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Background: Gaucher disease (GD) is the most common inherited lysosomal storage disorder in humans, caused by mutations in the gene encoding the lysosomal enzyme glucocerebrosidase (GBA1). GD is clinically heterogeneous and although the type of GBA1 mutation plays a role in determining the type of GD, it does not explain the clinical variability seen among patients. Cumulative evidence from recent
As the title largely illustrates, this study seeks to investigate, from a diplomatic-history and geostrategic perspective, the key developments in Anglo-Iranian relations during what may best be dubbed their “formative years,” ranging from 1907 to 1953. Having thus provided a glimpse into pre-twentieth-century Persian–British relations, it moves to analyze the division of Persia into two spheres o
This study evaluates whether the use of an acceleration advisor leads to fuel savings, to determine the change in traffic-related emissions and to analyse changes in driving patterns on various routes. The acceleration advisor provides advice to drivers through resistance in the accelerator pedal when they try to accelerate rapidly. In a test carried out in Southern Sweden, the acceleration adviso
We describe the chemical and electromagnetic enhancements of surface-enhanced resonance Raman scattering (SERRS) for the pyridine molecule absorbed on silver clusters, in which different incident wavelength regions are dominated by different enhancement mechanisms. Through visualization we theoretically investigate the charge transfer (CT) between the molecule and the metal cluster, and the charge
The cross section for the diffractive deep-inelastic scattering process ep -> eXp is measured, with the leading final state proton detected in the H1 Forward Proton Spectrometer. The data sample covers the range x(P) < 0.1 in fractional proton longitudinal momentum loss, 0.1 < vertical bar t vertical bar < 0.7 GeV2 in squared four-momentum transfer at the proton vertex and 4 < Q(2) < 700 GeV2 in p
Objective . To evaluate the clinical performance in a paediatric population of the Lund-Malmo creatinine-based glomerular filtration rate (GFR) prediction equations, primarily developed for adults. Material and methods . Iohexol clearance was used as the gold standard in 85 paediatric Caucasian patients (0.3-17 years; 37 F/48 M). One Lund-Malmo equation was based on age and gender (LM) and one inc
Stable carbon isotopes play an important role in the interpretation of biological activity, particularly when the fossil record is studied. In combination with morphological and chemical data, isotopic information can be useful in paleontology and astrobiology. In this paper the development of a microanalytical ion beam system for the measurement of the carbon isotope ratio with high spatial resol
This paper treats estimation of the Wigner-Ville spectrum (WVS) of Gaussian continuous-time stochastic processes using Cohen's class of time-frequency representations of random signals. We study the minimum mean square error estimation kernel for locally stationary processes in Silverman's sense, and two modifications where we first allow chirp multiplication and then allow nonnegative linear comb
An improved apparatus for obtaining luminescence (delayed light emission) images of plants is described. It consists of a phosphoroscope equipped with an imaging lens and an electronic image intensifier. It is also equipped with light-sources for obtaining images with reflected light and fluorescence light. Damage to the photosynthetic system caused by virus, insects, high or low temperature, UV r
Objective: To assess the therapeutic effects of oral zileuton tablets combined with low-dose beclomethasone compared to doubling the dose of beclomethasone, in improving lung function and reducing asthma symptoms. Methods: Randomized, active-controt, double-blind, parallel, multi-center study of zileuton (400 or 600mg QID)+200 mu g beclomethasone dipropionate (BDP) BID versus ptacebo+BDP 400pg BID
A critical stage for the construction of steel-concrete composite bridges occurs during casting of the bridge deck, when the wet concrete has still not hardened. The entire construction load is then taken by the non-composite steel sections. Bracing may be needed to make the slender girders rigid enough to resist lateral torsional buckling during this phase. If temporary formwork could be attached
Abstract Replacing fossil oil with renewable resources perhaps is the most urgent challenge that the human being now is facing. Bioenergy has been considered as one of the best alternatives to fossil oil. Diverse non-edible applications of plant oil have been explored in the chemical industry recent years. However, both quantity and quality need to be improved from different oil crops for diverse
The Archaean Yilgarn craton in Western Australia is a granite greenstone belt with abundant economically important mineral reserves, including several world-class gold deposits such as the giant Kalgoorlie deposit. Structural controls on gold deposition and the fact that most deposits were formed later in the craton’s tectonic history, has resulted in the general acceptance of a late-orogenic str
This master thesis work consists of the development of new hardware and software for the control box of an azimuthal guidance system used by aircrafts during the approach to the runway. The thesis work was done at Safegate International AB as a project running along the usual development projects. When designing electronics to be used on an airfield there are a lot of regulations and standards t
Ambiguity and controversy abounds the debate over the thousands of people trying to enter the European territory and their rights, especially in their procedural form. This paper explores the latest developments in the context of the European Court of Human Rights’ and the European Court of Justice’s expansive interpretation of the right to an effective remedy and the procedural guarantees afforde
Title: The use of art for branding purposes - A study of Louis Vuitton’s art collaborations Louis Vuitton’s creative director, Marc Jacobs, personally describes the fine arts as being higher up in the social hierarchy than fashion. After all, it is known as the ultimate luxury item. This essay will explore the way that the brand uses art in various ways as a means of branding. After extensive res
Are our cities prepared for the growing and rapid urbanization expected for the coming years? How do the new social, economic and environmental challenges affect the urban context, specifically informal settlements? How can we rethink sustainable urban design in vulnerable areas? To tackle these questions, this study will have a closer look at Port Elizabeth, a coastal city in South Africa, to und
