检索结果-南通市图书馆

genetic Analysis of 17 Children with Hunter Syndrome:Identification and Functional Characterization of Four Novel Mutations in the Iduronate-2-Sulfatase gene

引用

Journal of genetics and genomics 2014年第4期41卷 197-203页

作者： Dimitry A.Chistiakov Lyudmila M.Kuzenkova Kirill V.Savost’anov Anait K.gevorkyan Alexander A.Pushkov alexey g.nikitin Nato D.Vashakmadze Natalia V.Zhurkova Tatiana V.Podkletnova Leila S.Namazova-Baranova Alexander A.Baranove Department of Medical Nanobiotechnology Pirogov Russian State Medical University Department of Molecular and genetic Diagnostics Division of Laboratory MedicineInstitute of PediatricsResearch Center for Children’s Health Department of Psychoneurology and Psychosomatic Pathology Institute of PediatricsResearch Center for Children’s Health Institute of Preventive Pediatrics and Rehabilitation Research Center for Children’s Health Research Center for Children’s Health

Mucopolysaccharidosis type II （MPS II） is a rare X-linked disorder caused by alterations in the iduronate-2-sulfatase （IDS） gene. In this study, IDS activity in peripheral mononuclear blood monocytes （PMBCs） was measured with a fluorimetric enzyme assay. Urinary glycosaminoglycans （gAgs） were quantified using a colorimetric assay. All IDS exons and intronic flanks were bidirectionally sequenced. A total of 15 mutations （all exonic region） were found in 17 MPS II patients. In this cohort of MPS II patients, all alterations in the IDS gene were caused by point nucleotide substitutions or small deletions. Mutations ***88His and ***172＊ occurred twice. All mu- tations were inherited except for ***489Alafs＊7, a germline mutation. We found four new mutations （***142Phe, ***233gly, ***430＊, and ***360Tyrfs＊31）. In Epstein-Barr virus （EBV）-immortalized PMBCs derived from the MPS II patients, no IDS protein was detected in case of the ***142Phe and ***360Tyrfs＊31 mutants. For ***233gly and ***430＊, we observed a residual expression of IDS. The ***233gly and ***430＊ mutants had a residuary enzymatic activity that was lowered by 14.3 and 76-fold, respectively, compared with healthy controls. This observation may help explain the mild disease phenotype in MPS II patients who had these two mutations whereas the ***142Phe and ***360Tyrfs＊31 mutations caused the severe disease manifestation.

关键词： Hunter syndrome Mucopolysaccharidosis type II Iduronate-2-sulfatase Mutations glycosaminoglycans

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Previously unknown behavior in parasitic cuckoo females:male-like vocalization during migratory activity

引用

Avian Research 2021年第1期12卷 1-16页

作者： Swetlana g.Meshcheryagina alexey Opaev Institute of Plant and Animal Ecology Ural Branch of the Russian Academy of SciencesEkaterinburgRussia A.N.Severtsov Institute of Ecology and Evolution Russian Academy of SciencesMoscowRussia

Background:In the last decade,enigmatic male-like cuckoo calls have been reported several times in East *** calls exhibited a combination of vocal traits of both Oriental Cuckoo(Cuculus optatus)and Common Cuckoo(Cuculus canorus)advertising calls,and some authors therefore suggested that the enigmatic calls were produced by either Common×Oriental Cuckoo male hybrids or Common Cuckoo males having a gene ***,the exact identity of calling birds are still ***:We recorded previously unknown male-like calls from three captive Oriental Cuckoo females,and compared these calls with enigmatic vocalizations recorded in the wild as well as with advertising vocalizations of Common and Oriental Cuckoo *** achieve this,we measured calls ***,we video-recorded captive female emitting male-like calls,and compared these recordings with the YouTube recordings of calling males of both Common and Oriental Cuckoos to get insight into the mechanism of call ***:The analysis showed that female male-like calls recorded in captivity were similar to enigmatic calls recorded in the ***,Oriental Cuckoo females might produce the latter *** features of these female calls appeared to be unusual among ***,females produced male-like calls at the time of spring and autumn migratory activity and on migration in the *** of this,functional significance of this call remained ***,the male-like female call unexpectedly combined features of both closed-mouth(closed beak and simultaneous inflation of the‘throat sac')and open-mouth(prominent harmonic spectrum and the maximum neck extension observed at the beginning of a sound)vocal ***:The Cuculus vocalizations outside the reproductive season remain poorly ***,we found for the first time that Oriental Cuckoo females can produce male-like calls in that *** of its rarity,this call might be an ***,fe

