WorldWideScience

Sample records for kamchatika konovalov shevlyakov

  1. [WILSON-KONOVALOV'S DISEASE IN TWO SISTERS: DIFFERENCES IN THE CLINICAL PICTURE AND SUCCESSFUL THERAPY].

    Science.gov (United States)

    Ignatova, T M; Solov'eva O V; Arion, E A; Balashova, M S; Rozina, T P

    2016-01-01

    Wilson-Konovalov's disease is a rare genetic pathology of copper metabolism that in the first place affects liver and CNS. Due to autosomal-recessive inheritance of this condition, it most frequently occurs in sibs. We report a case of Wilson-Konovalov's disease in two sisters differing in its clinical course: severe abdominal variant in the younger sister and largely neurologic form in the elder one. This observation demonstrates clinical variability of Wilson-Konovalov's disease, the possibility of its late clinical manifestation (at the age 45 years), the necessity of examination of all sibs of a proband regardless of age, and the possibility of radical improvement of prognosis even when the disease is diagnosed at the stage of decompensated liver cirrhosis.

  2. Hulluse hoomamatud piirid / Margot Visnap

    Index Scriptorium Estoniae

    Visnap, Margot, 1959-

    2006-01-01

    30. sept. Ugalas esietendunud Jaan Krossi "Keisri hull" (autoridramatiseering), lavastajad Jaak Allik ja Peeter Tammearu. Kunstnik on Jaanus Laagriküll ja muusikaline kujundaja Peeter Konovalov. Peaosas Peeter Tammearu

  3. "Keisri hull" kui müstiline fantasy / Siimon Prii

    Index Scriptorium Estoniae

    Prii, Siimon

    2006-01-01

    30. sept. Ugalas esietendunud Jaan Krossi "Keisri hull" (autoridramatiseering), lavastajad Jaak Allik ja Peeter Tammearu. Kunstnik on Jaanus Laagriküll ja muusikaline kujundaja Peeter Konovalov. Peaosas Peeter Tammearu

  4. Hulluse hoomamatud piirid / Margot Visnap

    Index Scriptorium Estoniae

    Visnap, Margot, 1959-

    2006-01-01

    30. sept. Ugalas esietendunud Jaan Krossi "Keisri hull" (autoridramatiseering), lavastajad Jaak Allik ja Peeter Tammearu. Kunstnik on Jaanus Laagriküll ja muusikaline kujundaja Peeter Konovalov. Peaosas Peeter Tammearu

  5. "Keisri hull" kui müstiline fantasy / Siimon Prii

    Index Scriptorium Estoniae

    Prii, Siimon

    2006-01-01

    30. sept. Ugalas esietendunud Jaan Krossi "Keisri hull" (autoridramatiseering), lavastajad Jaak Allik ja Peeter Tammearu. Kunstnik on Jaanus Laagriküll ja muusikaline kujundaja Peeter Konovalov. Peaosas Peeter Tammearu

  6. Komitee mehed / Mihkel Kärmas

    Index Scriptorium Estoniae

    Kärmas, Mihkel, 1974-

    1999-01-01

    Kaheksa aastat pärast ENSV KGB likvideerimist on endised julgeolekutöötajad läbi löönud äris: R. Sillar, J. Zimin, V. Pool, A. Kruuser, E. Bluman, V. Konovalov, M. Trofimov, P. Härma, M. Larsson, P. Toom jt. Ilmunud ka: Narvskaja Gazeta 9. dets. lk. 5, Vesti : Nedelja Pljus 19. nov., lk. 11

  7. New deep-sea large free-living nematodes from macrobenthos in the Kuril-Kamchatka Trench (the North-Western Pacific)

    Science.gov (United States)

