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6. Macro- and microscopic properties of strontium doped indium oxide

Author

Nikolaenko, Y. M., Kuzovlev, Y. E., Medvedev, Y. V., Mezin, N. I., Fasel, C., Gurlo, A., Schlicker, L., Bayer, T. J. M., and Genenko, Y. A.

Subjects
Condensed Matter - Materials Science
Abstract

Solid state synthesis and physical mechanisms of electrical conductivity variation in polycrystalline, strontium doped indium oxide In2O3:(SrO)x were investigated for materials with different doping levels at different temperatures (T=20-300 C) and ambient atmosphere content including humidity and low pressure. Gas sensing ability of these compounds as well as the sample resistance appeared to increase by 4 and 8 orders of the magnitude, respectively, with the doping level increase from zero up to x=10%. The conductance variation due to doping is explained by two mechanisms: acceptor-like electrical activity of Sr as a point defect and appearance of an additional phase of SrIn2O4. An unusual property of high level (x=10%) doped samples is a possibility of extraordinarily large and fast oxygen exchange with ambient atmosphere at not very high temperatures (100-200 C). This peculiarity is explained by friable structure of crystallite surface. Friable structure provides relatively fast transition of samples from high to low resistive state at the expense of high conductance of the near surface layer of the grains. Microscopic study of the electro-diffusion process at the surface of oxygen deficient samples allowed estimation of the diffusion coefficient of oxygen vacancies in the friable surface layer at room temperature as 3x10^(-13) cm^2/s, which is by one order of the magnitude smaller than that known for amorphous indium oxide films., Comment: 19 pages, 7 figures, 39 references

Published
2014
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7. Magnetostatic Spin Waves and Magnetic-Wave Chaos in Ferromagnetic Films. III. Numeric Simulations of Microwave-Band Magnetic Chaos, Its Synchronization and Application to Secure Communication

Author

Glushchenko, A. A., Kuzovlev, Yu. E., Medvedev, Yu. V., and Mezin, N. I.

Subjects
Condensed Matter - Other Condensed Matter, Nonlinear Sciences - Chaotic Dynamics
Abstract

Selected results of original numeric simulations of non-linear magnetostatic spin waves and microwave-frequency magnetic chaos in ferrite films are expounded, as third part of the work whose first two parts are recent arXive preprints 1204.0200 and 1204.2423 . Especially we consider crucial role of parametric processes in creating the chaos and simultaneously obstacles to its synchronization, and examine some possibilities of good enough synchronization (to an extent allowing its use for direct secure communication in microwave band)., Comment: 37 pages, 21 figures, latex2e revtex4

Published
2012

8. Magnetostatic spin waves and magnetic-wave chaos in ferromagnetic films. II. Numerical simulations of non-linear waves

Author

Kuzovlev, Yu. E., Medvedev, Yu. V., and Mezin, N. I.

Subjects
Condensed Matter - Other Condensed Matter, Nonlinear Sciences - Chaotic Dynamics
Abstract

A method and some results of numeric simulations of magnetostatic spin waves in ferromagnetic films are exponded, in comparison with the theory earlier presented in arXiv preprint 1204.0200. In particular, roles of films finiteness (edges) and defects in formation of linear and non-linear magnetostatic wave patterns, excitation and evolution of two-dimensional solitons, and chaotic non-linear ferromagnetic resonance are considered., Comment: 24 pages, 11 figures, latex2e revtex4

Published
2012

9. Magnetostatic spin waves and magnetic-wave chaos in ferromagnetic films. I. Theory of magnetostatic waves in plates under arbitrary anisotropy and external fields

Author

Kuzovlev, Yu. E., Medvedev, Yu. V., and Mezin, N. I.