关键词： Closed-mouth vocal behavior Cuckoo call Cuculus optatus Female song Male-like vocalization

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

On Splitting of the Normalizer of a Maximal Torus in E6(q)

引用

Algebra Colloquium 2019年第2期26卷 329-350页

作者： alexey galt alexey Staroletov Sobolev Institute of Mathematics Novosibirsk Russia

Let g be a finite group of Lie type E6 over Fq (adjoint or simply connected) and W be the Weyl group of g. We describe maximal tori T such that T has a complement in its algebraic normalizer N(g, T). It is well known that for each maximal torus T of g there exists an element w ∈ W such that N(g, T )/T ■ CW(w). When T does not have a complement isomorphic to CW(w), we show that w has a lift in N(g, T) of the same order.

关键词： finite group of Lie type maximal torus algebraic normalizer split extension

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Polariton polarization rectifier

引用

Light(Science & Applications) 2019年第1期8卷 505-514页

作者： Evgeny S.Sedov Yuri g.Rubo alexey V.Kavokin Westlake University 18 Shilongshan RoadHangzhou 310024 Zhejiang ProvinceChina Institute of Natural Sciences Westlake Institute for Advanced Study18 Shilongshan RoadHangzhou 310024 Zhejiang ProvinceChina Vladimir State University Gorky str.87Vladimir 600000Russia Instituto de Energias Renovables Universidad Nacional Autonoma de Mexico62580 TemixcoMORMexico Spin Optics Laboratory Saint Petersburg State University1 UlianovskayaSt.Petersburg 198504Russia

We propose a novel photonic device,the polariton polarization rectifier,intended to transform polariton pulses with arbitrary polarization into linearly polarized pulses with controllable orientation of the polarization *** is based on the interplay between the orbital motion of the polariton wave packet and the dynamics of the polariton pseudospin governed by the spatially dependent effective magnetic *** latter is controlled by the TE-TM splitting in a harmonic *** show that the unpolarized polariton pulse acquires linear polarization in the course of propagation in a harmonic *** gives the considered structure an extra function as a linear polarizer of polariton pulses.

关键词： field polarized harmonic

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Roof model recommendation for complex buildings based on combination rules and symmetry features in footprints

引用

International Journal of Digital Earth 2018年第10期11卷 1039-1063页

作者： Xuke Hu Hongchao Fan alexey Noskov gIScience Research group Institute of GeographyHeidelberg UniversityHeidelbergGermany School of Remote Sensing and Information Engineering Wuhan UniversityWuhanPeople’s Republic of China

Currently,very few roof shape information for complex buildings is available on ***,additional data requirements(e.g.3D point clouds)limit the applicability of many roof reconstruction *** mitigate this issue,we propose an approach to roof shape recommendations for complex buildings by exploring the inherited characteristics of building footprints:the disclosure of rectangles combinations in a partition of footprints and the symmetrical features of ***,it decomposes a complex footprint into rectangles by using an advanced minimal non-overlapping cover ***,a graph-based symmetry detection algorithm is proposed to identify all the symmetrical sub-clusters in ***,a set of selection rules are defined to rank partitions,and the best ones are chosen for roof shape ***,a set of combination rules and a symmetry rule are *** enables to evaluate the probability of a footprint being a certain combination of roof *** results show the growth of the probability of correctly recommending roof shapes for single rectangles and buildings from a prior probability of 17–45%and from a prior probability of 0.29–14.3%,removing 60%and 93%of the incorrect roof shape options,respectively.

