为是高功耗负载（例如不同电动汽车中的电动机等）的适当应用。三级逆变器属于多级逆变器拓扑,它与两级逆变器有很大的差异,可以认为是两级逆变器的扩展。由于与两级拓扑多电平逆变器相比,工作电压范... 详细信息

在近几年以来,三电平NPC逆变器被认为是高功耗负载（例如不同电动汽车中的电动机等）的适当应用。三级逆变器属于多级逆变器拓扑,它与两级逆变器有很大的差异,可以认为是两级逆变器的扩展。由于与两级拓扑多电平逆变器相比,工作电压范围更大,因此可提供更好的输出特性。通常它们的输出信号更接近正弦波形。由于电压范围的变化,多电平逆变器存在电压不稳定问题。这是这类设备的主要问题。解决这些问题的一种方法是将其他部分集成到拓扑中,另一种方法是显着更改拓扑。第一种方法可能更容易接受,因为包含在拓扑中的零件数量较少。此调查由几个部分组成,因此可以区分几个部分组。第一部分包括:现有的三级逆变器拓扑,三级三相NPC逆变器的建议和数学模型,对现有三级逆变器控制策略的全面综述。第二个步骤包括:定义各种电路参数对三级NPC逆变器工作流程的影响。最后一组致力于中性点电压稳定和减少波动的硬件解决方案的分析。

关键词：三电平NPC逆变器空间矢量PWM 三电平NPC逆变器数学建模正弦PWM

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Modifying action of heavy metal salts on an-ti-inflammatory aspirin action

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Health 2010年第6期2卷 630-633页

作者： Denis R. Husainov viktoriya V. Shylina lvan l korenyuk viktor F. Shulgin 不详

Nowadays pollution of the environment is one of the major problems of mankind. Moreover, studying of the effect of different kinds of med- icine on selected, specially bred, non-exposed to external pollutants animals is becoming distant from reality. Thus in this work we have investigated the modifying action of heavy metals on anti-inflammatory effect of aspirin. The investigation were carried out on rats which were injected during 7 days intraperitoneally by PbCl3, HgCl2, CdCl2 in concentration of 100, 20, 1 mg/kg accordingly, modulating accumulation of the metals in the organism tissues. On the 8 days inflammation was invoked by formalin. As anti-inflammatory medicine was use aspirin. Judging by obtained results the conclusion can be drawn that cadmium in concentration 1 mg/kg significantly increases anti-inflammatory aspirin activity. The observed outcome can be explained in the following way. It is generally known that zinc in a certain concentration demonstrates anti-inflammatory properties. Be- ing an element of the same group cadmium has similar properties and also can have anti-inflammatory action. Lead and mercury suppre- ssed anti-inflammatory aspirin activity. Obvious inhibitory action of mercury and lead salts on aspirin action related to the fact that these elements by themselves were inflammation factors. From the obtained results the following conclusion can be drawn: a definite dose of anti-inflammatory medicine (aspirin) which is sufficient in normal conditions became less effective against the background of accumulation of ions of some heavy metals in an organism.

关键词： Heavy Metals Lead Mercury Cadmium Acetylsalicylic Acid Prostaglandin Inflammation Anti-Inflammatory Activity

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Impact of hospital type on risk-adjusted,traffic-related 30-day mortality:a population-based registry study

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Burns & Trauma 2021年第1期9卷 594-602页

作者： viktor Ydenius Robert Larsen Ingrid Steinvall Denise Bäckström Michelle Chew Folke Sjöberg Department of Biomedical and Clinical Sciences(BVk) Linköping UniversityLinköpingSweden Department of Anaesthesiology and Intensive care Linköping University HospitalSweden Department of Hand Surgery Plastic Surgery and Burns Linköping University HospitalSweden Life Regiment Hussars K3KarlsborgSweden

Background:Traffic incidents are still a major contributor to hospital admissions and trauma-related *** aim of this nationwide study was to examine risk-adjusted traffic injury mortality to determine whether hospital type was an independent survival ***:Data on all patients admitted to Swedish hospitals with traffic-related injuries,based on International Classification of Diseases codes,between 2001 and 2011 were extracted from the Swedish inpatient and cause of death *** the binary outcome measure of death or survival,data were analysed using logistic regression,adjusting for age,sex,comorbidity,severity of injury and hospital *** severity of injury was established using the International Classification of Diseases Injury Severity Score(ICISS).Results:The final study population consisted of 152,693 hospital *** individuals(0–25 years of age)were overrepresented,accounting for 41%of traffic-related *** were overrepresented in all age *** at university hospitals had the lowest mean(SD)ICISS 0.68(0.19).Regional and county hospitals had mean ICISS 0.75(0.15)and 0.77(0.15),respectively,for fatal traffic *** crude overall mortality in the study population was 1193,with a mean ICISS 0.72(0.17).Fatalities at university hospitals had the lowest mean ICISS 0.68(0.19).Regional and county hospitals had mean ICISS 0.75(0.15)and 0.77(0.15),respectively,for fatal traffic *** regional and county hospitals were merged into one group and its risk-adjusted mortality compared with university hospitals,no significant difference was found.A comparison between hospital groups with the most severely injured patients(ICISS0.85)also did not show a significant difference(odds ratio,1.13;95%confidence interval,0.97–1.32).Conclusions:This study shows that,in Sweden,the type of hospital does not influence risk adjusted traffic related mortality,where the most severely injured patients are tran

关键词： Epidemiological International classification of diseases injury severity score Injury Risk-adjusted mortality Trauma

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