Subjects
Condensed Matter - Other Condensed Matter
Abstract

General phenomenological theory of magnetic spin waves in ferromagnetic media is originally reformulated and applied to analysis of magnetostatic waves in films and plates with arbitrary nisotropy under arbitrary external field. Exact expressions are derived for propagator of linear waves between antennae (inductors) and mutual impedances of antennae, and exact unified dispersion equation is obtained which describes all types of magnetostatic wave eigen-modes. Characteristic frequencies (spectra) and some other important properties of main modes are analytically considered and graphically illustrated. Besides, several aspects of non-linear excitations and magnetic-wave chaos are discussed, including two-dimensional "non-linear Schrodinger equations" for magnetostatic wave packets and envelope solitons., Comment: 67 pages, 16 figures, latex2e amsart

Published
2012

11. Excitation of magnetostatic spin waves in ferromagnetic films

Author

Kuzovlev, Yu., Mezin, N., and Yarosh, G.

Subjects
Condensed Matter - Other Condensed Matter, Condensed Matter - Materials Science
Abstract

The torque equation of nonlinear spin dynamics is considered in the magnetostatic approximation. In this framework, exact expressions for propagator of linear magnetostatic waves in ferromagnetic film between two antennas and corresponding mutual impedance of the antennas are derived, under conditions of uniform but arbitrarily oriented static magnetization and arbitrary anisotropy. The results imply also full description of spectrum of the waves., Comment: 6 pages, no figures, to be submitted to a physical journal

Published
2004

12. La passion selon saint Matthieu : Matthieu 26-28

Author

Venard, Olivier-Thomas, Sous la direction de, Álvares, Maria Cristina, Ariño-Durand, Louis-Marie, Aujay de La Dure, Geoffroy, Aznar Sánchez, Carolina, Banderier, Gilles, Bar Asher, Méir, Baudoin, Anne-Catherine, Beaud, Mathieu, Benoteau-Alexandre, Marie-Ève, Bertin-Hugault, Anne, Bourgeois, Christophe, Bruyère, Agnès (Nathalie), Burnet, Régis, Chango, Paul-Marie Fidèle, Costa, José, Costea, Andrei, Cronier, Jean, Decout, Maxime, Delanoy, Blandine, Duclos-Grenet, Pauline, Durand, Emmanuel, Ente, Benoît, Évin, Jacques, Ferran, France, Friche, François, Galand, David, Galinier-Pallerola, Jean-François, Gałuszka, Tomasz, Gardeil, Pierre, Gérain, Sybille, Giambrone, Anthony, Gil, Marie, Giraud, Hervé, Gosselin-Noat, Monique, Heldt, Petra, Lafontaine, Xavier, Leroy, Marc, Lozier, Anne-Claire, Mahieu, Bieke, Marie-Ancilla, Méténier, Étienne, Mézin, Gonzague, Micos, Pauline, Millet, Clément, Moulin, Isabelle, Mouquin, Sophie, Pinon, Esther, Popko, Łukasz, Prigent, Gaël, Rico, Christophe, Rigot, Ioan, Robert, Olivier, Rousseau-Minier, Marjorie, Ruggeri, Marc, Ruzer, Serge, Saint-Aubin, Marie-Madeleine (Lucie), Silly, Renaud, Taillandier, Marie-Claire, Tavardon, Augustin (Paul), van Tooren, Marjolein, Vargas, Jorge, Venard, Olivier-Thomas, Vincent, David, Wohlman, Avital, Venard, Olivier-Thomas, Álvares, Maria Cristina, Ariño-Durand, Louis-Marie, Aujay de La Dure, Geoffroy, Aznar Sánchez, Carolina, Banderier, Gilles, Bar Asher, Méir, Baudoin, Anne-Catherine, Beaud, Mathieu, Benoteau-Alexandre, Marie-Ève, Bertin-Hugault, Anne, Bourgeois, Christophe, Bruyère, Agnès (Nathalie), Burnet, Régis, Chango, Paul-Marie Fidèle, Costa, José, Costea, Andrei, Cronier, Jean, Decout, Maxime, Delanoy, Blandine, Duclos-Grenet, Pauline, Durand, Emmanuel, Ente, Benoît, Évin, Jacques, Ferran, France, Friche, François, Galand, David, Galinier-Pallerola, Jean-François, Gałuszka, Tomasz, Gardeil, Pierre, Gérain, Sybille, Giambrone, Anthony, Gil, Marie, Giraud, Hervé, Gosselin-Noat, Monique, Heldt, Petra, Lafontaine, Xavier, Leroy, Marc, Lozier, Anne-Claire, Mahieu, Bieke, Marie-Ancilla, Méténier, Étienne, Mézin, Gonzague, Micos, Pauline, Millet, Clément, Moulin, Isabelle, Mouquin, Sophie, Pinon, Esther, Popko, Łukasz, Prigent, Gaël, Rico, Christophe, Rigot, Ioan, Robert, Olivier, Rousseau-Minier, Marjorie, Ruggeri, Marc, Ruzer, Serge, Saint-Aubin, Marie-Madeleine (Lucie), Silly, Renaud, Taillandier, Marie-Claire, Tavardon, Augustin (Paul), van Tooren, Marjolein, Vargas, Jorge, Venard, Olivier-Thomas, Vincent, David, and Wohlman, Avital

