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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 solvent-free microextraction technique based on liquid-gas-liquid microextraction was investigated for the extraction and preconcentration of 2,4-di-tert-butyl phenol from aqueous samples. In this study 16 mu L NaOH 1 M as acceptor phase was introduced in the lumen of a polypropylene hollow fiber, with one of its ends heat-sealed. A small piece (about 2 cm) of metallic wire was used to seal the
We prove a uniqueness result for autonomous divergence-free systems of ODE's in the plane and give an application to the study of water flows with vorticity.
Biogas is a renewable, high-quality fuel, currently produced at more than 200 locations in Sweden. The present production is some 5 PJ/year but the potential is approximately 10 times higher. Biogas can be produced from a wide range of raw materials, from organic waste to dedicated energy crops, and can be utilised for various energy services such as heat, combined heat and power or as a vehicle f
Popular Abstract in Swedish "Det var en gång en kung som aldrig gjorde något bra för sina undersåtar. Han gjorde faktiskt aldrig nånting. Det enda han ville var att äta och dricka, och berusa sig tillsammans med sitt hov. Under tiden svalt folket ihjäl." Anna frågar: varför kunde inte människorna äta hemma? "Tyst nu, och lyssna på fortsättningen…" (citat ur avhandlingen) Martina Camparts forskniAbstract The point of departure for this study is the author's experience as educator in a residential home for children (age 6-14) with social-emotional problems, in Italy, between 1980 and 1989. One important element of the pedagogical approach was the use of narratives (personal, of others, of fiction) to prompt children's interest/ motivation to reflect about issues of personal concern. A conv
The emerging field of proteomics has created a need for new high-throughput methodologies for the analysis of gene products. An attractive approach is to develop systems that allow for clonal selection of interacting protein pairs from large molecular libraries. In this study, we have characterized a novel approach for identification and selection of protein-protein interactions, denoted SPIRE (se
BACKGROUND. In recent studies serum levels of human glandular kallikrein 2 (hK2) demonstrated significant differences in pathologically organ-confined versus non-organ-confined prostate cancer (Pca). In this study we investigated whether hK2 adds independent information when considered together with traditionally used parameters to predict organ confined (pT2a/b) PCa. METHODS. Serum levels of hK2,
There are accelerating trends for the implementation of electronic commerce(e-commerce) as an extra marketing channel for selling products globally via the access of the Internet. This is expected to in¯uence the shape of future cities and the conservation of natural resources. This paper critically reviews the current research work to date regarding the environmental implications of e-commerce. T
