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Hypomyelinating leukodystrophy (HLD) is a rare genetic heterogeneous disease that can affect myelin development in the central nervous system. This study aims to analyze the clinical phenotype and genetic function of a family with HLD-7 caused by POLR3A mutation. The proband (IV6) in this family mainly showed progressive cognitive decline, dentin dysplasia, and hypogonadotropic hypogonadism. Her three old brothers (IV1, IV2, and IV4) also had different degrees of ataxia, dystonia, or dysarthria besides the aforementioned manifestations. Their brain magnetic resonance imaging showed bilateral periventricular white matter atrophy, brain atrophy, and corpus callosum atrophy and thinning. The proband and her two living brothers (IV2 and IV4) were detected to carry a homozygous mutation of the POLR3A (NM_007055.4) gene c. 2300G > T (p.Cys767Phe), and her consanguineous married parents (III1 and III2) were p.Cys767Phe heterozygous carriers. In the constructed POLR3A wild-type and p.Cys767Phe mutant cells, it was seen that overexpression of wild-type POLR3A protein significantly enhanced Pol III transcription of 5S rRNA and tRNA Leu-CAA. However, although the mutant POLR3A protein overexpression was increased compared to the wild-type protein overexpression, it did not show the expected further enhancement of Pol III function. On the contrary, Pol III transcription function was frustrated (POLR3A, BC200, and tRNA Leu-CAA expression decreased), and MBP and 18S rRNA expressions were decreased. This study indicates that the POLR3A p.Cys767Phe variant caused increased expression of mutated POLR3A protein and abnormal expression of Pol III transcripts, and the mutant POLR3A protein function was abnormal. [ABSTRACT FROM AUTHOR]

C36H26N4O5Co, triclinic, P212121 (no. 19), a = 10.6380(5) Å, b = 10.6639(5) Å, c = 26.5443(15) Å, V = 3011.2(3) Å3, Z = 4, R gt(F) = 0.0330, wR ref(F 2) = 0.0681, T = 200 K.

Introduction: Traumatic brain injury (TBI) is a complex pathophysiological process, and increasing attention has been paid to the important role of post‐synaptic density (PSD) proteins, such as glutamate receptors. Our previous study showed that a PSD protein Arc/Arg3.1 (Arc) regulates endoplasmic reticulum (ER) stress and neuronal necroptosis in traumatic injury in vitro. Aim: In this study, we investigated the expression, regulation and biological function of Arc in both in vivo and in vitro experimental TBI models. Results: Traumatic neuronal injury (TNI) induced a temporal upregulation of Arc in cortical neurons, while TBI resulted in sustained increase in Arc expression up to 24 h in rats. The increased expression of Arc was mediated by the activity of metabotropic glutamate receptor 5 (mGluR5), but not dependent on the intracellular calcium (Ca2+) release. By using inhibitors and antagonists, we found that TNI regulates Arc expression via Gq protein and protein turnover. In addition, overexpression of Arc protects against TBI‐induced neuronal injury and motor dysfunction both in vivo and in vitro, whereas the long‐term cognitive function was not altered. To determine the role of Arc in mGluR5‐induced protection, lentivirus‐mediated short hairpin RNA (shRNA) transfection was performed to knockdown Arc expression. The mGluR5 agonist (RS)‐2‐chloro‐5‐hydroxyphenylglycine (CHPG)‐induced protection against TBI was partially prevented by Arc knockdown. Furthermore, the CHPG‐induced attenuation of Ca2+ influx after TNI was dependent on Arc activation and followed regulation of AMPAR subunits. The results of Co‐IP and Ca2+ imaging showed that the Arc‐Homer1 interaction contributes to the CHPG‐induced regulation of intracellular Ca2+ release. Conclusion: In summary, the present data indicate that the mGluR5‐mediated Arc activation is a protective mechanism that attenuates neurotoxicity following TBI through the regulation of intracellular Ca2+ hemostasis. The AMPAR‐associated Ca2+ influx and ER Ca2+ release induced by Homer1‐IP3R pathway might be involved in this protection. [ABSTRACT FROM AUTHOR]