Published
2021
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34. Improving the Corrosion Resistance of Polished Stainless Steel Parts by Introducing the Energy of the Ultrasonic Field into the Forming Zone

Author

Mezin, Nikolay, Shtubov, Nikita, and Kiselev, Evgeni

Abstract

This article presents the results of experimental studies of the effect of processing conditions on the corrosion resistance of polished parts made of stainless chromium-nickel steel of austenite class 12X18N10T. It is established that the introduction of the ultrasonic field energy into the shaping zone during grinding can increase the corrosion resistance of parts by 12-15 %, depending on the elements of the grinding mode. The latter is explained by a significantly lower heat-force stress during processing, which, in turn, leads to a decrease in the magnitude and depth of distribution of technological residual tensile stresses along the surface layer.

Published
2022
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35. Using a Computer Simulator to Create a Digital Model of a CCGT Power Unit

Author

Arakelyan, E.K., Andryushin, A.V., Mezin, S.V., Pashchenko, F.F., and Kosoy, A.A.

Abstract

The report is devoted to the problem of creating a digital model of an energy facility in relation to the PGU-450T power unit in the absence of the possibility of conducting field experiments to obtain initial information. Under these conditions, it is proposed to use a computer simulator of the object under study. The main provisions of checking the adequacy and validity of the simulator to a real object for static and dynamic processes are outlined, examples of such verification are given. The application of a specific simulator of JSC "Simulators for power plants" for modeling the parameters of the equipment of the CCGT and the CCGT as a whole during the operation of the CCGT at reduced loads is justified. The results of the numerical experiments carried out on the simulator were used to create a digital model of the PGU-450T in the form of a data library and regression equations in the form of dependencies of the main indicators of gas turbines, waste heat boilers, steam turbines and CCGT as a whole on the power of the CCGT, outdoor air temperature, fuel consumption for various modes of gas and steam turbines. The effectiveness of the use of the digital model of the PGU-450T (Russian model of CCGT) is illustrated by the example of solving the practical problem of optimal distribution of the total load of the GTU between gas turbines when the CCGT operates at reduced loads.

Published
2022
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36. Application of the basic principles of "Industry 4.0" in the intellectualization of automated control systems of modern thermal power plants.

Author

Arakelyan, E.K., Kosoy, A.A., Mezin, S.V., and Pashchenko., F.F.

Subjects
AUTOMATIC control systems, INDUSTRY 4.0, PROCESS control systems, POWER plants, GAS power plants, INTELLIGENCE levels, INTELLIGENT buildings
Abstract

The description of the basic principles embodied in the concept of "Industry 4.0" and the possibility of their use in the development of measures aimed at increasing intelligence of modern control system of thermal power plants (TPP) the results of the analysis of the level of intelligence and efficiency of modern control system on the basis of program-technical complexes (PTС). It is shown that, despite the widespread introduction of modern PTС in the construction of new and modernization of existing ACSTPs of TPP, the improvement of the quality of technological processes, optimization of their modes and parameters, occurs mainly at the level of equipment and power unit and to a much lesser extent - at the level of power plants and as a result-an insufficient level of automation and low technical and economic efficiency. Another conclusion from the analysis - when implementing control algorithms in automated process control systems based on modern PTC, their wide information and software capabilities are not fully used. The main ways of further improvement of ACSTPs in order to increase their efficiency and the level of intelligence of ACSTPs of power plants based on PTC are given The assessment of the level of increase in intelligence and economic effect from the implementation of the proposed solutions is given. [ABSTRACT FROM AUTHOR]

Published
2021
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37. Taking into Account the Operating Modes of the Steam Turbine Stage When Modeling Hydrodynamic and Thermal Processes in Non-Steam and Motor Modes.

