Usher Syndrome in the Inner Ear: Etiologies and Advances in Gene Therapy

Hearing loss is the most common sensory disorder with ~466 million people worldwide affected, representing about 5% of the population. A substantial portion of hearing loss is genetic. Hearing loss can either be non-syndromic, if hearing loss is the only clinical manifestation, or syndromic, if the hearing loss is accompanied by a collage of other clinical manifestations. Usher syndrome is a syndromic form of genetic hearing loss that is accompanied by impaired vision associated with retinitis pigmentosa and, in many cases, vestibular dysfunction. It is the most common cause of deaf-blindness. Currently cochlear implantation or hearing aids are the only treatments for Usher-related hearing loss. However, gene therapy has shown promise in treating Usher-related retinitis pigmentosa. Here we review how the etiologies of Usher-related hearing loss make it a good candidate for gene therapy and discuss how various forms of gene therapy could be applied to Usher-related hearing loss.     

A differential inclusion approach for modeling and analysis of dynamical systems under uncertainty

In this paper we deal with the application of differential inclusions to modeling nonlinear dynamical systems under uncertainty in parameters. In this case, differential inclusions seem to be better suited to modeling practical situations under uncertainty and imprecision than formulations by means of fuzzy differential equations. We develop a practical algorithm to approximate the reachable sets of a class of nonlinear differential inclusion, which eludes the computational problems of a previous set-valued version of the Heun’s method. Our algorithm is based on a complete discretization (time and state space) of the differential inclusion and it suits hardware features, handling the memory used by the method in a controlled fashion during all iterations. As a case of study, we formulate a differential inclusion to model an epidemic outbreak of dengue fever under Cuban conditions. The model takes into account interaction of human and mosquito populations as well as vertical transmission in the mosquito population. It is studied from the theoretical point of view to apply the Practical Algorithm. Also, we estimate the temporal evolution of the different human and mosquito populations given by the model in the Dengue 3 epidemic in Havana 2001, through the computation of the reachable sets using the Practical Algorithm.     

Thin-film iron-oxide nanobeads as bifunctional electrocatalyst for high activity overall water splitting

Developing only Fe derived bifunctional overall water splitting electrocatalyst both for oxygen evolution reaction (OER) and hydrogen evolution reaction (HER) while performing at low onset overpotential and with high catalytic stability is a rare instance. We present here the first demonstration of unique iron-oxide nanobeads (FeO_x-NBs) based electrocatalyst executing both OER and HER with high activity. Thin-film electrocatalytic FeO_x-NBs assembly is surface grown via simple spray coating (SC). The unique SC/FeO_x-NBs propels OER initiating water oxidation just at 1.49 V_RHE (η = 260 mV) that is the lowest observable onset potential for OER on simple Fe-oxide based catalytic films reported so far. Catalyst also reveals decently high HER activity and competent overall water splitting performance in the FeO_x-NBs two-electrode system as well. Catalyst also presents stable kinetics, with promising high electrochemically active surface area (ECSA) of 1765 cm², notable Tafel slopes of just 54 mV dec^1? (OER) and 85 mV dec^1? (HER), high exchange current density of 1.10 mA cm^2? (OER), 0.58 mA cm^2? (HER) and TOF of 74.29s^1?@1.58V_RHE, 262s^1?@1.62V_RHE (OER) and 82.5s^1?@-0.45V_RHE, 681s^1?@-0.56V_RHE (HER).     

Surface-assembled Fe-Oxide colloidal nanoparticles for high performance electrocatalytic water oxidation

Nanoscale electrocatalytic materials having enhanced electroactive sites has been considered trendier and can drive kinetically uphill OER at much lower energy cost with high efficiency. However, very complex synthetic strategies, extensive functionalization processes, and less stability have stimulated quest for economically viable, straightforward and facile preparative methods for designing stable, robust and active nanoscale electrocatalysts engaging geologically abundant materials to ensure their industrial implications. Here we present surface-assembled Fe(OH)_x/FeO_x type colloidal catalytic thin-films, with or without post annealing, derived from Fe-colloidal NPs in simple carbonate system for efficient water oxidation. Comprehensive electrochemical studies including cyclic voltammetry, chronoamperometry, chronopotentiometry, impedance spectroscopy, Tafel slope analysis, mass activity, electrochemically active surface area measurements are conducted to comparatively evaluate the performance of simple (FeO_x/HCO₃^?@FTO and annealed (FeO_x/HCO₃^?@FTO₂₅₀, FeO_x/HCO₃^?@FTO₅₀₀) catalysts for oxygen evolution reaction (OER) under employed conditions. The FeO_x/HCO₃^?@FTO₂₅₀ annealed at 250 °C initiates water oxidation at much lower overpotential of 1.52 V vs. RHE with remarkable stability during long-term electrochemical experimentations. In addition to enhanced OER activity as evidence by better onset potential (<1.55 V vs. RHE), lower Tafel slope value (36 mV dec^1?) and negligible charge transfer resistance, the Fe(OH)_x/HCO₃^?@FTO type catalyst presented excellent electroactive nature during long term controlled potential electrolysis experiments where more and more electroactive sites were getting exposed during continuous hours of electrolysis. The catalysts behave as a potential enduring, inexpensive and competent candidate for catalyzing water oxidation reaction when tested under begin conditions.     

Monte Carlo uncertainty analysis of an ANN-based spectral analysis method

Neural Computing and Applications - This work presents the uncertainty analysis of an artificial neural network (ANN)-based method, called multiharmonic ANN fitting method (MANNFM), which is able...     

Effects of metal doping agent on the properties of Nb2−xMxO5 (M = Mn,Fe, and Ni) system

In order to improve the behavior Niobium pentoxide on oxidation‐reductionreactions to degrade organic compounds in aqueous solutions, we developed the synthesis of Niobium pentoxide with the metal doping agents: manganese,iron, and nickel (Nb_2?xM_xO₅), respectively, and analyzing its effects on the structural and vibrational properties. The synthesis of the Nb_2?xM_xO₅ (M = Mn, Fe, and Ni; x = 0.02 and 0.05) was performed according the Pechini method, but before the annealing, the samples were submitted to Thermogravimetric analysis study showing better pre‐calcination annealing temperatures at 500 and 700°C for the materials. Then, we performed the crystal structural analysis by X‐ray Diffraction, obtaining crystallite sizes between 15 and 47 nm, the accurate size analysis was calculated by Scherrer equation. Structural analysis was also performed by IR spectroscopy to evaluate the vibrational modes of synthetized samples. The dopping effects were evaluated by UV‐Vis spectrophotometry through the energy band gap values, showing that doped samples with manganese and iron had lower values than undoped, except the sample doped with nickel. However, samples doped with iron at 2.5% showed better photocatalytic performance.     

Ginzburg–Landau Simulation of Superconducting Matter in a Semicircular Film

We numerically investigate the superconductor matter of a film type-II superconductor with circular geometry numerically in a computational simulation using the link variable method to solve the time dependent Ginzburg–Landau equation. We apply the implemented algorithm to a half circular film surrounded by a thin layer of another superconductor at a higher critical temperature. The film is immersed in an external magnetic field applied perpendicularly to its plane. The boundary condition is taken into account with the de Gennes extrapolation length b. We calculate the magnetization, vortex number, the third critical thermodynamical field as a function of the external magnetic field, and the b parameter. We find that for these interfaces the third critical field $H_{\mathrm{c}_{3}}$ and the magnetization M are largely increased.