Usher syndrome (USH) is the most common form of monogenic deaf-blindness. Loss of vision is untreatable and there are no suitable animal models for testing therapeutic strategies of the ocular constituent of USH, so far. By introducing a human mutation into the harmonin-encoding USH1C gene in pigs, we generated the first translational animal model for USH type 1 with characteristic hearing defect, vestibular dysfunction, and visual impairment. Changes in photoreceptor architecture, quantitative motion analysis, and electroretinography were characteristics of the reduced retinal virtue in USH1C pigs. Fibroblasts from USH1C pigs or USH1C patients showed significantly elongated primary cilia, confirming USH as a true and general ciliopathy. Primary cells also proved their capacity for assessing the therapeutic potential of CRISPR/Cas-mediated gene repair or gene therapy in vitro. AAV-based delivery of harmonin into the eye of USH1C pigs indicated therapeutic efficacy in vivo.

Present investigation reports the synthesis of stable as well as visible-light active CdS/Ag 2 O nanocomposite photocatalysts in three varied ratios to be engaged in degradation of methylene blue (MB) dye. As-synthesized photocatalysts have been characterized with the aid of diverse characterization techniques such as X-ray diffraction (XRD), FT-Raman, Fourier transform infrared spectroscopy (FT-IR), X-ray photoelectron spectroscopy (XPS), UV-visible and Photoluminescence (PL) spectroscopy, and transmission electron microscopy (TEM). XRD pattern clearly depicts the presence of hexagonal CdS phase and cubic Ag 2 O phase. Results show a remarkable enhancement of photocatalytic activity for MB degradation especially for CdS/Ag 2 O nanocomposite with maximum efficiency up to 88.8 %. Moreover, the effective bandgap of CdS/ Ag 2 O (1 : 2) nanocomposite has been significantly reduced to 1.71 eV from pure CdS (2.15 eV). It can be derived that CdS/ Ag 2 O photocatalysis may be envisaged for treatment of diluted waste water containing organic pollutants. Figure 13. Reusability of CdS/Ag 2 O (1 : 2) nanocomposite for degradation of MB. ChemistrySelectFull Papers

Despite the prospects of intrinsically porous planar nanomaterials in separation applications, their synthesis on a large scale remains challenging. In particular, preparing water-selective carbon nanomembranes (CNMs) from selfassembled monolayers (SAMs) is limited by the cost of epitaxial metal substrates and molecular precursors with specific chemical functionalities. In this work, we present a facile fabrication of CNMs from polycyclic aromatic hydrocarbons (PAHs) that are drop-cast onto arbitrary supports, including foils and metalized films. The electron-induced carbonization is shown to result in continuous membranes of variable thickness, and the material is characterized with a number of spectroscopic and microscopic techniques. Permeation measurements with freestanding membranes reveal a high degree of porosity, but the selectivity is found to strongly depend on the thickness. While the permeance of helium remains almost the same for 6.5 and 3.0 nm thick CNMs, water permeance increases by 2 orders of magnitude. We rationalize the membrane performance with the help of kinetic modeling and vapor adsorption experiments.

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X-ray resonant magnetic reflectivity (XRMR) allows for the simultaneous measurement of structural, optical, and magneto-optic properties and depth profiles of a variety of thin film samples. However, a same-beamtime same-sample systematic quantitative comparison of the magnetic properties observed using XRMR and x-ray magnetic circular dichroism (XMCD) is still pending. Here, the XRMR results (Pt L 3 absorption edge) for the magnetic proximity effect in Pt deposited on the two different ferromagnetic materials Fe and Co 33 Fe 67 are compared with quantitatively analyzed XMCD results. The obtained results are in very good quantitative agreement between the absorptionbased (XMCD) and reflectivity-based (XRMR) techniques, taking into account an ab initio calculated magneto-optic conversion factor for the XRMR analysis. Thus, it is shown that XRMR provides quantitative reliable spin depth profiles important for spintronic and spin caloritronic transport phenomena at this type of magnetic interfaces.