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Functionality of a bicistronic construction containing HEXA and HEXB genes encoding β-hexosaminidase A for cell-mediated therapy of Gm2 gangliosidoses

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Neural Regeneration Research 2022年第1期17卷 122-129页

作者： Alisa A.Shaimardanova Daria S.Chulpanova Valeriya V.Solovyeva aleksandr m.aimaletdinov Albert A.Rizvanov Institute of Fundamental medicine and Biology Kazan Federal UniversityKazanRussia

Tay-Sachs disease and Sandhoff disease are severe hereditary neurodegenerative disorders caused by a deficiency ofβ-hexosaminidase A(HexA)enzyme,which results in the accumulation of Gm2 gangliosides in the nervous system *** this work,we analyzed the efficacy and safety of cell-mediated gene therapy for Sandhoff disease and Sandhoff disease using a bicistronic lentiviral vector encoding cDNA of HexAα-andβ-subunit genes separated by the nucleotide sequence of a P2A peptide(HEXA-HEXB).The functionality of the bicistronic construct containing the HEXA-HEXB genetic cassette was analyzed in a culture of HEK293T cells and human umbilical cord blood mononuclear cells(hUCBmCs).Our results showed that the enzymatic activity of HexA in the conditioned medium harvested from genetically modified HEK293T-HEXA-HEXB and hUCBmCs-HEXA-HEXB was increased by 23 and 8 times,respectively,compared with the conditioned medium of native *** blot analysis showed that hUCBmCs-HEXA-HEXB secreted both completely separated HEXA and HEXB proteins,and an uncleaved protein containing HEXA+HEXB linked by the P2A *** injection of genetically modified hUCBmCs-HEXA-HEXB to laboratory Wistar rats was carried out,and the HexA enzymatic activity in the blood plasma of experimental animals,as well as the number of live cells of immune system organs(spleen,thymus,bone marrow,lymph nodes)were determined.A significant increase in the enzymatic activity of HexA in the blood plasma of laboratory rats on days 6 and 9(by 2.5 and 3 times,respectively)after the administration of hUCBmCsHEXA-HEXB was *** the same time,the number of live cells in the studied organs remained ***,the functionality of the bicistronic genetic construct encoding cDNA of the HEXA and HEXB genes separated by the nucleotide sequence of the P2A peptide was shown in vitro and in *** hypothesize that due to the natural ability of hUCBmCs to overcome biological barriers,such a strategy can rest

关键词： bicistronic vector cell-mediated gene therapy Gm2 gangliosidosis P2A peptide Sandhoff disease Tay-Sachs disease umbilical cord blood mononuclear cells β-hexosaminidase

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Increasingβ-hexosaminidase A activity using genetically modified mesenchymal stem cells

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Neural Regeneration Research 2024年第1期19卷 212-219页

作者： Alisa A.Shaimardanova Daria S.Chulpanova Valeriya V.Solovуeva Shaza S.Issa Aysilu I.mullagulova Angelina A.Titova Yana O.mukhamedshina Anna V.Timofeeva Alexander m.aimaletdinov Islam R.Nigmetzyanov Albert A.Rizvanov Institute of Fundamental medicine and Biology Kazan Federal UniversityKazanRussia Department of Histology Cytology and EmbryologyKazan State Medical UniversityKazanRussia

Gm2 gangliosidoses are a group of autosomal-recessive lysosomal storage disorde *** diseases result from a deficiency of lysosomal enzymeβ-hexosaminidase A(HexA),which is responsible for Gm2 ganglioside *** deficiency causes the accumulation of Gm2-gangliosides mainly in the nervous system cells,leading to severe progressive neurodegeneration and *** date,there is no treatment for these ***-mediated gene therapy is considered a promising treatment for Gm2 *** study aimed to evaluate the ability of genetically modified mesenchymal stem cells(mSCs-HEXA-HEXB)to restore HexA deficiency in Tay-Sachs disease patient cells,as well as to analyze the functionality and biodistribution of mSCs in *** effectiveness of HexA deficiency cross-correction was shown in mutant mSCs upon intera ction with *** results also showed that the mSCs-HEXA-HEXB express the functionally active HexA enzyme,detectable in vivo,and intravenous injection of the cells does not cause an immune response in *** data suggest that genetically modified mesenchymal stem cells have the potentials to treat Gm2 gangliosidoses.