    Fadeeva, Natalia; Mordukhovich, Vladimir; Zograf, Julia

    2015-01-01

    The composition of deep-sea nematode assemblages of the North-Western (NW) Pacific is poorly-studied. According to the available literature data more than 700 valid species of nematodes were reported from the depth of 400 m and deeper, out of that only 6 species were registered in the NW Pacific: 2 species from the coast of the Japanese Islands and 4 from the Sea of Japan. Ecological studies of the deep-sea nematode communities of this region are scanty and represent very scarce and fragmentary information. The first recent study of free-living nematodes collected from the abyssal plain adjacent to the Kuril-Kamchatka Trench has revealed more than 50 species of nematodes in macrobenthic samples. Families Anticomidae, Comesomatidae, Desmodoridae, Leptosomatidae, Oncholaimidae, Oxystominidae, Phanodermatidae, Siphonolaimidae and Thoracostomopsidae were the most abundant and diverse. Such taxonomic composition differs greatly from previously described meiobenthical nematode communities of NW Pacific and even World Ocean and similar to macrofaunal nematode assemblages of the Atlantic and Arctic regions. Several genera of nematodes can be considered as new records for the NW Pacific region. Micoletzkyia kamchatika sp. nov., Metaphanoderma improvisa sp. nov., and Phylloncholaimus palmaris sp. nov. are described from the Kuril-Kamchatka Trench. Micoletzkyia kamchatika sp. nov. is particularly characterized by a developed cuticular cephalic capsule underlying amphids, relatively small amphids, and the shape of the gubernaculum. Metaphanoderma improvisa sp. nov. is particularly characterized by a developed cuticular cephalic capsule, relatively large amphids, and the absence of pigmented eye spots. Phylloncholaimus palmaris sp. nov. is similar to the type species in many measurements but may be differentiated from that by the smaller amphid, shape of spicules and gubernaculums and by large precloacal papillae with four setae.

  8. Challenge for more precise e- and ion-transport in gases and liquids

    Science.gov (United States)

    White, Ron

    2016-09-01

    The full potential of technologies driven by non-equilibrium electron and ion processes in gases, liquids and soft-matter can only be realised once the basic physics has been mastered. The central component in this pursuit is an ever increasing need for the precise determination of electron and ion transport in such media. Over the last few decades, the group at James Cook University and collaborators have developed a suite of multi-term Boltzmann equation solutions to treat temporal and spatial non-locality for electrons and ions in electric and magnetic fields in gaseous systems. In this presentation, we will highlight recent developments including (i) a space-time multi-term solution of Boltzmann's equation; (ii) a unified treatment of electron and ion solutions of Boltzmann's equation which avoids mass ratio expansions; (iii) the treatment dense gases and liquids, including coherent scattering, screened potentials and (self) trapped bubble state effects, the latter of which can give rise to fractional transport behaviour, and (iv) the application to consider the self-consistency of cross-sections for electrons in biomolecules. Contributors: G. Boyle, P. Stokes, M. Casey, N. Garland, D. Cocks, D. Konovalov, S. Dujko, R. E. Robson, K. F. Ness, M. Brunger, S. Buckman, J. de Urquijo and Z. Lj. Petrovic. Support: Australian Research Council.

  9. Role of zinc and copper ions in the pathogenetic mechanisms of Alzheimer's and Parkinson's diseases.

    Science.gov (United States)

    Stelmashook, E V; Isaev, N K; Genrikhs, E E; Amelkina, G A; Khaspekov, L G; Skrebitsky, V G; Illarioshkin, S N

    2014-05-01

    Disbalance of zinc (Zn2+) and copper (Cu2+) ions in the central nervous system is involved in the pathogenesis of numerous neurodegenerative disorders such as multisystem atrophy, amyotrophic lateral sclerosis, Creutzfeldt-Jakob disease, Wilson-Konovalov disease, Alzheimer's disease, and Parkinson's disease. Among these, Alzheimer's disease (AD) and Parkinson's disease (PD) are the most frequent age-related neurodegenerative pathologies with disorders in Zn2+ and Cu2+ homeostasis playing a pivotal role in the mechanisms of pathogenesis. In this review we generalized and systematized current literature data concerning this problem. The interactions of Zn2+ and Cu2+ with amyloid precursor protein (APP), β-amyloid (Abeta), tau-protein, metallothioneins, and GSK3β are considered, as well as the role of these interactions in the generation of free radicals in AD and PD. Analysis of the literature suggests that the main factors of AD and PD pathogenesis (oxidative stress, structural disorders and aggregation of proteins, mitochondrial dysfunction, energy deficiency) that initiate a cascade of events resulting finally in the dysfunction of neuronal networks are mediated by the disbalance of Zn2+ and Cu2+.

