Monitoring of cfrp-strengthened reinforced concrete bridge spans in low temperature conditions

The article discusses strengthening bridges using composite materials at extreme low temperatures. Provides the results some experimental studies FRP strengthened concrete samples at low temperatures

Разработка и формализация корпоративной стратегии предприятия

Выпускная квалификационная работа содержит 110 стр., 19 таблиц, 15 рисунков, 30 использованных источников, 2 приложения. Ключевые слова: корпоративная стратегия, SWOT-анализ, матрица Маккински, матрица Томпсона и Стрикленда, модель Артур де Литтл, модель «5 сил Портера», ключевые показатели эффективности, система управления по показателям, корпоративная социальная ответственность. Объектом исследования является корпоративная стратегия ОАО «ТЭМЗ». Целью дипломной работы является рассмотрение проблем разработки и формализации корпоративной стратегии предприятия. В процессе исследования использованы законодательные и методические материалы, учебные пособия, публикации в специальных журналах, связанные с вопросами корпоративного управления. В результате исследования была осуществлена разработка и формализация корпоративной стратегии ОАО «ТЭМЗ». Основные конструктивные, технологические и технико-эксплуатационные характеристики: введение раскрывает актуальность, цель исследования, теоретическую и практическую значимость работы, обосновывается выбор объекта и предмета исследования. В первой главе раскрыты теоретические основы разработки корпоративной стратегии. Во второй главе дана краткая характеристика предприятия, проведен анализ системы корпоративного управления на предприятии ОАО «ТЭМЗ». В третьей главе рассмотрен процесс разработки и формализации стратегии управления для ОАО «ТЭМЗ». Заключение содержит анализ результатов теоретических и экспериментальных исследований работы. Степень внедрения: одна из предложенных в результате разработки и формализации корпоративных стратегий уже принята на ОАО «ТЭМЗ» и включена в соответствующие разделы инвестиционного бизнес-плана. Область применения: полученные результаты разработки и формализации корпоративной стратегии, эффективности социальной ответственности управления могут быть использованы в управленческой работе ОАО «ТЭМЗ»». Экономическая эффективность/значимость работы. Разработанные и формализованные корпоративные стратегии позволят ОАО «ТЭМЗ» повысить производительность труда, уменьшить текучесть кадрового потенциала и производственный травматизм.Final qualifying work contains 110 pages, 19 tables, 15 figures, 30 of the used sources, 2 appendices. Key words: corporate strategy, SWOT analysis, matrix o Machinski, matrix Thompson and Strickland model Arthur de little, model "5 forces of porter", key performance indicators, control system indicators, corporate social responsibility. The object of study is the corporate strategy of JSC "TEMZ". The aim of the thesis is to examine the problems of the development and formalization of corporate strategy. In the process of the study used legislative and methodical materials, textbooks, publications in professional journals related to issues of corporate governance. The study was carried out to develop and formalization of the corporate strategy of JSC "TEMZ". The basic constructive, technological and technical-operational characteristics: the introduction reveals the relevance, research objective, theoretical and practical significance of the research, justify the choice of object and subject of research. The first Chapter describes theoretical basis of development of corporate strategy. The second Chapter gives a brief description of the enterprise, the analysis of the system of corporate management of JSC "TEMZ". The third Chapter describes the development process and the formalization of the strategy management for JSC "TEMZ". The conclusion contains an analysis of the results of theoretical and experimental research work. Degree of implementation: one of the proposed in the development and formalization of corporate strategies already adopted at JSC "TEMZ" and included in relevant sections of the investment business plan. Application field: the results of the development and formalization of corporate strategy, efficiency, social responsibility management can be used in managerial work of JSC "TEMZ". Economic efficiency and significance of the work. Developed and formalized corporate strategy will allow JSC "TEMZ" to increase productivity, to decrease the fluidity of human resources and industrial injuries

Loss of function mutations in HARS cause a spectrum of inherited peripheral neuropathies.

Inherited peripheral neuropathies are a genetically heterogeneous group of disorders characterized by distal muscle weakness and sensory loss. Mutations in genes encoding aminoacyl-tRNA synthetases have been implicated in peripheral neuropathies, suggesting that these tRNA charging enzymes are uniquely important for the peripheral nerve. Recently, a mutation in histidyl-tRNA synthetase (HARS) was identified in a single patient with a late-onset, sensory-predominant peripheral neuropathy; however, the genetic evidence was lacking, making the significance of the finding unclear. Here, we present clinical, genetic, and functional data that implicate HARS mutations in inherited peripheral neuropathies. The associated phenotypic spectrum is broad and encompasses axonal and demyelinating motor and sensory neuropathies, including four young patients presenting with pure motor axonal neuropathy. Genome-wide linkage studies in combination with whole-exome and conventional sequencing revealed four distinct and previously unreported heterozygous HARS mutations segregating with autosomal dominant peripheral neuropathy in four unrelated families (p.Thr132Ile, p.Pro134His, p.Asp175Glu and p.Asp364Tyr). All mutations cause a loss of function in yeast complementation assays, and p.Asp364Tyr is dominantly neurotoxic in a Caenorhabditis elegans model. This study demonstrates the role of HARS mutations in peripheral neuropathy and expands the genetic and clinical spectrum of aminoacyl-tRNA synthetase-related human disease

Mutation of FIG4 causes neurodegeneration in the pale tremor mouse and patients with CMT4J

