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Fano resonances in nanostructures have attracted widespread research interests in the past few years for their potential applications in sensing, switching and nonlinear optics. In this paper, a mid-infrared Fano resonance in a hybrid metal-graphene metamaterial is studied. The hybrid metamaterial consists of a metallic grid enclosing with graphene nanodisks. The Fano resonance arises from the coupling of graphene and metallic plasmonic resonances and it is sharper than plasmonic resonances in pure graphene nanostructures. The resonance strength can be enhanced by increasing the number of graphene layers. The proposed metamaterial can be employed as a high-performance mid-infrared plasmonic sensor with an unprecedented sensitivity of about 7.93 μ m/RIU and figure of merit (FOM) of about 158 . 7 .Congenital deficiency of the lysosomal sialidase neuraminidase 1 (NEU1) causes the lysosomal storage disease, sialidosis, characterized by impaired processing/degradation of sialo-glycoproteins and sialo-oligosaccharides, and accumulation of sialylated metabolites in tissues and body fluids. Sialidosis is considered an ultra-rare clinical condition and falls into the category of the so-called orphan diseases, for which no therapy is currently available. In this study we aimed to identify potential therapeutic modalities, targeting primarily patients affected by type I sialidosis, the attenuated form of the disease. We tested the beneficial effects of a recombinant protective protein/cathepsin A (PPCA), the natural chaperone of NEU1, as well as pharmacological and dietary compounds on the residual activity of mutant NEU1 in a cohort of patients' primary fibroblasts. We observed a small, but consistent increase in NEU1 activity, following administration of all therapeutic agents in most of the fibroblasts tested. Interestingly, dietary supplementation of betaine, a natural amino acid derivative, in mouse models with residual NEU1 activity mimicking type I sialidosis, increased the levels of mutant NEU1 and resolved the oligosacchariduria. Overall these findings suggest that carefully balanced, unconventional dietary compounds in combination with conventional therapeutic approaches may prove to be beneficial for the treatment of sialidosis type I.Low-cost sensors can be used to improve the temporal and spatial resolution of an individual's particulate matter (PM) intake dose assessment. In this work, personal activity monitors were used to measure heart rate (proxy for minute ventilation), and low-cost PM sensors were used to measure concentrations of PM. Intake dose was assessed as a product of PM concentration and minute ventilation, using four models with increasing complexity. The two models that use heart rate as a variable had the most consistent results and showed a good response to variations in PM concentrations and heart rate. On the other hand, the two models using generalized population data of minute ventilation expectably yielded more coarse information on the intake dose. Aggregated weekly intake doses did not vary significantly between the models (6-22%). Propagation of uncertainty was assessed for each model, however, differences in their underlying assumptions made them incomparable. The most complex minute ventilation model, with heart rate as a variable, has shown slightly lower uncertainty than the model using fewer variables. Similarly, among the non-heart rate models, the one using real-time activity data has less uncertainty. Minute ventilation models contribute the most to the overall intake dose model uncertainty, followed closely by the low-cost personal activity monitors. The lack of a common methodology to assess the intake dose and quantifying related uncertainties is evident and should be a subject of further research.Despite intensive efforts, the fluorescence of perovskite nanocrystals (NCs) still suffers from a poor color purity, which limits the applications in light emitting and multicolor display. A deep understanding on the fundamental of the photoluminescent (PL) spectral broadening is thus of great significance. Herein, the PL decay curves of the CsPbClxBr3-x NCs are monitored at different wavelengths covering the entire PL band. Moreover, energy relaxation time τ and radiative recombination time β are obtained by numerical fittings. Selleckchem GSK467 The dependences of τ and 1/β on the detection wavelength agree well with the steady-state PL spectrum, indicating the observed PL broadening is an intrinsic effect due to the resonance and off-resonance exciton radiative recombination processes. This work not only provides a new analysis method for time-resolved PL spectra of perovskites, but also gains a deep insight into the spectral broadening of the lead halide perovskite NCs.Many inherited metabolic diseases (IMD) have cardiac manifestations. The aim of this study was to estimate the prevalence of IMD in adult patients with hypertrophic cardiomyopathy (HCM) and cardiac rhythm abnormalities that require cardiac implantable electronic devices (CIEDs). The study included a review of the medical files of patients aged 18 to 65 years who were followed in our cardiology department during the period 2010-2017. Metabolic explorations for Fabry disease (FD), mitochondrial cytopathies, and fatty-acid metabolism disorders were carried out in patients with unexplained etiology. The prevalence of IMD in patients with HCM was 5.6% (confidence interval (CI) 2.6-11.6). Six cases of IMD were identified 1 mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome, 1 Hurler syndrome, 2 Friedreich's ataxia, 1 FD, and 1 short-chain acyl-CoA dehydrogenase deficiency. Three cases of IMD were identified in patients requiring CIEDs 1 patient with Leber hereditary optic neuropathy, 1 FD, and 1 short chain acyl-CoA dehydrogenase (SCAD) deficiency. IMD prevalence in patients with CIEDs was 3.1% (CI 1.1-8.8). IMD evaluation should be performed in unexplained HCM and cardiac rhythm abnormalities adult patients, since the prevalence of IMD is relatively important and they could benefit from specific treatment and family diagnosis.
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