  10. NOx emission trends in megacities derived from satellite measurements

    Science.gov (United States)

    Konovalov, Igor; Beekmann, Matthias; Richter, Andreas

    2010-05-01

    performed by means of an original algorithm enabling filtering out noisy fluctuations caused by measurement and model errors from the retrieved time series of the NOx emission estimates. Our algorithm does not require any a priori knowledge of uncertainties in the satellite measurements and in the model results or of a character of the nonlinearity; it uses artificial neural networks for fitting the trend and the probabilistic approach along with the cross validation technique for estimation of their optimal parameters. As a result, statistically significant nonlinearities of the NOx emission trends are revealed in 5 megacities (Barcelona, Madrid, Milan, Moscow and Paris) out of 12 megacities considered. Our NOx emission trend estimates are compared with the corresponding data of the EMEP emission inventory. This comparison confirmed that the emission control measures have been efficient for most of Western Europe, but indicated also possible inaccuracies of the EMEP data for some megacity regions. Results of model runs using the obtained estimates of emission changes are found to be consistent with data of independent near-surface measurements of nitrogen oxides in London, Madrid, Milan and Paris. Moreover, it is found that the nonlinear trends of NOx emissions (where they are revealed) are more consistent with near surface measurements than the linear trends. References 1. Konovalov, I.B., M. Beekmann, A. Richter, J.P. Burrows (2006), Inverse modelling of the spatial distribution of NOx emissions on a continental scale using satellite data, Atmos. Chem. Phys., 6, 1747-1770. 2. Konovalov I.B, M. Beekmann, J.P. Burrows, A. Richter (2008), Satellite measurement based estimates of decadal changes in European nitrogen oxides emissions. Atmos. Chem. Phys., 8, 2623-2641.

  11. Decrease of the Black Sea Oxygen Inventory through the second half of the XXth century

    Science.gov (United States)

    Capet, Arthur; Stanev, Emil; Gregoire, Marilaure

    2015-04-01

    We investigated the dynamics of the oxygen inventory and penetration depth in the Black Sea over the period 1955-2013 using CTD and ARGO profiles. Several authors (e.g. Codispoti et al., 1991; Konovalov and Murray, 2001; Stanev et al., 2013) have proposed that the upper interface of the suboxic layer has shoaled on a density scale during the last decades in answer to eutrophication. Those studies are based on the assumption that oxygen vertical properties are sufficiently stable, spatially and seasonally, when expressed on a density scale to permit representative interannual statistics. We used the Data Interpolating Variational Analysis (DIVA) software and in particular the de-trending algorithm (Capet et al. 2014) to explicitly evaluate the spatial, seasonal and interannual variabilities of the total Oxygen vertical content and penetration depth (in depth and density scales). The most striking result is the strong and quasi-monotonous decrease of the Black Sea total oxygen inventory which, in 2013, only amounts to 70% of what it was in 1955. The study also confirms a shoaling of the [O2]=20 mmol/m3 iso-surface, on the order of 8m (0.06 kg/m3) per decade. Profiles obtained from two Argo float (2010-2012) confirmed the present status of the properties obtained from CTD cast, and were used to assess the role of the Cold Intermediate Layer in ventilating the upper part of the Black Sea open basin. It effectively appears that the important dense water formation in the period 1985-1995 refrained the upper-mentioned trends and that the lower formation in 1995-2010 has contributed to their more recent increases.

  12. Point Defects in SiC

    Science.gov (United States)

    Zvanut, Mary Ellen

    2004-03-01

    Production of high frequency, high power electronic devices using wide bandgap semiconductors has spurred renewed interest in point defects in SiC. Recent electron paramagnetic resonance (EPR) spectroscopy studies focus on centers in as-grown high purity semi-insulating substrates because intrinsic defects are thought to compensate unavoidable shallow centers, thus creating the high resistivity required. The EPR studies address the chemical/structural composition of the defects, the defect level (energy with respect to a band edge with which the defect can accept or release an electron) and thermal stability. Thus far, the positively charged carbon vacancy, the Si vacancy, a carbon-vacancy/carbon antisite pair, and several as yet-unidentified centers have been observed in as-grown electronic-grade 4H-SiC [1-3]. The talk will review the types of defects recently identified in SiC and discuss their possible relationship to compensation. The photo-induced EPR experiments used to determine defect levels will be discussed, with a particular focus on the carbon vacancy. The use of high frequency EPR to resolve the many different types of centers in SiC will also be covered. Finally, the presentation will review the thermal stability of the intrinsic defects detected in as-grown 4H SiC. 1. M. E. Zvanut and V. V. Konovalov, Appl. Phys. Lett. 80, 410 (2002). 2. N.T. Son, Z. Zolnai, and E. Janzen, Phys. Rev. B64, 2452xx (2003). 3. W.E. Carlos, E.R. Glaser, and B.V. Shanabrook, in Proceedings of the 22nd conference on Defects in Semiconductors, Aarhus, Denmark, July 2003.

