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

The Paleogeographic Reconstruction for the Central Regions of the Western Siberian Basin in the Middle Jurassic Epoch

引用

地学前缘 2009年第S1期16卷 193-193页

作者： Aleksey Yu.Popov lyudmila G.Vakulenko Valeriy A.Kazanenkov Ilicha-21 Str. Novosibirsk 630090Russia

The complex investigation of the Bathonian sedimentary deposits in central regions of the WestSiberian basin was *** the basis both of lithofacial analysis of core clefts and the geophysical investigation data and taking into account the tectonic features of the Jurassic structural storey the sedimenttological model of deposits was built and the main regularities of its building were *** the initial stage the framework of alluvial lower plain

关键词： paleogeography lithology sediment-tary environments Middle Jurassic Western Siberia

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

Bulk Composite Nanomaterial with Multiwall Carbon Nanotubes

引用

Materials Sciences and Applications 2012年第10期3卷 728-732页

作者： Levan Ichkitidze Vitally Podgaetsky Alexander Prihodko Sergei Selishchev Eugenie Blagov Vyacheslav Galperin Yuri Shaman lyudmila Tabulina Belarusian State University of Informatics and Radioelectronics Minsk Belarus Institute of nanotechnology of microelectronics RAS Moscow Russia National Research University of Electronic Technology (MIET) Moscow Russia Scientific Manufacturing Complex “Technological Centre” Moscow Russia

The properties of the composite nanomaterials (CNM) based on bovine serum albumin (BSA) and multi-walled carbon nanotubes (MWCNT), both functionalized and non-functionalized, were investigated. In order to obtain the solid-state bulk CNM from the ultradispersed aqueous solutions of 25 wt.% BSA and (0.0015 - 0.04) wt.% MWCNT, the methods of nanotechnology and laser technology were used. It is revealed that the CNM density is 10% - 20% higher than that of water and the hardness is higher than that of BSA by a factor of 3 - 6 times. An increase in hardness Hv (by Vickers) of CNM correlated with an increase in the concentration of MWCNT, and Hyreached ~300 MPa for the case of the non-functionalized MWCNT, while for the case of the functionalized MWCNT, i.e. MWCNTf, Hy was 25% lower.

关键词： Carbon Nanotubes Functionalization Albumine Biocompatible Composite Nanomaterial

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

Ombitasvir, paritaprevir, ritonavir, dasabuvir and ribavirin in cirrhosis after complete destruction of hepatocellular carcinoma

引用

World Journal of Gastroenterology 2016年第8期22卷 2630-2635页

作者： Zahariy Krastev Deian Jelev Krasimir Antonov Tanya Petkova Evelina Atanasova Nadezhda Zheleva Bojidar Tomov Yana Boyanova lyudmila Mateva Clinic of Gastroenterology University Hospital "St. Ivan Rilski"

We observed a sustained viral response(SVR) of ombitasvir/paritaprevir/ritonavir, dasabuvir and ribavirin therapy, for 12 wk, in two cases with compensated liver cirrhosis and fully destroyed early hepatocellular carcinoma(HCC). Patients were infected with hepatitis C virus(HCV) genotype 1b and were previous null responders/relapsers to interferon-alpha/ribavirin(IFN/RBV). There was a rapid suppression of HCV RNA to undetectable levels within the first two treatment weeks. SVR was achieved even after marked reduction of the RBV dose. The treatment was well tolerated. Both subjects experienced worsening of liver disease during therapy, in different patterns: severe, transient, predominantly direct hyperbilirubinemia without cytolysis(case 1) or progressive increase of aminotransferases(grade 4) without severe hyperbilirubinemia(case 2).Adverse events spontaneously resolved. The patients remained in a good clinical condition without hepatic decompensation. There was no re-occurrence of HCC. This is the first report for treatment of HCV cirrhosis after complete HCC destruction.

关键词： Ombitasvir Paritaprevir Ritonavir Dasabuvir Ribavirin Hepatitis C virus cirrhosis Hepatocellular car

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

Cultural Orientation Through Public Speaking Contests

引用

Sino-US English Teaching 2013年第10期10卷 806-811页

作者： lyudmila Chikileva Financial University Under the Government of the Russian Federation Moscow Russia

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

In search of effective therapies to overcome resistance to Temozolomide in brain tumours

引用

Cancer Drug Resistance 2019年第4期2卷 1018-1031页

作者： Kaouthar Bouzinab Helen Summers Jihong Zhang Malcolm F.G.Stevens Christopher J.Moody lyudmila Turyanska Neil R.Thomas Pavel Gershkovich Marianne B.Ashford Emily Vitterso Lisa C.D.Storer Richard Grundy Tracey D.Bradshaw School of Pharmacy University of NottinghamNottingham NG72RDUK School of Chemistry University of NottinghamNottingham NG72RDUK School of Physics and Astronomy University of NottinghamNottingham NG72RDUK Advanced Drug Delivery Pharmaceutical SciencesR&DAstraZenecaMacclesfield SK104TFUK School of Medicine Queen’s Medical CentreUniversity of NottinghamNottingham NG72UHUK.

