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.

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Within the scope of the project ENERTHERM: Energy efficiency of thermal processes which is funded by the Bavarian Ministry of Economy (BMWi) components and systems for high-temperature are being developed. Monolithic oxide ceramics (mullite, Al2O3) and oxide-fiber reinforced ceramics (O-CMC) show a high potential for high temperature (HT) applications. Additionally, the demand for complex HT-components such as HT-fanwheels for kilns, supporting HT-lightweight structures or hot gas liners increases. In according to the required component design, joining techniques are needed in order to realize such complex geometries. The generated joints were made by using commercial glass solder from the type Al2O3-SiO2-MgO. For realizing the joining process sintering-joining in HT-kilns was used because of the homogeneous temperature distribution. A CO2 laser (wavelength of 10.6 microns and a power of 1.5 kW) was used for rapid joining process. The mechanical properties were determined in according to DIN EN 843-1 and DIN EN 658-3 (4-point bending strength) and evaluated according to Weibull distribution.

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