  13. Correlation for fitting multicomponent vapor-liquid equilibria data and prediction of azeotropic behavior

    Directory of Open Access Journals (Sweden)

    Khalid Farhod Chasib Al-Jiboury

    2007-01-01

    Full Text Available Correlation equations for expressing the boiling temperature as direct function of liquid composition have been tested successfully and applied for predicting azeotropic behavior of multicomponent mixtures and the kind of azeotrope (minimum, maximum and saddle type using modified correlation of Gibbs-Konovalov theorem. Also, the binary and ternary azeotropic point have been detected experimentally using graphical determination on the basis of experimental binary and ternary vapor-liquid equilibrium data.In this study, isobaric vapor-liquid equilibrium for two ternary systems: “1-Propanol – Hexane – Benzene” and its binaries “1-Propanol – Hexane, Hexane – Benzene and 1-Propanol – Benzene” and the other ternary system is “Toluene – Cyclohexane – iso-Octane (2,2,4-Trimethyl-Pentane” and its binaries “Toluene – Cyclohexane, Cyclohexane – iso-Octane and Toluene – iso-Octane” have been measured at 101.325 KPa. The measurements were made in recirculating equilibrium still with circulation of both the vapor and liquid phases. The ternary system “1-Propanol – Hexane – Benzene” which contains polar compound (1-Propanol and the two binary systems “1-Propanol – Hexane and 1-Propanol – Benzene” form a minimum azeotrope, the other ternary system and the other binary systems do not form azeotrope.All the data passed successfully the test for thermodynamic consistency using McDermott-Ellis test method (McDermott and Ellis, 1965.The maximum likelihood principle is developed for the determination of correlations parameters from binary and ternary vapor-liquid experimental data which provides a mathematical and computational guarantee of global optimality in parameters estimation for the case where all the measured variables are subject to errors and the non ideality of both vapor and liquid phases for the experimental data for the ternary and binary systems have been accounted.The agreement between prediction and experimental data is

  14. Multi-GPU three dimensional Stokes solver for simulating glacier flow

    Science.gov (United States)

    Licul, Aleksandar; Herman, Frédéric; Podladchikov, Yuri; Räss, Ludovic; Omlin, Samuel

    2016-04-01

    .C.A., Hubbard, A., Johnson, J.V., Kleiner, T., Konovalov,Y., Martin, C., Payne, A.J., Pollard, D., Price, S., Rckamp, M., Saito, F., Souk, O.,Sugiyama, S. & Zwinger, T., 2008. Benchmark experiments for higher-order and full-stokes ice sheet models (ismiphom). The Cryosphere 2, 95-108.

  15. Top-down constraints to aerosol emissions from open biomass burning: the role of gas-particle partitioning and secondary organic aerosol formation

    Science.gov (United States)

    Konovalov, Igor B.; Beekmann, Matthias; Berezin, Evgeny V.; Petetin, Hervé

    2014-05-01

    featured a unique set of the control parameter values, which were selected within the range of values reported in experimental studies of OA emissions. In addition, a model run was performed with a "standard" OA modelling scheme. The simulations were analysed in combination with data of both ground based and satellite observations of aerosol and CO in order to derive corresponding top-down BB emission estimates. These estimates together with their uncertainties were used to formally test a hypothesis that the discrepancy between the OA&BC/CO emission ratios given by bottom-up emission inventories (such as GFED3.1) and those derived from atmospheric measurements can be entirely explained by statistical uncertainties (in particular, those associated with the uncertainties in CO and OA emission factors). The results of the hypothesis testing are presented for each model configuration in terms of the confidence level, and the best configuration (featuring the highest confidence level that the above hypothesis is true) is selected. The obtained results confirm that gas-particle partitioning and oxidation of lower-volatility organic emissions can play a very important role in evolution of BB aerosol. Implications of the results of this study for validation of fire emission inventories and for inverse modelling of BB aerosol emissions are discussed. References: 1. Kaiser, J. W., Heil, A., Andreae, M. O., Benedetti, A., Chubarova, N., Jones, L., Morcrette, J.-J., Razinger, M., Schultz, M. G., Suttie, M., and van der Werf, G. R.: Biomass burning emissions estimated with a global fire assimilation system based on observed fire radiative power, Biogeosciences, 9, 527-554, doi:10.5194/bg-9-527-2012, 2012. 2. Konovalov I.B., Berezin, E.V., Ciais, P., Broquet, G., Beekmann, M., Hadji-Lazaro, J., Clerbaux, C., Andreae M.O., Kaiser, J.W., and Schulze, E.-D.: Constraining CO2 emissions from open biomass burning by satellite observations of co-emitted species: a method and its application

  16. PREFACE: Progress in the ITER Physics Basis

    Science.gov (United States)

    Ikeda, K.

