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PURPOSE. To determine the proportion of male patients presenting simplex retinal degenerative disease (RD: retinitis pigmentosa [RP] or cone/cone-rod dystrophy [COD/CORD]) with mutations in the X-linked retinal degeneration genes RPGR and RP2. METHODS. Simplex males were defined as patients with no known affected family members. Patients were excluded if they had a family history of parental consa

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Popular Abstract in Swedish Hur förklarar vi vad som hände efter Big Bang? Hur är det möjligt att titta 14 miljarder år tillbaka i tiden, isolera de första miljarddelarna av en sekund, och studera vad som pågår precis där och då? Svaret är såklart, att vi inte kan det. Det vi kan göra, är att om och om igen återskapa några viktiga aspekter av de förhållanden som rådde då, och studera vad som händeA new energy regime has recently become accessible in collisions at the Large Hadron Collider at CERN. Abundant in hadron collisions, the two-jet final state explores the structure of the constituents of matter and the possible emergence of new forces of nature, in the largest momentum transfer collisions produced. The results from searches for phenomena beyond the Standard Model in the dijet angu

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Combining technology enabled learning and knowledge management is a very promising strategy to systematic competence building in software organizations. In order to set-up a combined learning and knowledge management environment and to support organization members in packaging small chunks of knowledge into courseware modules, Fraunhofer IESE developed the IntView-KM methodology. IntView-KM define

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A new hybrid ventilation system for low energy buildings has been investigated theoretically using TRNSYS. The system is based on natural ventilation but utilizes a fan during hours when the natural driving forces are not sufficient. Heat from the exhaust air is recovered in an air-to-water heat exchanger. The heated water is pumped to the incoming ventilation air where it is heat exchanged to pre

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One significant environmental impact from assembly operations includes packaging material use and subsequent waste generation. Although current practice involves reduction of unnecessary materials handling, there is potential to adapt packaging material in order to simultaneously improve the environmental performance and reduce cost in a “lean and green” mindset. Hence, with increased emphasis on

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Popular Abstract in Swedish Polymerer är en grupp av kemiska ämnen som vi idag har stor användning av för, bland annat, tillverkning av olika plaster. Det finns polymerer av en mängd olika typer varav de baserade på stärkelse och cellulosa, såkallade cellulosa- och stärkelsederivat, är av särskilt industriellt och vetenskapligt intresse då de är både biokompatibla och finns i mycket stora mängder Molar mass and molar mass distribution of polymers are parameters of fundamental importance as they influence the performance of the polymer in applications. Polysaccharide derivatives are a class of polymers that have attracted significant scientific and commercial interest as they can be manufactured from a wide range of abundant sources in nature and thereby often are both cheap and biocompatib

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Common sense tells us that cost cutting leads to saving, and spending should therefore be minimized. However, a little reflection tells us that this sometimes leads to false economies. In an organizational context, these can lead on to a downward spiral of organizational ‘suicide’. Examples of false economies may include: saving on maintenance; saving on research and development expenditure; savin

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A numerical study has been carried out for slot jet impinging on flat and concave surfaces. Five turbulence models are employed for the predictions of these strongly strained turbulent flows, namely linear eddy viscosity model (LEVM), low-Re explicit algebraic Reynolds stress model (EASM) and three different V2F models. A bounded formulation for Cμ was also introduced to account for the

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Testing is used to ensure high quality chip production. High test quality implies the application of high quality test data; however, the technology development has lead to a need of an increasing test data volume to ensure high test quality. The problem is that the test data volume has to fit the limited memory of the ATE (Automatic Test Equipment). In this paper, we propose a test data truncatio

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Initial investigations were directed at studying the interaction between polyamines and various glycosaminoglycans (GAGs). The polyamine spermine displayed binding to dermatan sulphate (DS) and heparan sulphate (HS) with similar (Kd, 3.9 x 10-4 M) and higher (Kd, 0.37 x 10-6 M) affinity, respectively, than to DNA. Antiproliferative spermine-binding DS fragments (tetra- to decasaccharides), and aff

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Combining the strengths of an advanced mathematical model of human physiology and a thermal manikin is a new paradigm for simulating thermal behaviour of humans. However, the forerunners of such adaptive manikins showed some substantial limitations. This project aimed to determine the opportunities and constraints of the existing thermal manikins when dynamically controlled by a mathematical model

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The soil salinity distribution and solute transport properties of three different soil types were investigated and compared within a project area in northeastern Egypt. For this purpose, dye tracer experiments and salinity sampling were carried out. The resulting salinity maps showed that the soil salinity in the cultivated western site of the project area is 8-10 times higher than that in the cul

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Abstract in UndeterminedAgricultural systems continuously evolve and are forced to change as a result of a range of global and local driving forces. Agricultural technologies and agricultural, environmental and rural development policies are increasingly designed to contribute to the sustainability of agricultural systems and to enhance contributions of agricultural systems to sustainable developm

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Soil pH is one of the most influential variables in soil, and is a powerful factor in influencing the size, activity and community structure of the soil microbial community. It was previously shown in a century old artificial pH gradient in an arable soil (pH 4.0-8.3) that bacterial growth is positively related to pH, while fungal growth increases with decreasing pH. In an attempt to elucidate som

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The measurement of radioactive solutions containing organic or aggressive media may cause stability problems in liquid and plastic scintillation (PS) techniques. In the case of PS, this can be overcome by adding a crosslinker to the polymer structure. The objectives of this study are to synthesise a suitable crosslinked plastic scintillator (C-PS) for radioactivity determination in organic and agg