Autosomal Dominant Gyrate Atrophy-Like Choroidal Dystrophy Revisited: 45 Years Follow-Up and Association with a Novel C1QTNF5 Missense Variant

We present a long-term follow-up in autosomal dominant gyrate atrophy-like choroidal dystrophy (adGALCD) and propose a possible genotype/phenotype correlation. Ophthalmic examination of six patients from two families revealed confluent areas of choroidal atrophy resembling gyrate atrophy, starting in the second decade of life. Progression continued centrally, reaching the fovea at about 60 years of age. Subretinal deposits, retinal pigmentation or choroidal neovascularization as seen in late-onset retinal degeneration (LORD) were not observed. Whole genome sequencing revealed a novel missense variant in the C1QTNF5 gene (p.(Q180E)) which was found in heterozygous state in all affected subjects. Haplotype analysis showed that this variant found in both families is identical by descent. Three-dimensional modeling of the possible supramolecular assemblies of C1QTNF5 revealed that the p.(Q180E) variant led to the destabilization of protein tertiary and quaternary structures, affecting both the stability of the single protomer and the entire globular head, thus exerting detrimental effects on the formation of C1QTNF5 trimeric globular domains and their interaction. In conclusion, we propose that the p.(Q180E) variant causes a specific phenotype, adGALCD, that differs in multiple clinical aspects from LORD. Disruption of optimal cell-adhesion mechanisms is expected when analyzing the effects of the point mutation at the protein level.     

Progress in emitter wrap-through solar cell fabrication on boron doped Czochralski-grown silicon

We report on RISE-EWT (Rear Interdigitated Single Evaporation-Emitter Wrap-Through) solar cells on full area (12.5×12.5 cm²) pseudo square boron doped Czochralski-grown silicon wafers. We investigate the main efficiency optimisation factors of these cells by investigating the dependence of RISE-EWT cell parameters on the base dopant concentration N_A. We furthermore detail the effects of large feature sizes in base and emitter regions at the rear of the solar cell and investigate these effects with particular attention to the edge regions. EWT solar cells typically exhibit rather low fill factors. However, our results show that the improved fill factors can be achieved by increasing N_A, which in return leads to optimised efficiency values. For our RISE-EWT solar cells made from boron doped Cz-Si wafers, this benefit is maintained even after light-induced degradation. Our investigation of edge area related effects shows the importance of proper cell design in these areas, leading to a further 2.8% absolute improvement in the fill factor. Combining increased base dopant concentration with optimised edge design, we achieve 19.0% efficiency on (12.5×12.5 cm²) boron doped Cz silicon wafers before light-induced degradation, resulting in 18.1% efficiency in the light-degraded state.     

Analysis of Human TAAR8 and Murine Taar8b Mediated Signaling Pathways and Expression Profile

The thyroid hormone derivative 3-iodothyronamine (3-T₁AM) exerts metabolic effects in vivo that contradict known effects of thyroid hormones. 3-T₁AM acts as a trace amine-associated receptor 1 (TAAR1) agonist and activates G_s signaling in vitro. Interestingly, 3-T₁AM-meditated in vivo effects persist in Taar1 knockout-mice indicating that further targets of 3-T₁AM might exist. Here, we investigated another member of the TAAR family, the only scarcely studied mouse and human trace-amine-associated receptor 8 (Taar8b, TAAR8). By RT-qPCR and locked-nucleic-acid (LNA) in situ hybridization, Taar8b expression in different mouse tissues was analyzed. Functionally, we characterized TAAR8 and Taar8b with regard to cell surface expression and signaling via different G-protein-mediated pathways. Cell surface expression was verified by ELISA, and cAMP accumulation was quantified by AlphaScreen for detection of G_s and/or G_i/o signaling. Activation of G-proteins G_q/11 and G_12/13 was analyzed by reporter gene assays. Expression analyses revealed at most marginal Taar8b expression and no gender differences for almost all analyzed tissues. In heart, LNA-in situ hybridization demonstrated the absence of Taar8b expression. We could not identify 3-T₁AM as a ligand for TAAR8 and Taar8b, but both receptors were characterized by a basal G_i/o signaling activity, a so far unknown signaling pathway for TAARs.     

