Spinal muscular atrophy (SMA) is a leading genetic cause of infant mortality. The advent of approved treatments for this devastating condition has significantly changed SMA patients' life expectancy ...and quality of life. Nevertheless, these are not without limitations, and research efforts are underway to develop new approaches for improved and long-lasting benefits for patients. Protein arginine methyltransferases (PRMTs) are emerging as druggable epigenetic targets, with several small-molecule PRMT inhibitors already in clinical trials. From a screen of epigenetic molecules, we have identified MS023, a potent and selective type I PRMT inhibitor able to promote SMN2 exon 7 inclusion in preclinical SMA models. Treatment of SMA mice with MS023 results in amelioration of the disease phenotype, with strong synergistic amplification of the positive effect when delivered in combination with the antisense oligonucleotide nusinersen. Moreover, transcriptomic analysis revealed that MS023 treatment has minimal off-target effects, and the added benefit is mainly due to targeting neuroinflammation. Our study warrants further clinical investigation of PRMT inhibition both as a stand-alone and add-on therapy for SMA.
This paper analyzes the audience’s reception of Colonia, a 2016 film by Florian Gallenberger. As a popular work, Colonia is a docudrama film that presents historical events happened in Chile during ...the coup of General Augusto Pinochet in 1973. The problems analyzed in this article are the reception process of respondents and the quality of Colonia as popular films. The film is chosen for the study as it received five nominations from German Film Awards 2016 and won Bavarian Film Awards 2016 for Best Production category, but it only has a rating of 26% from 47 reviews by Rotten Tomatoes. The respondents involved in this study are members of Kronik Filmedia of Diponegoro University. Kronik Filmedia is the university film club that focuses on producing short films and appreciating films by conducting film discussions. The purpose of the study is to describe the reception processes of respondents who are affected by their horizon of expectations and to prove that not all of popular works are low literature. The theory used in this paper is the Aesthetic of Reception theory by Hans Robert Jauss. The main concept of this theory is horizon of expectations. Horizon of expectations is a reader’s preferences from the previous experiences or readings. For the methodology, this paper uses qualitative research and focus group discussion as the data collecting method. Focus group discussion is a gathering data method by means of interviewing respondents in a forum consisted of five to eight people. Focus group discussion is an effective method to observe a social phenomenon or a case study. The data obtained is then analyzed as texts to draw a conclusion. The research involves eight members of Kronik Filmedia as respondents. The results of the research show the reception of Kronik Filmedia as Colonia can satisfy and even surpass seven out of eight respondents’ horizon of expectations as the film goes to be more ‘interesting’ and ‘thrilling’. All respondents also view that Colonia is worth to be studied and discussed as those of high literature.
The organic electrochemical transistor (OECT), capable of transducing small ionic fluxes into electronic signals in an aqueous environment, is an ideal device to utilize in bioelectronic ...applications. Currently, most OECTs are fabricated with commercially available conducting poly(3,4-ethylenedioxythiophene) (PEDOT)-based suspensions and are therefore operated in depletion mode. Here, we present a series of semiconducting polymers designed to elucidate important structure–property guidelines required for accumulation mode OECT operation. We discuss key aspects relating to OECT performance such as ion and hole transport, electrochromic properties, operational voltage, and stability. The demonstration of our molecular design strategy is the fabrication of accumulation mode OECTs that clearly outperform state-of-the-art PEDOT-based devices, and show stability under aqueous operation without the need for formulation additives and cross-linkers.
Organic electrochemical transistors (OECTs) are receiving significant attention due to their ability to efficiently transduce biological signals. A major limitation of this technology is that only ...p-type materials have been reported, which precludes the development of complementary circuits, and limits sensor technologies. Here, we report the first ever n-type OECT, with relatively balanced ambipolar charge transport characteristics based on a polymer that supports both hole and electron transport along its backbone when doped through an aqueous electrolyte and in the presence of oxygen. This new semiconducting polymer is designed specifically to facilitate ion transport and promote electrochemical doping. Stability measurements in water show no degradation when tested for 2 h under continuous cycling. This demonstration opens the possibility to develop complementary circuits based on OECTs and to improve the sophistication of bioelectronic devices.
