Purpose: In Gaucher disease, diminished activity of the lysosomal enzyme, acid β-glucosidase, leads to accumulation of glucosylceramides and related substrates, primarily in the spleen, liver, and bone marrow. Eliglustat is an oral substrate reduction therapy approved in the European Union and the United States as a first-line treatment for adults with type 1 Gaucher disease who have compatible CY

Earth observing systems are now routinely used to infer leaf area index (LAI) given its significance in spatial aggregation of land surface fluxes. Whether LAI is an appropriate scaling parameter for daytime growing season energy budget, surface conductance (Gs), water- and light-use efficiency and surface–atmosphere coupling of European boreal coniferous forests was explored using eddy-covariance

This study examines perceptions of humour and happiness in a world where NPM ideas have a profound influence on many aspects of everyday living. The researchers interviewed comedians and their circle of co-workers at comedy festivals. Comedians seek to make us laugh, but this paper examines whether they have a serious message, too. This research reveals comedians as critical thinkers, as edge-workThis study examines perceptions of humour and happiness in a world where NPM ideas have a profound influence on many aspects of everyday living. The researchers interviewed comedians and their circle of co-workers at comedy festivals. Comedians seek to make us laugh, but this paper examines whether they have a serious message, too. This research reveals comedians as critical thinkers, as edge-work

In this paper, we propose a soft-output detector for multiple-input multiple-output (MIMO) channels that utilizes achievable information rate (AIR) based partial marginalization (PM). The proposed AIR based PM (AIR-PM) detector has superior performance compared to previously proposed PM designs and other soft-output detectors such as K-best, while at the same time yielding lower computational comp

In this paper, we propose a class of Generalized Gough-Stewart Platforms (GGSPs) used, as a novel approach, to eliminate the classical isotropic constraint of GSPs (hexapods). GGSPs are based on the standard GSP architecture with additional rotations of the three strut-pairs. Despite the architectural generalization introduced in GGSPs, they do not require much more effort in order to be fabricate

A techno-economic analysis was conducted to assess the feasibility of using forestry residues with different bark contents for bioethanol production. A proposed cellulosic ethanol biorefinery in Sweden was simulated with Aspen Plus. The plant was assumed to convert different forestry assortments (sawdust and shavings, fuel logs, early thinnings, tops and branches, hog fuel and pulpwood) to ethanol

Post-zygotic variation refers to genetic changes that arise in the soma of an individual and that are not usually inherited by the next generation. Although there is a paucity of research on such variation, emerging studies show that it is common: individuals are complex mosaics of genetically distinct cells, to such an extent that no two somatic cells are likely to have the exact same genome. Alt

Background: Diffusion magnetic resonance imaging (MRI) is the current-state-of-the-art technique to clinically investigate acute (0-24 h) ischemic stroke tissue. However, reduced apparent diffusion coefficient (ADC)-considered a marker of tissue damage-was observed to reverse spontaneously during the subacute stroke phase (24-72 h) which means that low ADC cannot be used to reflect the damaged tis

Objectives: Chronic pain is common in older adults, yet little is known of its development and the factors that predict its persistence and onset at old age. The aims of this longitudinal cohort study were to examine the prevalence and incidence of chronic pain and to explore possible risk factors for its persistence and onset in a representative sample of older Swedish adults. Method: Data were c

The use of this material under current conditions is supported by existing information. This material was evaluated for genotoxicity, repeated dose toxicity, developmental and reproductive toxicity, local respiratory toxicity, phototoxicity/photoallergenicity, skin sensitization, as well as environmental safety. Data show that this material is not genotoxic nor does it have skin sensitization pote

Traumatic brain injury (TBI) survivors frequently suffer from life-long deficits in cognitive functions and a reduced quality of life. Axonal injury, observed in many severe TBI patients, results in accumulation of amyloid precursor protein (APP). Post-injury enzymatic cleavage of APP can generate amyloid-β (Aβ) peptides, a hallmark finding in Alzheimer's disease (AD). At autopsy, brains of AD and

Traditional farming landscapes in the temperate zone that have persisted for millennia can be exceptionally species-rich and are therefore key conservation targets. In contrast to Europe’s West, Eastern Europe harbours widespread traditional farming landscapes, but drastic socio-economic and political changes in the twentieth century are likely to have impacted these landscapes profoundly. We reco

An analytical derivation of the probability density function (PDF) tail describing the strongly correlated interface growth governed by the nonlinear Kardar-Parisi-Zhang equation is provided. The PDF tail exactly coincides with a Tracy-Widom distribution i.e. a PDF tail proportional to exp (cw2 3/2), Where w 2 is the the width of the interface. The PDF tail is computed by the instanton method in t

Quantum dots (QDs) are good model systems for fundamental studies of mesoscopic transport phenomena using thermoelectric effects because of their small size, electrostatically tunable properties and thermoelectric response characteristics that are very sensitive to small thermal biases. Here we provide a review of experimental studies on thermoelectric properties of single QDs realized in two-dime

The energy and rapidity dependence of the average transverse momentum (pT) in pp and pA collisions at energies currently available at the BNL Relativistic Heavy Ion Collider (RHIC) and CERN Large Hadron Collider (LHC) are estimated using the color glass condensate (CGC) formalism. We update previous predictions for the pT spectra using the hybrid formalism of the CGC approach and two phenomenologi