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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

Identification of the causes of past climate change requires detailed knowledge of one of the most important natural factors—solar forcing. Prior to the period of direct solar observations, radionuclide abundances in natural archives provide the best-known proxies for changes in solar activity. Here we present two independent reconstructions of changes in solar activity during the last 1000 yr, wh

We report on the phase behavior of poly(9,9-dihexylfluorene) (PF6), poly(9,9-diheptylfluorene) (PF7), poly(9,9-diocylfluorene) (PF8) poly(9,9-dinonylfluorene) (PF9), and poly(9,9-didecylfluorene) (PF10) in methylcyclohexane (MCH). After a heating-cooling cycle, in the 10-50 mg/mL. concentration range. PF6/ MCH. PF7/MCH. PF8/MCH, and PF9/MCH systems were found to be gel-like, while PF10/MCH appears

We use agarose gel electrophoresis to characterize how the monovalent catioinic surfactant cetyltrimethylammonium bromide (CTAB) compacts double-stranded DNA, which is detected as a reduction in electrophoretic DNA velocity. The velocity reaches a plateau at a ratio R = 1.8 of CTAB to DNA-phosphate charges, i.e., above the neutralization point, and the complexes retain a net negative charge at lea

The adsorption and dissociation of carbon monoxide were studied with plane-wave density functional theory on flat Rh(111), stepped and kinked Rh(553), and Ni-decorated Rh(553) surfaces. The theoretical results were compared to high-resolution X-ray photoelectron spectroscopy (HR-XPS) experiments. The most favorable CO adsorption sites for low coverages were identified by a systematic calculation o

Atmospheric dispersion represents a relatively overlooked problem in connection with the ultimate quality of ELT images corrected by adaptive optics (AO). The aim of this paper is to evaluate the contribution from atmospheric dispersion to the background level of the point-spread function (PSF). Since proper suppression of this level is important for the prospects for direct exo-planet observation

Eosinophil degranulation is a characteristic feature of asthma and allergic rhinitis. However, degranulated eosinophils have not been convincingly demonstrated in the common mouse models of these airway diseases. This study uses eosinophil peroxidase (EPO) histochemistry and transmission electron microscopy (TEM) analysis to assess eosinophil degranulation in the airways of ovalbumin (OVA)-sensiti

The Chemotaxis Inhibitory Protein of Staphylococcus aureus (CHIPS) binds and blocks the C5a receptor (C5aR) and formyl-peptide receptor (FPR). This way, CHIPS is a potent inhibitor of the immune cell recruitment associated with inflammation. Truncation of the protein and the introduction of mutations, shifts the expression towards the insoluble fraction of Escherichia coli, whereas the wild-type p

Temperate and boreal forests in the Northern Hemisphere cover an area of about 2 times 107 square kilometres and act as a substantial carbon sink (0.6–0.7 petagrams of carbon per year)1. Although forest expansion following agricultural abandonment is certainly responsible for an important fraction of this carbon sink activity, the additional effects on the carbon balance of established forests of

We used ultrasonic imaging to monitor short-term changes in the pectoral muscle size of captive red knots Calidris canutus. Pectoral muscle thickness changed rapidly and consistently in parallel with body mass changes caused by flight, fasting and fuelling. Four knots flew repeatedly for 10 h periods in a wind tunnel. Over this period, pectoral muscle thickness decreased in parallel with the decre

Linear pentadecamer and cyclic hexamer peptide phage libraries were used to isolate phage clones with binding affinity toward lactoferrins purified from human and bovine milk. Phage clones with high specificity toward lactoferrin were selected with different binding strengths depending on the sequence of the peptide displayed by the phage. Phages coated to a microtiterplate were able to capture la

Climate change is expected to have profound ecological effects, yet shifts in competitive abilities among species are rarely studied in this context. Blue tits (Cyanistes caeruleus) and great tits (Parus major) compete for food and roosting sites, yet coexist across much of their range. Climate change might thus change the competitive relationships and coexistence between these two species. Analys

In this work hollow fibre liquid phase microextraction combined with liquid chromatography mass spectrometry was applied for the determination of the nonsteroidal anti-inflammatory drugs (NSAIDs) ketoprofen, naproxen, diclofenac and ibuprofen as well as eight of their known human metabolites in wastewater samples. Extraction time and addition of tri-n-octylphosphine oxide (TOPO) to the liquid memb

This qualitative case study explores the empowering potentialities of clitoral reconstruction for mutilated women in Burkina Faso. In this developing country of sub-Saharan Africa, historical high prevalence rates of excision leave millions of women in the most physical and mental ill being. This, as a consequence, causes their marginalisation from the development process. The methodology is bond

The aim of this study was to understand the experiences that social workers in the social services child protection unit has regarding their working conditions and how they reflect about their workload, staff turnovers, wellbeing in regards to the role of a social worker. Our qualitative study was based on seven interviews with social workers who worked in a middle-sized municipality in Skåne. Th