Background: Colletotrichum species are among the most common pathogens in agriculture and forestry, and their control is urgently needed. Methods: In this study, a total of 68 strains of biocontrol bacteria were isolated and identified from Photinia × fraseri rhizosphere soil. Results: The isolates were identified as Brevibacillus brevis by 16S rRNA. The inhibitory effect of TR-4 on Colletotrichum was confirmed by an in vitro antagonistic experiment. The inhibitory effect of TR-4 was 98% at a concentration of 10 µl/ml bacterial solution, protection of the plant and inhibition of C. siamense was evident. Moreover, the secretion of cellulase and chitosan enzymes in the TR-4 fermentation liquid cultured for three days was 9.07 mol/L and 2.15 µl/mol, respectively. Scanning electron microscopy and transmission electron microscopy confirmed that TR-4 destroyed the cell wall of C. siamense, resulting in leakage of the cell contents, thus weakening the pathogenicity of the bacteria. [ABSTRACT FROM AUTHOR]

Ultrathin metal films emerge as an innovative category of transparent electrodes in recent decades, holding great promises enabling the next-generation flexible organic light-emitting devices (OLEDs). Although metal thin films with polymer nucleation inducers have been extensively studied in OLEDs, satisfying the requirements of both superior optoelectrical and high optical outcoupling characteristics is still challenging. Here, we demonstrate a metal oxide/ultrathin Ag/polymer (MAP) composite electrode with low sheet resistance of 15.1 Ω/sq, high transmittance of 87.4% at 550 nm, and smooth morphology with surface roughness of 0.768 nm. Besides, the composite electrodes significantly enhance the outcoupling of the light trapped in OLEDs due to the relatively high-refractive index polymer. Flexible OLEDs with the MAP anodes exhibit over 2.3 times enhancement in efficiency to that of indium tin oxide (ITO)-based OLEDs. The flexible OLEDs can survive 1000 bending cycles at a bending radius of 8 mm with negligible decrease in electroluminescent performance.

Solution-processing thin-film solar techniques, such as organic solar cells (OSCs) and perovskite solar cells (PeSCs), hold great promise as cost-effective renewable energy sources with feasible large-scale manufacturing. However, these devices are suffering from the incomplete photon absorption and thereby cannot unlock the full potential of device efficiency despite their rapid development in recent decades. Incorporation of plasmonic metal nanoparticles (NPs) into the thin active layers has been considered as a breakthrough strategy to solve this inherent limit and represent an imperative milestone toward the highly efficient OSCs and PeSCs, arising from the significantly enhanced light absorption and electrical characteristics in fundamental. Herein, the recent advances in fabrication and incorporation strategies of plasmonic NPs are reviewed. The in-depth efficiency and stability enhancement mechanisms are investigated and highlighted. Meanwhile, potential strategies and perspectives for their further development of NP-based solution-processing OSCs and PeSCs are presented.

Organic solar cells (OSCs) enjoy a huge market and public attention as an emerging renewable and clean energy source due to their accessibility, sustainability, translucency, good flexibility, non-toxicity, and low preparation cost. However, the choice of an acceptor material is now the pivotal factor restricting the development of OSCs. To continuously improve stability and increase power conversion efficiency (PCE) for better performance, highperformance acceptor materials are an important part of enabling OSCs. From fullerenes and their derivatives and non-fullerenes, we sum up the latest research progress on high-performance acceptor materials for OSCs and then introduce the synthesis methods of non-fullerenes. The latest strategies to enhance the performance of organic solar cells and the wide range of applications of non-fullerene acceptors (NFAs) on different OSCs are also discussed. In addition, challenges faced by OSCs in improving their performance and the prospects for future development are revealed, providing new ideas for designing the next generation of high-performance OSCs. [ABSTRACT FROM AUTHOR]

Lightweight and low-cost organic photovoltaics (OPVs) hold great promise as renewable energy sources. The most critical challenge in developing high-performance OPVs is the incomplete photon absorption due to the low diffusion length of the carrier in organic semiconductors. To date, various attempts have been carried out to improve light absorption in thin photoactive layer based on optical engineering strategies. Nanostructure-induced light harvesting in OPVs offers an attractive solution to realize high-performance OPVs, via the effects of antireflection, plasmonic scattering, surface plasmon polarization, localized surface plasmon resonance and optical cavity. In this review article, we summarize recent advances in nanostructure-induced light harvesting in OPVs and discuss various light-trapping strategies by incorporating nanostructures in OPVs and the fabrication processing of the micro-patterns with high resolution, large area, high yield and low cost.