Prader-Willi syndrome (PWS) is a neurogenetic multifactorial disorder caused by the deletion or inactivation of paternally imprinted genes on human chromosome 15q11-q13. The affected homologous locus is on mouse chromosome 7C. The positional conservation and organization of genes including the imprinting pattern between mice and men implies similar physiological functions of this locus. Therefore, considerable efforts to recreate the pathogenesis of PWS have been accomplished in mouse models. We provide a summary of different mouse models that were generated for the analysis of PWS and discuss their impact on our current understanding of corresponding genes, their putative functions and the pathogenesis of PWS. Murine models of PWS unveiled the contribution of each affected gene to this multi-facetted disease, and also enabled the establishment of the minimal critical genomic region (PWScr) responsible for core symptoms, highlighting the importance of non-protein coding genes in the PWS locus. Although the underlying disease-causing mechanisms of PWS remain widely unresolved and existing mouse models do not fully capture the entire spectrum of the human PWS disorder, continuous improvements of genetically engineered mouse models have proven to be very powerful and valuable tools in PWS research. 相似文献
First examples of multichain (polycatenar) compounds, based on the π-conjugated [1]benzothieno[3,2-b]benzothiophene unit are designed, synthesized, and their soft self-assembly and charge carrier mobility are investigated. These compounds, terminated by the new fan-shaped 2-brominated 3,4,5-trialkoxybenzoate moiety, form bicontinuous cubic liquid crystalline (LC) phases with helical network structure over extremely wide temperature ranges (>200 K), including ambient temperature. Compounds with short chains show an achiral cubic phase with the double network, which upon increasing the chain length, is at first replaced by a tetragonal 3D phase and then by a mirror symmetry is broken triple network cubic phase. In the networks, the capability of bypassing defects provides enhanced charge carrier mobility compared to imperfectly aligned columnar phases, and the charge transportation is non-dispersive, as only rarely observed for LC materials. At the transition to a semicrystalline helical network phase, the conductivity is further enhanced by almost one order of magnitude. In addition, a mirror symmetry broken isotropic liquid phase is formed beside the 3D phases, which upon chain elongation is removed and replaced by a hexagonal columnar LC phase. 相似文献
European Journal of Wood and Wood Products - Medium density fibreboards (MDF) are currently not recycled after service life, but various publications report on recycling by the disintegration of... 相似文献
All-solid-state batteries constitute a very promising energy storage device. Two very important properties of these battery cells are the ionic and the electrical conductivity, which describe the ion and the electron transport through the electrodes, respectively. In this work, a numerical method is presented to model the electrical conductivity, considering the outcome of discrete-element method simulations and the intrinsic conductivities of both the active material particles and the conductive additive particles. The results are calibrated and validated with the help of experimental data of real manufactured electrodes. The tortuosity, which strongly influences the ionic conductivity, is also presented for the analyzed electrodes, taking their microstructure into account. 相似文献
Programming and Computer Software - Recently, it has been shown constructively how a finite set of hypergeometric products, multibasic hypergeometric products or their mixed versions can be modeled... 相似文献
The article discusses future trends and their implications on separation technologies starting from the main drivers of technological developments. Especially the consequences of the expected energy and raw material change and the increasing international economic competition on process technologies are addressed. Special attention is given to the opportunities of increasing computational power and the availability of high-performance networks for scientific modeling and their use in process industries. 相似文献
Loop acceleration can be used to prove safety, reachability, runtime bounds, and (non-)termination of programs. To this end, a variety of acceleration techniques have been proposed. However, so far all of them have been monolithic, i.e., a single loop could not be accelerated using a combination of several different acceleration techniques. In contrast, we present a calculus that allows for combining acceleration techniques in a modular way and we show how to integrate many existing acceleration techniques into our calculus. Moreover, we propose two novel acceleration techniques that can be incorporated into our calculus seamlessly. Some of these acceleration techniques apply only to non-terminating loops. Thus, combining them with our novel calculus results in a new, modular approach for proving non-termination. An empirical evaluation demonstrates the applicability of our approach, both for loop acceleration and for proving non-termination.