关键词： Roof recommendation footprint decomposition symmetry detection symmetry rule combination rules intrinsic roof reconstruction OpenStreetMap

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Photocurrent in the Polyfluorene Copolymer/PTCDI Heterojunction Enhanced by Reabsorption of Fluorescence Emission

引用

Chinese Journal of Polymer Science 2024年第12期42卷 1941-1947,I0009页

作者： Dmitriy A.Lypenko alexey E.Aleksandrov Artem V.Dmitriev Anton A.Yakimanskiy Ilya E.Kolesnikov Tatiana g.Chulkova Alexander V.Yakimansky alexey R.Tameev Frumkin Institute of Physical Chemistry and Electrochemistry of the Russian Academy of Sciences 31/bld.4 Leninskiy ProspectMoscow119071Russia Institute of Macromolecular Compounds of the Russian Academy of Sciences 31 Bolshoi pr.VOSaint Petersburg199004Russia Saint Petersburg State University 7/9 Universitetskaya nab.Saint Petersburg199034Russia

Copolyfluorenes are of great interest due to their ability to form thin films with tunable optical and electrical *** this paper,copolymers of polyfluorene with electron withdrawing dicyanostilbene and dicyanophenanthrene moieties were synthesized;their thin films were characterized by electron spectroscopy,cyclic voltammetry,electrical,and photoelectrical *** mobility of charge carriers in the copolymers was measured for the first time,with the acceptor components providing balanced electron and hole mobilities of the order of 10^(-6) cm^(2)·V^(-1)·s^(-1).Photodetectors based on the copolymer/PTCDI heterojunction exhibited the photoresponse band extended into the green region due to the absorption of PTCDI and an increased photocurrent in the UV-blue absorption band of the copolymer,which is related to the absorption of photoluminescent emission of the copolymers in *** presented approach to improving the performance of a polymer-based photodetector is promising in organic optoelectronics.

关键词： Polyfluorene copolymer Perylenedicarboximide derivative Charge carrier mobility Fluorescence Photoconductivity

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Categories and Weak Equivalences of graded Algebras

引用

Algebra Colloquium 2019年第4期26卷 643-664页

作者： alexey gordienko Ofir Schnabel James Zhang Department of Higher Mathematics Moscow State Technical University of Civil Aviation Kronshtadtsky Boulevardd.20125993 MoscowRussia Department of Mathematics ORT Braude College2161002 KarmiclIsrael 不详

In the study of the structure of graded algebras(such as graded ideals,graded subspaces,and radicals)or graded polynomial identities,the grading group can be replaced by any other group that realizes the same *** we come to the notion of weak equivalence of gradings:two gradings are weakly equivalent if there exists an isomorphism between the graded algebras that maps each graded component onto a graded *** group grading on an algebra can be weakly equivalent to g-gradings for many different groups g;however,it turns out that there is one distinguished group among them,called the universal group of the *** this paper we study categories and functors related to the notion of weak equivalence of *** particular,we introduce an oplax 2-functor that assigns to each grading its support,and show that the universal grading group functor has neither left nor right adjoint.

关键词： algebra grading adjoint functor oplax 2-functor (co)limit

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Effect of Surfactant Stabilizers on the Distribution of Carbon Nanotubes in Aqueous Media

引用

Journal of Wuhan University of Technology(Materials Science) 2018年第3期33卷 533-536页

作者： Yury N Tolchkov Zoya A Mikhaleva R D A Sldozian Nariman R Memetov alexey g Tkachev Department of Technology and Methods of Nanoproducts Manufacturing Tambov State Technical University

The results of a study on the homogeneity of suspensions are described considering the effect of different types of surfactant stabilizers and their concentrations on the uniform distribution of a carbon nanotubes（CNTs）-based modifying additive to construction materials in an aqueous medium. This problem was solved herein by using surfactants and ultrasound. The sonication treatment of CNTs particle agglomerates allowed for dispersing their globules and achieving a 15-20-fold decrease in their average size, for which it became possible to make better use of the CNTs as cement modifier. As a result of the experimental studies carried out, the effect of the surfactant type and concentration promoting uniform distribution of the CNTs in the bulk of the suspension and, correspondingly, in the structural composite matrix was revealed. The CNTs-based additive improved the physical-mechanical and operational characteristics of the material.

