165 research outputs found

    X-Ray Scattering Measurements of the Transient Structure of a Driven Charge-Density-Wave

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    We report time-resolved x-ray scattering measurements of the transient structural response of the sliding {\bf Q}1_{1} charge-density-wave (CDW) in NbSe3_{3} to a reversal of the driving electric field. The observed time scale characterizing this response at 70K varies from \sim 15 msec for driving fields near threshold to \sim 2 msec for fields well above threshold. The position and time-dependent strain of the CDW is analyzed in terms of a phenomenological equation of motion for the phase of the CDW order parameter. The value of the damping constant, γ=(3.2±0.7)×1019\gamma = (3.2 \pm 0.7) \times 10^{-19} eV \cdot seconds \cdot \AA3^{-3}, is in excellent agreement with the value determined from transport measurements. As the driving field approaches threshold from above, the line shape becomes bimodal, suggesting that the CDW does not depin throughout the entire sample at one well-defined voltage.Comment: revtex 3.0, 7 figure

    The continual chemical changing aspects in metallurgy production scheduling

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    Due to keeping the continuous changes of the chemical composition of alloys in the sequence of manufactured products it is required to apply a specific approach at creation of models of production scheduling. The production schedule is created for the workplace continuous process at first, and then the following production schedules of work are derived for another workplaces. The model was implemented in the company RS Slovakia s.r.o

    The Bile Acid Synthesis Pathway Is Present and Functional in the Human Ovary

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    Background: Bile acids, end products of the pathway for cholesterol elimination, are required for dietary lipid and fat-soluble vitamin absorption and maintain the balance between cholesterol synthesis in the liver and cholesterol excretion. They are composed of a steroid structure and are primarily made in the liver by the oxidation of cholesterol. Cholesterol is also highly abundant in the human ovarian follicle, where it is used in the formation of the sex steroids. Methodology/Principal Findings: Here we describe for the first time evidence that all aspects of the bile acid synthesis pathway are present in the human ovarian follicle, including the enzymes in both the classical and alternative pathways, the nuclear receptors known to regulate the pathway, and the end product bile acids. Furthermore, we provide functional evidence that bile acids are produced by the human follicular granulosa cells in response to cholesterol presence in the culture media. Conclusions/Significance: These findings establish a novel pathway present in the human ovarian follicle that has the capacity to compete directly with sex steroid synthesis

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

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    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

    Megatrends and Trends Shaping Supply Chain Innovation

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    Companies operate in a macro-environment that is changing considerably due to large, transformative global forces namely megatrends and trends. The wave of these megatrends and trends generates new prospects as well as challenges for the future of supply chains. This chapter provides a review of 23 major megatrends and 72 trends identified in multiple dimensions along Political, Economic, Social, Technological, Legal, and Environmental (PESTLE) dimensions. The results are based on a systematic literature review and an experts’ workshop, and can be used to generate future supply chain scenarios

    Recent advances in amyotrophic lateral sclerosis

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    Główne wytyczne zarządzania jakością i technologią w łańcuchu dostaw na przykładzie branży metalurgicznej

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    The main objective of the study was to develop a model supporting the process of quality management and technology in the supply chain. This model will be enough general and universal in nature that the guidelines will be able to implement various production companies. Assumptions of the model have been developed during the four-month research internship author of one of the largest and most advanced foundry in Poland pressure, Meta-Zel Company. According to the author developed model should significantly improve the functioning of enterprises of the metallurgical industry through: the construction of effective forms of customer communication, the realization of the logistics processes based on customer requirements, ensuring customer service at a high level.Głównym celem pracy było opracowanie modelu wspierającego proces zarządzania jakością i technologią w łańcuchu dostaw. Model ten będzie miał na tyle ogólny i uniwersalny charakter, że jego wytyczne będą mogły implementować różne przedsiębiorstwa prowadzące działalność produkcyjną. Założenia modelu zostały opracowane podczas czteromiesięcznego stażu naukowego autora w jednej z największych i najnowocześniejszych odlewni ciśnieniowych w Polsce, frimie Meta-Zel. Zdaniem autora opracowany model powinien znacząco usprawnić funkcjonowanie przedsiębiorstw z branży metalurgicznej poprzez: budowę skutecznych form komunikacji z klientem, realizację procesów logistycznych w oparciu o wymagania klienta, zagwarantowanie obsługi klienta na wysokim poziomie, itp

    Effect of implementing standardized management systems on functioning of food supply chains

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