关键词： adeno-associated viral vectors cell therapy cell-mediated gene therapy gene therapy Gm2 gangliosidosis Sandhoff disease Tay-Sachs disease β-hexosaminidase

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Crystal structure and thermal expansion of perovskites containing uranium(VI) and rare-earth elements

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Journal of Rare Earths 2009年第1期27卷 4-11页

作者： aleksandr Knyazev Anna Ershova Nikolai Chernorukov Nizhny Novgorod State University

By the method of high-temperature reactions in solid phase, compounds with the general formula mⅡ（AⅢ2/3U1/3）O3（mⅡ=Sr, AⅢ=Sc, In;mⅡ=Ba, AⅢ=Sc, In, Y, Nd-Lu） were *** structures（space groups Fm 3 m and Pnma） were refined by the Rietveld method and morphotropic transition in Ba（Ln2/3U1/3）O3 on the border of Gd-Tb was *** means of high-temperature X-ray diffraction, phase transitions were studied and thermal expansion coefficients were determined.

关键词： perovskite uranium X-ray diffraction Rietveld structure thermal expansion rare earths

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Variable retention forestry in European boreal forests in Russia

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Ecological Processes 2019年第1期8卷 424-434页

作者： Ekaterina Shorohova Sergey Sinkevich aleksandr Kryshen Ilkka Vanha-majamaa Forest Research Institute of the Karelian Research Centre of the Russian Academy of Sciences Pushkinskaya str.11Petrozavodsk 185910Russia Saint-Petersburg State Forest Technical University Institutsky str.5Saint Petersburg 194021Russia Natural Resources Institute Finland(Luke)Latokartanonkaari 9FI-00790 HelsinkiFinland

We explored whether,and to what extent,variable retention(VR)forestry has been applied in European boreal forests in northwestern *** survey revealed VR since *** 1910 and the 1960s,the statistics showing how much was retained are largely ***,for example,in the 1950s,on a large scale in the Republic of Karelia,up to 200-ha-sized harvesting areas,18–33%,were retention patches with a mean growing stock of 30–40m3 ha−*** the fellings defined as“incomplete clear fellings,”which were the most common final felling type at that time,11–40%of the growing stock was *** the 1960s and the early 1990s,with more efficient harvesting and skidding techniques,conventional clear fellings with a much lower amount of retention were *** about the regeneration of harvested areas gradually led to smaller(maximum 50 ha)harvesting areas and the increase of silvicultural *** now,to ensure natural regeneration,patches of understory and 20–25 seed trees(i.e.,ca.15–25m3)per ha have been left permanently in harvesting ***-scale retention for protecting ecosystem functions and biodiversity was legislated in 1978 by preserving key biotopes up to 1000 ha in *** 2001,promoted by forest certification,the key biotopes,such as paludified forest patches,buffers around water bodies,and habitats of red-listed species,have also been retained in harvesting areas,together with a dispersed retention of different *** estimates of the amount of key biotopes are largely ***,estimates of 1–13%in harvesting areas and 23%in whole managed landscapes have been *** applied during the last century has emulated natural disturbances and created diverse uneven-aged forest structures with high amounts of diverse coarse woody *** conclude that an analysis of past and current retention practices is essential for estimating the global role of Russian *** decisions on the general direction

关键词： Biological legacies Nature conservation Biodiversity Clear felling Key biotopes Coarse woody debris,Silviculture Dispersed and aggregated retention

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Strain-Rate Dependency of a Unidirectional Filament Wound Composite under Compression

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Computer modeling in Engineering & Sciences 2023年第12期137卷 2149-2161页

作者： Stepan Konev Victor A.Eremeyev Hamid m.Sedighi Leonid Igumnov Anatoly Bragov aleksandr Konstantinov Ayaulym Kuanyshova Ivan Sergeichev Skolkovo Institute of Science and Technologies Center for Materials TechnologiesMoscowRussia National Research Lobachevsky State University of Nizhny Novgorod Nizhny NovgorodRussia Dipartimento di Ingegneria Civile Ambientale e Architettura(DICAAR)Universitàdegli Studi di CagliariCagliari(CA)Italy mechanical Engineering Department Faculty of EngineeringShahid Chamran University of AhvazAhvazIran Drilling Center of Excellence and Research Center Shahid Chamran University of AhvazAhvazIran