Author

Arakelyan, E.K., Pikina, G.A., Andryushin, A.V., Mezin, S.V., Andryushin, K.A., and Pashchenko, F.F.

Subjects
STEAM-turbines, STEAM flow, MOTORS, TURBINES, HEAT equation
Abstract

The modes of operation of the steam turbine stage in non-steam and motor modes are considered. Analytical models of the operation of the stage with and without active power generation are obtained. Calculation expressions are given for quantifying the maximum allowable total power of the turbine stages when the cooling steam flows into the flow part of the turbine from the safety condition of the generator when it is accidentally disconnected from the network. The efficiency of the method using experimental data of the motor mode on the K-200 power unit is shown. Based on the thermal and balance equations, a condition is obtained that excludes the possibility of external steam flows into the intermediate stages of the turbine affecting the parameters of the steam flowing through the flow part. [ABSTRACT FROM AUTHOR]

Published
2020
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38. Features of steam turbine stages operation in low-flow modes when modeling hydrodynamic processes in the turbine in steamless and motor modes.

Author

Arakelyan, E.K., Pikina, G.A., Andryushin, A.V., Mezin, S.V., Andryushin, K.A., Kosoy, A.A., and Pashchenko, F.F.

Subjects
STEAM-turbines, TURBINES, FRICTION losses, MOTORS, STEAM flow, VENTILATION
Abstract

Operating peculiarities of the stage, the group of stages and the flow part of the steam turbine in low-flow modes, in particular, in the steamless and motor modes, and methodological approaches to their registration in the simulation of hydrodynamic processes are considered. Particular attention is paid to determining the power losses for friction and ventilation. The technique and algorithm for calculating the temperature state of the flow part of a steam turbine are given. Based on the conducted studies, the regularities characteristic of the motor regime of most steam turbines are revealed. [ABSTRACT FROM AUTHOR]

Published
2020
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39. Methodological provisions of the assessment of the increase degree in the intelligence of TPP ACS TP on the basis of modern PTC.

Author

Arakelyan, E.K., Andryushin, A.V., Pashchenko, F.F., Mezin, S.V., and Kosoy, A.A.

Subjects
INTELLIGENT control systems, ARTIFICIAL intelligence, TRANSACTION systems (Computer systems), PRODUCTION control, MANUFACTURING processes
Abstract

The article deals with the actual problems of increasing the intelligence of TPP (thermal power plant) control systems built on the basis of modern software and hardware complexes (PTC). It is shown that from the standpoint of the modern approach, complex technical control systems satisfy the definition of intelligent control systems as systems that act rationally and optimally. It is from these positions that the report considers the problems of increasing the intelligence of the TPP control system on the basis of creating a unified system of optimal control of technological and production processes in all modes of operation of power units and the station as a whole. As assessment of the degree of intellectuality is considered the conventional "factor of intelligence", which is the fraction of intelligent technologies in the total functions of ACS on a given task or level of control. [ABSTRACT FROM AUTHOR]

Published
2020
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41. The Use of Phosphate Compositions for Chemical Treatment of Make-Up Water in Centralized Heat Supply Systems

Author

Ponurko, I.V., Krylova, S.A., Limarev, A.S., and Mezin, I.Y.

Abstract

The paper investigates two different protective phosphate compositions for the chemical treatment of water and the use of them with a view to improve quality of “make-up” water in water heating systems. The research proved a positive effect of reducing the corrosive activity of water and the amount of scale.

Published
2021
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42. Problems of Optimizing the Modes of CHP with a Complex Composition of Equipment, Taking into Account the Peculiarities of the CCGT and Steam Power Units Operation

Author

Arakelyan, E.K., Andryushin, A.V., Mezin, S.V., Pashchenko, F.F., Kosoy, A.A., and Yagupova, Y.Y.

