Full scale, microscopically resolved tomographies of sandstone and carbonate rocks augmented by experimental porosity and permeability values.

We report a dataset containing full-scale, 3D images of rock plugs augmented by petrophysical lab characterization data for application in digital rock and capillary network analysis. Specifically, we have acquired microscopically resolved tomography datasets of 18 cylindrical sandstone and carbonate rock samples having lengths of 25.4 mm and diameters of 9.5 mm. Based on the micro-tomography data, we have computed porosity-values for each imaged rock sample. For validating the computed porosity values with a complementary lab method, we have measured porosity for each rock sample by using standard petrophysical characterization techniques. Overall, the tomography-based porosity values agree with the measurement results obtained from the lab, with values ranging from 8% to 30%. In addition, we provide for each rock sample the experimental permeabilities, with values ranging from 0.4 mD to above 5D. This dataset will be essential for establishing, benchmarking, and referencing the relation between porosity and permeability of reservoir rock at pore scale.

Early disruption of photoreceptor cell architecture and loss of vision in a humanized pig model of usher syndromes.

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.