This article presents the results of experimental studies concerning the dynamic deformation and failure of a unidirectional carbon fiber reinforced plastic(T700/LY113)under *** test samples were manufactured through the filament winding of flat *** establish the strain rate dependencies of the strength and elastic modulus of the material,dynamic tests were carried out using a drop tower,the Split Hopkinson Pressure Bar method,and standard static *** samples were loaded both along and perpendicular to the direction of the reinforcing *** applicability of the obtained samples for static and dynamic tests was confirmed through finite elementmodeling and the high-speed imaging of the deformation and failure of samples during *** a result of the conducted experimental studies,static and dynamic stress-strain curves,time dependencies of deformation and the stress and strain rates of the samples during compression were *** on these results,the strain rate dependencies of the strength and elasticity modulus in the strain rate range of 0.001-6001/s are *** is shown that the strain rate significantly affects the strength and deformation characteristics of the unidirectional carbon fiber composites under *** increase in the strain rate by 5 orders of magnitude increased the strength and elastic modulus along the fiber direction by 42%and 50%,*** loading resulted in a strength and elastic modulus increase by 58%and 50%,*** average strength along the fibers at the largest studied strain rate was about *** obtained results can be used to design structural elements made of polymer composite materials operating under dynamic shock loads,as well as to build models of mechanical behavior and failure criteria of such materials,taking into account the strain rate effects.

关键词： High strain rate composites filament winding dynamic strength Split Hopkinson Pressure Bar compression

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Porphyrins,phthalocyanines,and related covalent-organic frameworks in the photochemical and electrochemical water splitting:A review

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Particuology 2024年第7期90卷 236-265页

作者： Oxana V.Kharissova Yolanda Peña méndez Boris I.Kharisov aleksandr LNikolaev Edith Luévano-Hipólito Lucy T.González Universidad Autónoma de Nuevo León Ave.Universidad s/nSan Nicolás de los GarzaNuevo León66455Mexico Department of Chemistry Moscow State UniversityVorobievy HillsMoscowRussia CONACYT-Universidad Autónoma de Nuevo León Facultad de Ingeniería Civil-Departamento de Ecomateriales y EnergíaCd.UniversitariaC.P.66455San Nicolás de los GarzaNLMexico Tecnologico de monterrey Escuela de Ingeniería y CienciasMonterreyNuevo León 64890Mexico Tecnologico de monterrey Centro del AguaMonterreyNuevo León 64890Mexico

In this review,the possibilities of photochemical and electrochemical water splitting(WS)using porphyrins,phthalocyanines,related macrocycles,and their metal complexes are *** efforts are being carried out in order to develop novel and cheap methods for water decomposition to generate inexhaustible greener energy sources(molecular hydrogen)instead of oil and *** processes of WS in heterogeneous and homogeneous media require novel photo-and electrocatalysts,in particular those on macrocycle basis.A high WS efficiency has been observed for porphyrins and phthalocyanines,showing their suitability both for HER and OER processes are available using these relative compounds for WS *** can be used in photocatalytic and electrocatalytic WS processes both in their free forms or as metal porphyrins/porphyrinates,mainly loaded on various inorganic materials,such as Au NPs,C_(3)N_(4),GaP,KTaO_(3),GaP,and so on,as part of porphyrin-containing polymers or on electrode surface as modifying coatings,being sometimes better than commercial catalysts,such as Pt/*** H2 evolution can be considerably depended on the shape of porphyrin particles;in particular,porphyrin nanowires can yield up to 20 times more H2 than its *** certain cases,both HER and OER can be driven in the same electrolyte using metal *** capacity of metal porphyrins to generate ROS(in particular,singlet oxygen 1O_(2))under ultrasonic treatment is known and can be successfully used for H_(2)O_(2) and O_(2) *** can be used in similar processes in free form or as composites with inorganic or organic counterparts,being immobilized on nanocarbons,metal oxides and salts,or simply dissolved in water acting in a homogeneous *** additional use of ultrasound can lead to better results due to the synergistic effect of the sonochemical treatment and ***-organic frameworks are also applied for WS purposes and are mainly based

关键词： Porphyrins Phthalocyanines COFs Water splitting Photocatalysis Electrocatalysis

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Negative thermal expansion in one-dimension of a new double sulfate AgHo(SO_(4))_(2)with isolated SO_4 tetrahedra