Removal of Hexavalent Chromium by Different Modified Spruce Bark Adsorbents

In this work, spruce bark was used as a raw material to remove Cr(VI) ions from aqueous solutions. Three kinds of chemically modified bark adsorbents were prepared by treatment with formaldehyde (FB), dilute sulfuric acid (AB), and concentrated sulfuric acid (CB), respectively. The chemical modifications mainly changed the relative lignin content in the bark. Lower pH facilitated the adsorption of Cr(VI) ions because reduction of Cr(VI) ions to Cr(III) ions occurred during the adsorption process which consumed a large amount of H⁺ ions. Higher temperature accelerated the adsorption process, owing to the endothermic nature of the redox reaction. At initial solution pH around 1, the adsorption capacities of Cr(VI) ions on FB, AB, and CB were as high as 423, 503, and 759 mg/g, respectively, which were much higher than the reported adsorption capacities by other agricultural and forest biosorbents in the literatures. XPS analysis revealed the adsorption mechanism was adsorption-coupled reduction involving the electron-donor groups of lignin moieties.     

Helminth eggs inactivation efficiency by faecal sludge dewatering and co-composting in tropical climates

This study investigates helminth eggs removal and inactivation efficiency in a treatment process combining faecal sludge (FS) dewatering and subsequent co-composting with organic solid waste as a function of windrow turning frequency. Fresh public toilet sludge and septage mixed at a 1:2 ratio were dewatered on a drying bed. Biosolids with initial loads of 25-83 helminth eggs/g total solids (TS) were mixed with solid waste as bulking material for co-composting at a 1:2 volume ratio. Two replicate sets of compost heaps were mounted in parallel and turned at different frequencies during the active composting period: (i) once every 3 days and (ii) once every 10 days. Turning frequency had no effect on helminth eggs removal efficiency. In both setups, helminth eggs were reduced to <1 viable egg/g TS, thereby complying with the WHO guidelines 2006 for the safe reuse of FS.     

Control of Cell Adhesion and Neurite Outgrowth by Patterned Gold Nanoparticles with Tunable Attractive or Repulsive Surface Properties

Guiding of neuronal cells on surfaces is required for the investigation of fundamental aspects of neurobiology, for tissue engineering, and for numerous bioelectronic applications. A modular method to establish nanostructured chemical templates for local deposition of gold nanoparticles is presented. A process comprising nanoimprint lithography, silanization, lift‐off, and gold nanoparticle immobilization is used to fabricate the particle patterns. The chemical composition of the surface can be modified by in situ adsorption of cell‐binding ligands to locally addressed particles. The versatility of this approach is demonstrated by inverting the binding affinity between rat cortical neurons and nanopatterned surfaces via wet‐chemical means and thereby reversing the pattern of guided neurons.     

Catalytic asymmetric hydrogenation in micellar media with amphiphilic and non-amphiphilic ligands

The transfer of a homogeneous catalytic hydrogenation catalyst into an aqueous micellar system was investigated. In case of the asymmetric hydrogenation activity and enantioselectivity were enhanced in water due to the addition of surfactants. A variation of the microheterogenization in water was realized with new amphiphilic ligands derived from Brij (polyoxyethylene ethers) and PPM (4-diphenylphosphino-2-diphenylphosphinomethyl-pyrrolidine). The substrate could be solubilized in the micellar assemblies. Best results were observed in mixed micelles: the enantioselectivity achieved 96% ee. New practical aspects are given by use of polymerized surfactants which we prepared by polymerization of assemblies.     

Signal Transduction Pathways as Concurrent Reactive Systems: A Modeling and Simulation Approach Using LSCs and the Play-Engine

Signal transduction pathways describe how cells respond to extracellular signals which are received by receptors at the cell membrane and usually transferred into the nucleus. In this paper we present our approach to model these signal transduction pathways as concurrent reactive systems by means of Life Sequence Charts and to simulate them using the Play-Engine tool. This aspect is part of a bigger approach, where we provide an extendable system to generate models of signal transduction pathways in different modeling languages and to simulate these models with the corresponding simulation tools.     

Grenzenloser Mobile Commerce

Eine wichtige S?ule des Datenschutzrechts sind die Rechte der Betroffenen insbesondere auf Benachrichtigung und Auskunft sowie auf Berichtigung, L?schung und Sperrung personenbezogener Daten. Diese Rechte k?nnen nur dann einen effektiven Schutz der informationellen Selbstbestimmung bewirken, wenn sie gesetzlich normiert sind und gleichzeitig ihre gerichtliche Durchsetzbarkeit gew?hrleistet ist. Der Mobile Commerce überwindet staatliche Grenzen und führt dazu, dass der Umgang mit personenbezogenen Daten zunehmend durch verantwortliche Stellen erfolgt, die ihren Sitz nicht in Deutschland, sondern im Ausland haben. Der Aufsatz zeigt die sich aus dem Internationalen Prozess- und Privatrecht für den Betroffenen ergebenden prozess- und materiell-rechtlichen Konsequenzen auf, wenn die Datenverarbeitung im Ausland erfolgt.