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Gaucher disease (GD), the most common lysosomal storage disorder (LSD), is caused by defects in the activity of the lysosomal enzyme, glucocerebrosidase, resulting in intracellular accumulation of glucosylceramide (GlcCer). Neuronopathic forms, which comprise only a small percent of GD patients, are characterized by neurological impairment and neuronal cell death. Little is known about the pathway
The removal of chemical oxygen demand (COD) from wastewater-containing phenol was investigated using three-phase three-dimensional electrode reactor. Special attention was paid to experimentally probe the performance of the reactor in COD removal in the process of repeated batch runs. The experimental results showed that the reactor could remove COD from phenol-containing wastewater much more effi
The relation between the atrioventricular conduction properties of the atrioventricular node and the anterograde conduction ability over the accessory pathway in the Wolff-Parkinson-White syndrome has never been studied. Atrioventricular nodal characteristics were studied in 285 patients with manifest and 204 with concealed accessory pathway who underwent radiofrequency ablation, and compared with
Recent urban microclimate studies in Colombo, Sri Lanka, indicate that the maximum daily temperature within street canyons decreases with increasing height to width (H/W) ratio, but higher H/W ratio negatively affects street-level wind flow. There is also evidence pointing to the cooling effect of sea breeze. The nocturnal heat island is small in contrast to daytime urban-rural differences. In thi
The vitamin K-dependent anticoagulant protein S binds with high affinity to C4b-binding protein (C4BP), a regulator of complement. Despite the physiological importance of the complex, we have only a patchy view of the C4BP-binding site in protein S. Based on phage display experiments, protein S residues 447-460 were suggested to form part of the binding site. Several experimental approaches were n
We compute Poisson kernels for integer weight parameter standard weighted biharmonic operators in the unit disc with Dirichlet boundary conditions. The computations performed extend the supply of explicit examples of such kernels and suggest similar formulas for these Poisson kernels to hold true in more generality. Computations have been carried out using the open source computer algebra package
Purpose: To present an evaluation method for three-dimensional Look-Locker (3D-LL) based T1 quantification, calculating correct T1 values independent of local flip angle (FA) variations. The method was evaluated both in phantoms and in vivo in a delayed Gadolinium Enhanced MRI of Cartilage (dGEMRIC) study with 33 subjects. Materials and Methods: T1 was measured with 3D-LL, using both local FA corr
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
Sand and dust storms (SDS) represent a significant and widespread obstacle to sustainable development, affecting its economic, social, and environmental dimensions. SDS pose severe challenges to achieving several Sustainable Development Goals (SDGs), including the eradication of poverty, the achievement of zero hunger, the improvement of health and well-being, the provision of clean water and sani
This thesis investigates if dependence on natural resources causes a delayed demographic transition. The Plurinational State of Bolivia is taken as an example as it is a country with a high share of Mining \& Oil activities in its economy. The method of investigation is a time series analysis covering the period 1900--2010. The results suggest that Bolivia's resource-depleting activities d
In this study the willingness to pay and conformity-seeking behaviour of Swedish consumers when purchasing fair trade coffee were examined. It was conducted through a conjoint analysis on data collected from two different questionnaires; one that presented objective information on fair trade, and one that linked fair trade to aspirational cues. The results indicated that ethical claims of fair tra
Gestures are used in a pragmatic way in interactive contexts such as conversation. Agreement between interlocutors is an ordinary aspect of talk-in-interaction, which is expressed through various types of utterances and gestures. The interlocutors’ status, such as gender and familiarity, may affect both their verbal and non-verbal behavior as previous research has shown. This study investigates th
Canada is one of the only nations in the world that offers universal health coverage that excludes outpatient prescription drug services. This means that citizens may find out their diagnosis free of charge, but must incur out-of-pocket expenses for prescription drugs dispensed outside of hospitals. This paper examines the prevalence of prescription drug coverage in Canada across various socioecon
Over the last decade, financial disclosure and its impact on equity markets has increasingly become an area of interest. Firms suffering from poor operating performance have been shown to disclose bias earnings estimates as a means of evading negative market reactions. In this thesis, we attempt to discern whether managers purposefully bias their earnings estimates in periods of credit quality det
The purpose of this paper is to compare a collection of control variates for Monte Carlo-valuation of spread options on three assets with a view towards energy markets and to lay a foundation for continued research on control variates, e.g. combinations of control variates and adaption for quasi-Monte Carlo. The paper builds upon previous research on option pricing using Monte Carlo-simulation and
In a progressively globalized world the catapults of economic globalization, is in large, due to increased flows of foreign direct investments (FDI) across the world. FDI flows are considered essential vehicles for economic growth and development. The complex entities that embody a multinational company (MNC ) are often simplified to encompass either an exploitation approach of LDCs by MNCs or the
Vessel-source pollution is regarded as one of the major sources that harm marine environment. It has become an issue of public concern since 20th century due to a series of oil pollution incidents on the sea. Fortunately, international law has been developed to control this source of pollution taking consideration of the importance of protection and preservation of marine environment and sustainab