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Journal of materials Science & Technology 2021年第17期76卷 111-121页

作者： Yuriy G.Denisenko Victor V.Atuchin maxim S.molokeev Naizheng Wang Xingxing Jiang aleksandr S.aleksandrovsky Alexander S.Krylov aleksandr S.Oreshonkov Alexander E.Sedykh Svetlana S.Volkova Zheshuai Lin Oleg V.Andreev Klaus müller-Buschbaum Institute of Chemistry Tyumen State University.Tyumen625003Russia Institute of Inorganic and Analytical Chemistry Justus-Liebig-University of Giessen35392Giessen.Germany Department of General and Special Chemistry Industrial University of TyumenTyumen625000Russia Laboratory of Optical materials and Structures Institute of Semiconductor PhysicsSB RASNovosibirsk630090Russia Laboratory of Semiconductor and Dielectric materials Novosibirsk State UniversityNovosibirsk630090Russia Research and Development Department.Kemerovo State University Kemerovo650000Russia Laboratory of Crystal Physics Kirensky Institute of PhysicsFederal Research Center KSC SB RASKrasnoyarsk660036Russia Siberian Federal University Krasnoyarsk660041Russia Department of Physics Far Eastern State Transport UniversityKhabarovsk680021Russia Technical Institute of Physics and Chemistry Chinese Academy of SciencesBeijing100190China Laboratory of Coherent Optics Kirensky Institute of Physics.Federal Research Center KSC SB RASKrasnoyarsk660036Russia Institute of Nanotechnology Spectroscopy and Quantum ChemistrySiberian Federal UniversityKrasnoyarsk660041Russia Laboratory of molecular Spectroscopy Kirensky Institute of PhysicsFederal Research Center KSC SB RASKrasnoyarsk660036Russia Center for materials Research(Lama) Justus-Liebig-University GiessenGiessen35392Germany University of the Chinese Academy of Sciences Beijing100049China Laboratory of the Chemistry of Rare Earth Compounds Institute of Solid State ChemistryUB RASEkaterinburg620137Russia

A double holmium-silver sulfate was obtained for the first *** temperature intervals for the formation and stability of the compound were determined by differential scanning *** crystal structure of AgHo(SO_(4))_(2)was determined by Rietveld *** X-ray diffraction(XRD)analysis showed that the compound crystallizes in the monoclinic syngony,space group P2_1/m,with the unit cell parameters a=4.71751(4)A,b=6.84940(6)A and c=9.89528(9)A,β=95.1466(4)·,V=318.448(5)A^(3),Z=2,R_B=1.55%,T=303 *** types of sulfate tetrahedra were found in the structure,which significantly affected the spectral properties in the infrared *** the temperature range of 143-703 K,a negative thermal expansion along the b direction accompanied by a positive thermal expansion along the a and c directions was *** was established that negative thermal expansion is the result of the deformation of sulfate tetrahedra,which is affected by the movement of holmium and silver *** excitation in the blue spectral range(457.9 nm)produces a luminescence in light blue(489 nm),green(545 nm)and red(654 nm)spectral ranges,and the latter two were of comparable intensity that is favorable for WLED *** observed luminescent band distribution is ascribed to the specific crystal field at Ho^(3+)ion sites rather than a variation of radiationless probability.

关键词： Sulfate Crystal structure Thermal expansion Raman Photoluminescence Band structure

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Artemia cyst production in Russia

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Chinese Journal of Oceanology and Limnology 2015年第6期33卷 1436-1450页

作者： Liudmila I.LITVINENKO aleksandr I.LITVINENKO Elena G.BOIKO Kirill KUTSANOV Federal State Unitary Enterprise GosrybcenterTyumenRussia State Agrarian University of Northern Urals

In Western Siberia(Russia) there are about 100 Artemia lakes with total area over 1 600 km *** these lakes are located between 51°–56°N and 61°–82°*** general these lakes are shallow(depth less than 1.5 m),small or medium size(0.1 to 10 km 2);they are chloride;their total salinity is from 40 to 250 g/*** harvesting of cysts per year is only in 20–40 *** Russia 550 tons of dry Artemia cysts(14%–18% of the world production) were harvested *** includes about 350 tons in the Altai region and 200 tons in other *** our regular 20-year study period the cyst harvest was:95 tons in Kurgan;65 tons in Omsk,20 tons in Novosibirsk,20 tons in *** of increasing cyst harvest in Russia are considered in this *** the last 30 years the harvest of cysts in Russia has increased from 7–20 to 500–600 tons.A significant influence of dryness of the year was found on productivity in selected lakes,but taken for all the lakes together,the relationship was not *** optimal salinity for productivity of cysts in the lakes was *** of productivity of the lakes and the harvesting results showed that the stocks of cysts are underutilized by approximately 1.7 times.