Glioblastoma multiforme is the most common and lethal brain *** current standard of care includes Temozolomide(TMZ)***,inherent and acquired resistance to TMZ thwart successful *** direct repair protein methylguanine DNA methyltransferase(MGMT)removes the cytotoxic O6-methylguanine(O6-MeG)lesion delivered by TMZ and so its expression by tumours confers *** mismatch repair(MMR)is essential to process O6-MeG adducts and MMR-deficiency leads to tolerance of lesions,resistance to TMZ and further DNA *** this article,two strategies to overcome TMZ resistance are discussed:(1)synthesis of imidazotetrazine analogues-designed to retain activity in the presence of MGMT or loss of MMR;(2)preparation of imidazotetrazine-nanoparticles to deliver TMZ preferably to the brain and tumour *** promising results encourage belief in a future where better prognoses exist for patients diagnosed with this devastating disease.

关键词： Brain cancer temozolomide-resistance analogues drug delivery apoferritin

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

喀拉海有机质来源的稳定碳氮同位素指标

引用

海洋地质 2013年第3期29卷 70-81页

作者： Birgit Nagel Birgit Gaye lyudmila A. Kodina 常晓红(译) 不详中国科学院广州地球化学研究所

碳、氮稳定同位素比值（δ^12C、δ^15N）通常被用来判识有机质来源和演化过程。在此，我们讨论了鄂毕河、叶尼塞河口和喀拉海（北冰洋）的悬浮物和沉积物中的稳定同位素数据，以便考察它们是如何受河流径流的季节和年际变化、冰覆盖率... 详细信息

碳、氮稳定同位素比值（δ^12C、δ^15N）通常被用来判识有机质来源和演化过程。在此，我们讨论了鄂毕河、叶尼塞河口和喀拉海（北冰洋）的悬浮物和沉积物中的稳定同位素数据，以便考察它们是如何受河流径流的季节和年际变化、冰覆盖率以及生物活动的影响。研究表明碳、氮稳定同位素比值向滨外方向逐渐增大，δ^13C与海水盐度正相关。各年度悬浮物数据之间的差异揭示δ^13C值的各种调整过程，如偶然性的浮游生物大量繁殖、河流端元和年径流量的变更。尽管表层海水中悬浮物的δ^13C与海水盐度相关性的变化类似于无冰期表层沉积物的δ^13C与平均盐度相关性的变化。然而，沉积物的613C值则偏高2．2％o。由于深水中下沉颗粒的613C值与沉积物的813C值大致相同，因此利用沉积物的613C值与盐度的相关性可以重建距今10000a期间的叶尼塞河口的古盐度。

关键词： δ^13C δ^15N喀拉海古气候鄂毕河叶尼塞河

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

Mathematical Modeling of the Influence of the Wind Field Structure in the Atmosphere on the Cloud Formation Processes

引用

Atmospheric and Climate Sciences 2018年第1期8卷 84-96页

作者： Boris A. Ashabokov lyudmila M. Fedchenko Alexander V. Shapovalov Khazhbara M. Kalov Ruslan Kh. Kalov Alla A. Tashilova Vitaly A. Shapovalov High-Mountain Geophysical Institute Nalchik Russia Institute of Computer Science and Problems of Regional Management Kabardino-Balkarian Research Center Russian Academy of Sciences Nalchik Russia

The state of the physics of convective clouds and cloud seeding is discussed briefly. It is noted that at the present time there is a transition from the stage of investigation of “elementary” processes in the clouds to the stage of studying the formation of macro- and microstructural characteristics of clouds as a whole, taking into account their system properties. The main directions of the development of cloud physics at the upcoming stage of its development are discussed. The paper points out that one of these areas is the determination of the structure-forming factors for the clouds and the study of their influence on their formation and evolution. It is noted that one of such factors is the interaction of clouds with their surrounding atmosphere, and the main method of studying its role in the processes of cloud formation is mathematical modeling. A three-dimensional nonstationary model of convective clouds is presented with a detailed account of the processes of thermohydrodynamics and microphysics, which is used for research. The results of modeling the influence of the wind field structure in the atmosphere on the formation and evolution of clouds are presented. It is shown that the dynamic characteristics of the atmosphere have a significant effect on the formation of macro- and microstructural characteristics of convective clouds: the more complex the structure of the wind field in the atmosphere (i.e., the more intense the interaction of the atmosphere and the cloud), the less powerful the clouds are formed.

关键词： Wind in the Atmosphere Wind Field Structure Interaction with Clouds Mod-eling System Properties of Clouds Interaction of Clouds with the Atmosphere Three-Dimensional Cloud Model

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

The Study of Inhibition Effect of Rhenium Thioacetic and Dithiocarbonic Acids in Oxidation Reaction

引用

Advances in Chemical Engineering and Science 2015年第3期5卷 338-344页

作者： Fakhriyya M. Nasiri Fikret A. Kuliev Arif J. Efendy lyudmila I. Kojarova Farida A. Abdullayeva Irada H. Melikova Natevan F. Aykan Institute of Catalysis and Inorganic Chemistry Named after Academician M.F.Nagiyev of Azerbaijan Baku Azerbaijan Azerbaijan Medical University Baku Azerbaijan

Antioxidant activity of interaction products of the studied compounds with tret-Butyl hydroperoxide is studied. New, more active acceptors of free radicals are formed in the process of oxidative transformations of initial compounds. It is investigated that the products have high inhibiting properties. The parameter fk, characterizing inhibitory effect of an inhibitor is studied.

关键词： Synthesis Phenylthioacetic and Dithiocarbonic Acids Hydroperoxide Inhibitor

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

夜深时分

引用

作者： (俄)柳德米拉·彼得鲁舍夫斯卡娅(lyudmila Petrushevskaya)

来源：南通市图书馆图书评论

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

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

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：