Abstract

The problem of optimal control of the CHP operation modes with a complex composition of equipment, including traditional heating units and steam-gas installations, is considered. The features of the operation of the specified equipment at reduced loads and their consideration in the optimization of modes based on a multi-criteria approach, taking into account the criteria of efficiency and reliability, are given. The possibility of applying various mathematical methods of multi-criteria optimization to solve the problem in the presence of a large number of internal and external constraints and conditions in the form of inequalities and balance equations is considered. The features of solving this problem at various stages of entering the power and capacity market of the CHP are presented.

Published
2021
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43. Possibilities and Problems of Station-Level Optimization Algorithms Integration into the Application Software of Program and Technical Complexes of Intelligent Automated Control Systems of TPP

Author

Arakelyan, E.K., Mezin, S.V., Pashchenko, F.F., and Kosoy, A.A.

Abstract

The possibility of integrating station-level optimization algorithms into the application software of modern program and technical complexes (PTC) in order to increase the intelligence of the automatic control systems (ACS TP) of thermal power plants is considered. On the example of the actual task of managing the current technical and economic indicators (TEI) of power units, general methodological approaches to the development of a set of algorithms for calculating and analyzing current and regulatory indicators are considered. The article presents a methodology for evaluating the feasibility and timing of restoration work on the cooling surfaces of the condenser in order to eliminate or minimize the deviation of the current increased pressure in it from the standard value. The complexities of integrating algorithms for calculating and analyzing block-level TEI into the PTC software are shown, and possible ways to solve them are given.

Published
2021
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47. [Management of muscle and nerve biopsies: expert guidelines from two French professional societies, Société française de myologie et de l'Association française contre les myopathies] : Prise en charge des biopsies musculaires et nerveuses. Recommandations formalisées d'experts sous l'égide de la Société française de neuropathologie, de la Société française de myologie et de l'Association française contre les myopathies

Author

Uro-Soste, E., Fernandez, C., Authier, François-Jérôme, Bassez, G., Butori, C., Chapon, F., Delisle, M.-B., Dubourg, O., Feasson, L., Gherardi, R., Lacroix, C., Laquerriere, A., Letournel, F., Magy, Laurent, Maisonobe, T., Marcorelles, Pascale, Maurage, C.-A., Mezin, P., Mussini, J.-M., Penisson-Besnier, I., Romero, N.-B., Streichenberger, N., Vallat, Jean-Michel, Viennet, G., Vital, A., Voit, T., Boucharef, W., Figarella-Branger, D., Renseigné, Non, Service d'anatomie pathologique et histologie-cytologie [Rangueil], Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Hôpital de Rangueil, CHU Toulouse [Toulouse], Centre de référence des maladies rares neuromusculaires Aquitaine-Grand Sud Ouest, Service d'anatomie pathologique et de neurophatologie, Assistance Publique - Hôpitaux de Marseille (APHM)-CHU Marseille, Centre de référence des maladies rares neuromusculaires, INSERM U955, équipe 10, Département de pathologie [Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Centre de référence des maladies rares neuromusculaires, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor, Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Laboratoire de pathologie clinique et expérimentale, Centre Hospitalier Universitaire de Nice (CHU Nice), Service de Neurologie [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Centre de compétences pathologies neuromusculaires [CHU Caen], Thérapie des maladies du muscle strié, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de physiologie clinique, unité de myologie, CHU Saint-Etienne, Centre de référence des neuropathies amyloïdes et autres neuropathies rares, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Laboratoire de neuropathologie, Service d'Anatomie et Cytologie Pathologique [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Département de Pathologie Cellulaire et Tissulaire, Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Laboratoire de Neurobiologie et Neuropathologie, Service de Neurologie [CHU Limoges], CHU Limoges, Biomolécules Thérapies anti-tumorales (EA4021), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503), Centre de référence national neuropathies périphériques rares [CHU Limoges], Service d'Anatomomie pathologique, Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Centre de compétence des maladies rares neuromusculaires, Service d'anatomie pathologique, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Département d'anatomie et cythologie pathologique, CHU Grenoble-Hôpital Michallon, CHU Grenoble, Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Service de neurologie [Angers], Université d'Angers (UA)-Centre Hospitalier Universitaire d'Angers (CHU Angers), Service de pathologie et neuropatologie, Hospices Civils de Lyon (HCL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Estrogènes, Expression génique et pathologies du Système Nerveux Central - UFC (E2SNC / ESTROGENES), Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Service de pathologie [Bordeaux], Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, CHU Bordeaux [Bordeaux], Service des actions médicales, paramédicales et psychologiques, Association Française contre les Myopathies, Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse], IMRB - 'Biologie du système neuromusculaire' [Créteil] (U955 Inserm - UPEC), École nationale vétérinaire - Alfort (ENVA)-Institut Mondor de Recherche Biomédicale (IMRB), Centre de référence des maladies rares neuromusculaires [CHU Pitié-Salpétriêre], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université de Toulouse (UT)-Université de Toulouse (UT)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre de Reference des Maladies Neuromusculaires - Atlantique Occitanie Caraïbe (CRMN - AOC), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), and Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E)