关键词： carbon nanotubes nanomodification composite construction materials surfactants

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Spiral attractor created by vector solitons

引用

Light(Science & Applications) 2014年第1期3卷 355-362页

作者： Sergey V Sergeyev Chengbo Mou Elena g Turitsyna alexey Rozhin Sergei K Turitsyn Keith Blow Aston Institute of Photonic Technologies Aston UniversityBirmingham B47ETUK

Mode-locked lasers emitting a train of femtosecond pulses called dissipative solitons are an enabling technology for metrology,high-resolution spectroscopy,fibre optic communications,nano-optics and many other fields of science and ***,the vector nature of dissipative solitons has been exploited to demonstrate mode locked lasing with both locked and rapidly evolving states of ***,for an erbium-doped fibre laser mode locked with carbon nanotubes,we demonstrate the first experimental and theoretical evidence of a new class of slowly evolving vector solitons characterized by a double-scroll chaotic polarisation attractor substantially different from Lorenz,Ro¨ssler and Ikeda strange *** underlying physics comprises a long time scale coherent coupling of two polarisation *** observed phenomena,apart from the fundamental interest,provide a base for advances in secure communications,trapping and manipulation of atoms and nanoparticles,control of magnetisation in data storage devices and many other areas.

关键词： chaos mode-locked laser polarisation phenomena vector soliton

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Zircon U-Pb geochronology Recorded Late Cretaceous Fluid Activation in the Central Aldan gold Ore District, Aldan Shield, Russia: First Data

引用

Journal of Earth Science 2020年第3期31卷 481-491页

作者： Vasilii I.Leontev Sergey g.Skublov Nadezhda V.Shatova alexey V.Berezin Faculty of geological Prospecting Saint Petersburg Mining University199106 St.PetersburgRussia Institute of Precambrian geology and geochronology of Russian Academy of Sciences 199034 St.PetersburgRussia Karpinsky Russian geological Research Institute 199106 St.PetersburgRussia Institute of Earth Science St.Petersburg State University199034 St.PetersburgRussia

The gold mineralization in the Central Aldan ore district is genetically related to potassic calc-alkaline and alkaline magmatism dated at 115–150 *** objective of this study is to establish the age of hydrothermal processes that accompanied the formation of Au-Te mineralization at the Samolazovsky *** on the isotope-geochemical study of zircons from quartz-feldspar metasomatic rocks of the deposit,the granitoids and charnokites of the Nimnyr Complex(1900–1960 Ma)at the contact with the Yukhta monzonite-syenite massif(~127 Ma)were *** U-Pb dating was performed on a SHRIMP-II ion microprobe,and rare-earth and trace elements analyses of zircon were carried out in the same craters by secondary-ion mass spectrometry on a Cameca IMS-4f ion *** is revealed that the studied zircons have heterogeneous structures:dark core and lighter rim,which differ greatly in isotope-geochemical *** rims are cut by a network of fractures,extending into the central part of zircon *** rims yield a subconcordant age of 1937±24 Ma,with an average total REE content of 550 ppm,which corresponds to the formation age of the Nimnyr *** zircon cores yield a discordant age of 83±11 Ma and are characterized by a higher total REE content(~1812 ppm),as well as higher contents of U and non-formula elements(Ca,Sr,and Y)with respect to rims,due to the effect of fluid on *** the limited number of zircon grains,the additional geochronological study of zircons from syenites of the ore-bearing Ryabinovy Massif has revealed the presence of two distinct age clusters:~125–138 and 76–83Ма.The older ages of zircons from syenites are typical for the Central Aldan ore *** now,there is still no explanation for an age range(76–83 Ma)of single zircon grains from ore-bearing syenites of the Ryabinovy *** obtained data suggest that the processes of activation(the effect of fluid)within the Central Aldan ore district continued unt

关键词： U-Pb geochronology REE geochemistry zircon Samolazovskoe Deposit Ryabinovoe Deposit Aldan Shield

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

欢迎您,

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案：

请选择收藏分类：

通借通还

欢迎您,

建议与咨询 留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案： 新增检索档案 确定 取消

请选择收藏分类： 新增自定义分类 确定 取消

通借通还

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

请选择保存的检索档案：

请选择收藏分类：