    2007-06-01

    fundamental to its completion. I am pleased to witness the extensive collaborations, the excellent working relationships and the free exchange of views that have been developed among scientists working on magnetic fusion, and I would particularly like to acknowledge the importance which they assign to ITER in their research. This close collaboration and the spirit of free discussion will be essential to the success of ITER. Finally, the PIPB identifies issues which remain in the projection of burning plasma performance to the ITER scale and in the control of burning plasmas. Continued R&D is therefore called for to reduce the uncertainties associated with these issues and to ensure the efficient operation and exploitation of ITER. It is important that the international fusion community maintains a high level of collaboration in the future to address these issues and to prepare the physics basis for ITER operation. ITPA Coordination Committee R. Stambaugh (Chair of ITPA CC, General Atomics, USA) D.J. Campbell (Previous Chair of ITPA CC, European Fusion Development Agreement—Close Support Unit, ITER Organization) M. Shimada (Co-Chair of ITPA CC, ITER Organization) R. Aymar (ITER International Team, CERN) V. Chuyanov (ITER Organization) J.H. Han (Korea Basic Science Institute, Korea) Y. Huo (Zengzhou University, China) Y.S. Hwang (Seoul National University, Korea) N. Ivanov (Kurchatov Institute, Russia) Y. Kamada (Japan Atomic Energy Agency, Naka, Japan) P.K. Kaw (Institute for Plasma Research, India) S. Konovalov (Kurchatov Institute, Russia) M. Kwon (National Fusion Research Center, Korea) J. Li (Academy of Science, Institute of Plasma Physics, China) S. Mirnov (TRINITI, Russia) Y. Nakamura (National Institute for Fusion Studies, Japan) H. Ninomiya (Japan Atomic Energy Agency, Naka, Japan) E. Oktay (Department of Energy, USA) J. Pamela (European Fusion Development Agreement—Close Support Unit) C. Pan (Southwestern Institute of Physics, China) F. Romanelli (Ente per le

  17. EDITORIAL: Message from the Editor Message from the Editor

    Science.gov (United States)

    Thomas, Paul

    2010-02-01

    award was Steven A. Sabbagh et al for the paper entitled 'Resistive wall stabilized operation in rotating high beta NSTX plasmas' (Nucl. Fusion 46 635-644). Reviews Last year I announced a revival of Nuclear Fusion Reviews, following a decision by the Board of Editors. 'A review of zonal flow experiments', by Akihide Fujisawa was the first fruit of this. In 2010, we are expecting to publish further review articles, the first of which is entitled 'Gyrokinetic simulations of turbulent transport' by Xavier Garbet, Yasuhiro Idomura, Laurent Villard and Tomo-Hiko Watanabe. Letters At the 2009 Board of Editors Meeting in Atlanta, the current letters procedure was summarized and it was noted that the peer review time for Letters is quite variable. Some are accepted within a month of submission, others take longer. Since the purpose of Letters is to provide a route for rapid communication, this is quite an important matter. It was agreed that the Board of Editors would play a more active role in the Letter approval process. If a reviewer asks for a second revision the Editor or a Board of Editors member will be queried as to whether the submission should still be treated as a Letter rather than a regular Paper. The Board of Editors The following Board of Editors members reached the end of their term in 2009: Amanda Hubbard, Yaroslav Kolesnichenko, Kunioki Mima, Boris Sharkov and Michael Ulrickson. On behalf of the Nuclear Fusion Office and the Chairman of the Board of Editors, Mitsuru Kikuchi, I would like to thank them for their efforts in support of the journal. At the same time, we welcomed: Hiroshi Azechi, Xuru Duan, Richard Hawryluk, Sergey Konovalov, Bruce Lipschultz, Peter Norreys, Francesco Romanelli, Tony Taylor and Hartmut Zohm. I am sure that such an illustrious group does not need any introduction to the readers of Nuclear Fusion and I am confident that the new members can only further the success of the journal. It is with great sadness that I have to note the