关键词： Artemia brine shrimp cyst production Russian saline lakes population dynamics aquacultureindustry

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T Wave Amplitude Deviation as a Participation marker of Glycolysis in myocardial Repolarization

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World Journal of Cardiovascular Diseases 2019年第5期9卷 343-353页

作者： mikhail U. Alekseev aleksandr m. Alekseev Juventa Diagnostic Center Saint Petersburg Russia Tozka Diagnostics LLC Moscow Russia

Glucose metabolism is essential for heart physiology, especially in ischemic conditions. Anaerobic glycolysis participates along with aerobic fatty acid oxidation in generating energy supply, and the balance is shifted towards fatty acids. In the case of aerobic path restriction due to coronary artery disease (CAD), this ratio changes. The aim of the present research was to discover the aspects of glycolysis in cardiac electrophysiology.?We utilized the normalized (by average value) T wave amplitude deviation values, named the G criterion,?from high-resolution, 12-channel, 120-seconds-long electrocardiograms,?which were obtained using DACG technology. The calculated G criterion allows a quantitative assessment of the ischemic process and corresponds to the functional class (FC) according to the Canadian cardiovascular classification. We examined 22 healthy people and 79 proven CAD patients: 24 II FC, 40 III FC and 15 IV FC by original ECG and fasting blood sugar tests. A strong correlation was found between the average G criterion from all 12 channels and the level of fasting blood sugar (r = +0.88). In the control group, blood sugar levels were 91 ± 6.5 mg/dl, II FC 103 ± 14.8 mg/dl (p mg/dl (p moreover, the correlation factor (average G criterion and blood sugar) was strong and positive in each group: control +0.72, II FC +0.83, III FC +0.76, and IV FC +0.89. For the first time, heart electrical activity from the ECG curve was associated with high glucose level as one of the main CAD risk factors. The presence of such dependence of increased blood sugar level from the severity of the ischemic process in the myocardium indicates that hyperglycemic reaction is adaptive and can accelerate the anaerobic glycolysis for energy demand compensation in heart muscle.

关键词： Electrocardiogram T Wave Deviation Blood Sugar Hyperglycemia Glycolysis

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Permafrost distribution and temperature in the Elkon Horst, Russia

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Research in Cold and Arid Regions 2021年第2期13卷 107-122页

作者： mikhail Zhelezniak QingBai Wu Anatolii Kirillin Zhi Wen aleksandr Zhirkov Vladimir Zhizhin melnikov Permafrost Institute SB RAS Yakutsk 677000Russia State Key Laboratory of Frozen Soil Engineering Northwest Institute of Eco-Environment and ResourcesCASLanzhouGansu 730000China

The Elkon Horst is a geological structure that consists of heterogeneous strata with highly variable geocryological and temperature conditions. Gaining accurate knowledge of permafrost distribution patterns within this structure is of both scientific and practical importance. In mountainous terrain, the ground thermal regime is controlled by both surface and subsurface conditions. Surface conditions include snow cover characteristics, the presence or absence of vegetation, vegetation density, etc.. In contrast, subsurface conditions involve rock lithology or petrography, density, quantity and depth of fissures, groundwater, etc.. This article examines ground thermal regimes in various geomorphological settings based on temperature measurement data from geotechnical boreholes. The occurrence and extent of permafrost were evaluated for the entire horst area using direct and indirect methods. The maximum permafrost thickness measured in the Elkon Horst is 330 m, and the estimated maximum is 450 m at higher elevations. Thermophysical properties were determined for the major rock types, and the geothermal heat flux was estimated for the study area. The thermal conductivities were found to vary from 1.47 to 4.20 W/(m·K), and the dry bulk densities to range between 2,236 kg/m~3 and 3,235 kg/m~3. The average geothermal heat flux was estimated to be 44 mW/m~2.

关键词： permafrost ground temperature geothermal heat flux thermal conductivity horst

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