•Evaluation of module power with full-cell and half-cells under inhomogeneous soiling.•Influence of mounting orientation on module performance under inhomogeneous soiling.•Optimization of PV module ...performance under inhomogeneous soiling conditions.•Up to 65% higher power generation for the optimized design and mounting orientation.
Photovoltaic modules in desert areas benefit from high irradiation levels but suffer, among other parameters, from high soiling ratios, which influence the output power of the modules. Power loss due to soiling can reach more than 20% per month if not cleaned. Besides, inhomogeneous soiling by accumulation of dust on the corners and bottom of the modules may lead to total power loss of the modules. Modification in cell sizes, electrical interconnection, and mounting orientation of the modules can reduce the losses from inhomogeneous soiling. In this paper, we evaluate the performance of three module designs with full- and half-sized solar cells with different interconnection designs for different inhomogeneous soiling conditions. The modules are investigated in two mounting orientation of portrait and landscape installation with two scenarios of corner and edge soiling. The simulation results supported by the experimental data indicate that the PV modules in favorable mounting positions show up to 65% higher output power depending on the module design and soiling patterns. It is shown that our proposed optimized design mounted in favorable orientation is less sensitive to the inhomogeneous soiling and shows less current losses compared to the nominal operating current. Consequently, the module shows less current mismatch with the other modules in an array.
MAX-phase has excellent high-temperature lubrication performance, which has the potential to be used as the abradable seal coating in aircraft engine combustion chamber. Various concentrations of ...MAX-phase Ti3AlC2 (2, 5 and 10 wt %) are doped in YSZ-based abradable seal coating as the lubricating phase, and the coating produced by air plasma spraying (APS) on Ni-based alloy. The microstructure and element distribution of the seal coating are studied by field emission scanning electron microscopy (FE-SEM), electron X-ray probe microanalysis (EPMA). The mechanical properties are measured by Vickers hardness test, nanoindentation test and tribological test. The results reveal that with the content of the MAX-phase Ti3AlC2 increasing, the hardness of the abradable seal coating is increased and the resultant wear rate is reduced. The reduction of wear rate is due to the production of some tiny TiC and Al2O3 phases during deposition of Ti3AlC2 in plasma spraying. Comparing with the different MAX-phase content, the wear rate of the composite coating is different. The changes of the nanohardness and wear mechanism of the composite coating after plasma spraying will be discussed.
In bulk-heterojunction (BHJ) organic photovoltaics (OPVs), non-fullerene acceptors (NFAs) have lately surpassed their fullerene counterparts in photovoltaic performance. This progress in NFA OPVs may ...encourage the exploration of varied OPV device architectures, either deviating from or expanding upon the fundamental BHJ structure. This study employs numerical simulations on PBDB-T:NCBDT OPVs using graphene oxide (GO) as the hole transport layer (HTL) to examine the influence of thickness, defect density, and interface defects on device performance. Following optimization, the device exhibits short-circuit current (
J
SC
) of 27.92 mA cm
−2
, open-circuit voltage (
V
OC
) of 1.08 V, fill factor (FF) of 72.57%, and power conversion efficiency (PCE) of 21.84%. These findings support the further development of NFA-OPVs employing GO as the HTL.
The Al
2
O
3
forming MAX-phase Ti
2
AlC has potential to be used in the high-temperature combustion chambers of aircraft engines. In this work, Ti
2
AlC powder was compressed and synthesized by spark ...plasma sintering (SPS) at 1200°C and 30 MPa for different periods of time. The surface morphology, cross-section microstructure, mapping elemental distribution analysis, phase transition analysis, and mechanical properties were analyzed using a scanning electron microscope (SEM), energy dispersive spectroscopy (EDS), X-ray diffraction (XRD), and nanoindentation techniques. The results revealed that between 24 h and 100 h, Ti
2
AlC structure was primarily composed of rutile TiO
2,
with a small amount of α-Al
2
O
3
and numerous planar defects. When the oxidation time was increased, some additional oxides such as rutile-TiO
2
, α-Al
2
O
3
, TiO
2
-Al
2
TiO
5
, and TiO
2
-α-Al
2
O
3
were also formed. Although the planar defects were reduced at 300 h due to the inward diffusion of O
2−
and outward diffusion of Al
3+
and Ti
4+
, they spread throughout the entire structure rather than forming layers, leading to a significant loss in oxidation resistance.
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