Membrane-bound phosphoinositides are signalling molecules that have a key role in vesicle trafficking in eukaryotic cells(1). Proteins that bind specific phosphoinositides mediate interactions between membrane-bounded compartments whose identity is partially encoded by cytoplasmic phospholipid tags. Little is known about the localization and regulation of mammalian phosphatidylinositol-3,5-bisphosphate ( PtdIns( 3,5)P-2), a phospholipid present in small quantities that regulates membrane trafficking in the endosome - lysosome axis in yeast(2). Here we describe a multi-organ disorder with neuronal degeneration in the central nervous system, peripheral neuronopathy and diluted pigmentation in the 'pale tremor' mouse. Positional cloning identified insertion of ETn2 beta ( early transposon 2 beta)(3) into intron 18 of Fig4 (A530089I17Rik), the homologue of a yeast SAC ( suppressor of actin) domain PtdIns(3,5) P-2 5-phosphatase located in the vacuolar membrane. The abnormal concentration of PtdIns( 3,5) P2 in cultured fibroblasts from pale tremor mice demonstrates the conserved biochemical function of mammalian Fig4. The cytoplasm of fibroblasts from pale tremor mice is filled with large vacuoles that are immunoreactive for LAMP-2 (lysosomal-associated membrane protein 2), consistent with dysfunction of the late endosome - lysosome axis. Neonatal neurodegeneration in sensory and autonomic ganglia is followed by loss of neurons from layers four and five of the cortex, deep cerebellar nuclei and other localized brain regions. The sciatic nerve exhibits reduced numbers of large-diameter myelinated axons, slowed nerve conduction velocity and reduced amplitude of compound muscle action potentials. We identified pathogenic mutations of human FIG4 (KIAA0274) on chromosome 6q21 in four unrelated patients with hereditary motor and sensory neuropathy. This novel form of autosomal recessive Charcot - Marie - Tooth disorder is designated CMT4J.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/62835/1/nature05876.pd

Accumulation of Endogenous LITAF in Aggresomes

LITAF is a 161 amino acid cellular protein which includes a proline rich N-terminus and a conserved C-terminal domain known as the simple-like domain. Mutations in LITAF have been identified in Charcot-Marie tooth disease, a disease characterized by protein aggregates. Cells transfected with cellular LITAF reveal that LITAF is localized to late endosomes/lysosomes. Here we investigated the intracellular localization of endogenous LITAF. We demonstrated that endogenous LITAF accumulates at a discrete cytoplasmic site in BGMK cells that we identify as the aggresome. To determine the domain within LITAF that is responsible for the localization of LITAF to aggresomes, we created a construct that contained the C-terminal simple-like domain of LITAF and found that this construct also localizes to aggresomes. These data suggest the simple-like domain is responsible for targeting endogenous LITAF to the aggresome

Разработка автоматизированного ИТП жилого здания

Объектом разработки системы является жилой дом с инженерными сетями в микрорайоне «Северный» в Заречном поселении Томского района Томской области. Целью работы является разработка системы мониторинга и управления теплопотреблением здания, которая позволит вести точный учет потребляемой тепловой энергии, регулировать объем потребления в зависимости от текущих погодных условий, обеспечивать экономию энергоресурсов. В результате разработана система, содержащая в себе компоненты, позволяющие производить учет и управление теплопотреблением здания. Причем все данные о работе системы, объемах потребления и параметрах теплоносителя поступают диспетчеру, имеющему возможность отслеживать все параметры системы удаленно.The object of the development of the system is a residential building with engineering services in the neighborhood "North" in Zarechny settlement Tomsk region Tomsk region. The aim is to develop a building heat consumption monitoring and control system that will keep accurate records of heat energy consumption, adjusted consumption, depending on the current weather conditions, to ensure energy saving. As a result, we developed a system, which contains the components to allow for registration and control of heat consumption of the building. Moreover, all data on the system performance, volume and consumption parameters receives coolant controller having the ability to track all system parameters remotely

Genetic spectrum of hereditary neuropathies with onset in the first year of life

Early onset hereditary motor and sensory neuropathies are rare disorders encompassing congenital hypomyelinating neuropathy with disease onset in the direct post-natal period and Dejerine–Sottas neuropathy starting in infancy. The clinical spectrum, however, reaches beyond the boundaries of these two historically defined disease entities. De novo dominant mutations in PMP22, MPZ and EGR2 are known to be a typical cause of very early onset hereditary neuropathies. In addition, mutations in several other dominant and recessive genes for Charcot–Marie–Tooth disease may lead to similar phenotypes. To estimate mutation frequencies and to gain detailed insights into the genetic and phenotypic heterogeneity of early onset hereditary neuropathies, we selected a heterogeneous cohort of 77 unrelated patients who presented with symptoms of peripheral neuropathy within the first year of life. The majority of these patients were isolated in their family. We performed systematic mutation screening by means of direct sequencing of the coding regions of 11 genes: MFN2, PMP22, MPZ, EGR2, GDAP1, NEFL, FGD4, MTMR2, PRX, SBF2 and SH3TC2. In addition, screening for the Charcot–Marie–Tooth type 1A duplication on chromosome 17p11.2-12 was performed. In 35 patients (45%), mutations were identified. Mutations in MPZ, PMP22 and EGR2 were found most frequently in patients presenting with early hypotonia and breathing difficulties. The recessive genes FGD4, PRX, MTMR2, SBF2, SH3TC2 and GDAP1 were mutated in patients presenting with early foot deformities and variable delay in motor milestones after an uneventful neonatal period. Several patients displaying congenital foot deformities but an otherwise normal early development carried the Charcot–Marie–Tooth type 1A duplication. This study clearly illustrates the genetic heterogeneity underlying hereditary neuropathies with infantile onset