Subjects
MESH: Muscle, Skeletal, [SDV.GEN]Life Sciences [q-bio]/Genetics, MESH: Humans, [SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology, MESH: Paraffin Embedding, MESH: Immunohistochemistry, [SDV.BBM.BM]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Molecular biology, [SDV.MHEP.CHI]Life Sciences [q-bio]/Human health and pathology/Surgery, MESH: Peripheral Nerves, MESH: Biopsy, [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], MESH: Fixatives, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology, MESH: Glutaral, ComputingMilieux_MISCELLANEOUS
Abstract

International audience

Published
2010
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48. A new ACTA1 mutation in two unrelated sporadic cases of neonatal form of CFTD

Author

Deconinck, N., Monnier, N, Commare, Mc, Mezin, P, Michotte, Alex, Lunardi, J, and Anatomy

Abstract

Congenital Fibre Type Disproportion (CFTD) is defined by isolated uniform atrophy of type I fibres, at least 12% smaller than type II fibres in absence of structural changes. CFTD is also clinically and genetically heterogeneous. Recessive, dominant and X-linked inheritance patterns have been described. So far mutations in three genes have been shown to cause CFTD, ACTA1 in three sporadic cases with lethal neonatal presentation, SEPN1 in one recessive family and TPM3 in a panel of patients with dominant transmission and variable clinical course. We report herein the identification of a new ACTA1 mutation in two unrelated sporadic cases of neonatal form of CFTD. Both patients presented with a major axial and peripheral hypotonia during the first year of life. There was neither dysmorphy nor ophthalmoplegia but both presented with a myopathic facies, high arched palate. Further clinical course included delayed motor milestones, general amyotrophia, global hyperlaxity, and scoliosis diagnosed between the ages of 2 and 4. For one of the patients swallowing troubles required feeding with nasogastric catheter. Both patients encountered respiratory complications requiring nocturnal mechanical ventilatory assistance. Autonomous walking was acquired at 3 years of age for one and 4 years for the second one. Clinical evaluation at 4 years of age indicated real although slow improvements. Muscle biopsy showed in both patients a typical pattern of congenital myopathy with uniform atrophy of type I fibres, that were significantly smaller than type II fibres and in absence of structural changes. A c.764A>G change leading to the p.Glu251Gly substitution in the mature form of ACTA1 protein was identified in both children. The good correlation between clinical presentation and histological data may suggest that the pathogenic mechanism leading to fibre type disproportion could be specific of this new ACTA1 mutation identified in both children.

Published
2009

49. Quality Evaluation of Platinit Wire by Different Producers

Author

Kasatkina, E.G., Mezin, I.Y., Limarev, A.S., and Somova, J.V.

Abstract

The study presents a comparative analysis of consumer properties of platinit by different producers. Both the products of Russian companies and the foreign counterparts are considered. It was established that the domestic platinit, though not very different in composition from its foreign analogues, is still inferior to them in its parameters, such as physical and technological properties, as well as surface quality.

Published
2017
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