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Gaucher disease (GD) patients cannot metabolize glycosphingolipids properly due to deficiency of the enzyme glucosylceramidase (GCase). The lack of animal model for GD has hindered comprehensive investigation of disease mechanisms and also the development of curative treatment strategies such as hematopoietic stem cell (HSC) targeted gene therapy. Previous GCase deficient mice have either been let
Purpose – The purpose of this paper is to explore how students construct narratives of themselves as information seekers in a school context where their descriptions of their information activities are assessed and graded. Design/methodology/approach – Blog posts on credibility judgements written by 28 students at a Swedish upper secondary school were analysed through a bottom-up coding process b
Quantitative and fast analysis of gas mixtures is an important task in the field of chemical, security and environmental analysis. In this paper we present a diagnostic approach based on laser-induced breakdown spectroscopy (LIBS). A polarization filter in the signal collection system enables sufficient suppression of elastically scattered light which otherwise reduces the dynamic range of the mea
Objective: The purpose was to describe and characterize what women with rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) perceive as important in considering the performance of daily occupations to perceive good health. Methods: By using a phenomenographic research approach with semi-structured interviews with nine women between the ages of 42 and 65 the core category "Being able
The G protein-coupled oestrogen receptor GPER1, also known as GPR30, has been implicated in oestrogen signalling, but the physiological importance of GPER1 is not fully understood. The GPER1 agonist G-1 has become an important tool to assess GPER1-mediated cellular effects. Here, we report that this substance, besides acting via GPER1, affects the microtubule network in endothelial cells. Treatmen
Changes of the electronic structure accompanied by charge localization and a transition to an antiferromagnetic ground state were observed in the organic semiconductor (DOEO)(4)[HgBr4]center dot TCE. Localization starts in the temperature region of about 150 K and the antiferromagnetic state occurs below 60 K. The magnetic moment of the crystal contains contributions of inclusions (droplets), and
This Letter reports a measurement of the exclusive gamma gamma -> l(+)l(-) (l = e, mu) cross-section in proton-proton collisions at a centre-of-mass energy of 7 TeV by the ATLAS experiment at the LHC, based on an integrated luminosity of 4.6 fb(-1). For the electron or muon pairs satisfying exclusive selection criteria, a fit to the dilepton acoplanarity distribution is used to extract the fiducia
Bet-hedging is defined as a strategy that reduces the temporal variance in fitness at the expense of a lowered arithmetic mean fitness. After a few technical points, we will here argue that this definition of bet-hedging is badly suited for models with density- and/or frequency-dependent fitness. A strict interpretation cannot be employed; possibly, a modified definition is necessary.
The focus of this paper is on the development of a single phase PM machine with wave-windings where the design variables are the winding layout and design of a soft magnetic powder core for a stator. A number of BH curves are synthesised in order to characterise some of the existing powder cores and to study the influence of magnetic permeability on starting torque and mean driving torque. The goa
Background: Embryonic stem cells (ESC) have the capacity to self-renew and remain pluripotent, while continuously providing a source of a variety of differentiated cell types. Understanding what governs these properties at the molecular level is crucial for stem cell biology and its application to regenerative medicine. Of particular relevance is to elucidate those molecular interactions which gov
Background: Breathlessness and exercise intolerance frequently impact the daily life of patients with COPD. Methods: This double-blind, multicentre, three-period crossover study randomised 111 patients with COPD (mean age 64 years, mean FEV1 38% of predicted normal) to budesonide/formoterol 320/9 mu g, formoterol 9 mu g or placebo, twice daily for 1 week, following a 1-week run-in period with 1-we
The American Society of Nuclear Cardiology and the Society of Nuclear Medicine conclude that incorporation of attenuation corrected (AC) images in myocardial perfusion scintigraphy (MPS) will improve diagnostic accuracy. The aim was to investigate the value of adding AC stress-only images for the decision whether a rest study is necessary or not.
The exothermic nature of oxidation causes nearly all semiconductor applications in various fields like electronics, medicine, photonics, and sensor technology to acquire an oxidized semiconductor surface part during the application manufacturing. The significance of understanding and controlling the atomic scale properties of oxidized semiconductor surfaces is expected to increase even further wit
