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The accurate analysis and the characterization of guided-wave structures are important steps in the design of microwave and optical systems. A numerical methodology based on a three-dimensional discontinuous Galerkin time-domain (DGTD) finite element method is used to estimate the resonant frequencies of various structures in a single run, with several polychromatic sources (dipole, plane wave, etc). The proposed approach leads to simulations that are fast and accurate, this being the result of the local discretization strategy of the full-wave time-domain Maxwell’s equations on unstructured tetrahedral meshes that is easy to parallelize. The stability of the overall numerical scheme is obtained using an upwind numerical flux and an implicit second-order accurate time integration scheme. The numerical methodology is verified based on two problems with closed-form solutions. First, homogeneous and non-homogeneous waveguides are studied using the proposed methodology. The WR-284 waveguide is used, and cut-off frequencies of the first 7 modes are computed and compared with analytical solutions. Propagation characteristics of mode-selective transmission lines (MSTL) are then investigated. These MSTL simulation results are verified using measurement results of fabricated MSTL, microstrip, and RWG prototypes up to 110 GHz. Simulation results show the flexibility, the robustness, and the high accuracy of the proposed numerical strategy.

In this work, the concept of high-order space harmonics (HSHs) applied to a hybrid form of nonradiative dielectric (NRD) waveguide and substrate-integrated square waveguide (SIW) involving both TE10 and TE01 modes is investigated with aim to develop multi-functional dually polarized leaky-wave antennas (DP-LWAs). A polarization-selective coupling (PSC) mechanism is used to guide the two orthogonally oriented modes in the hybrid structure to achieve a polarization diversity feature. The polarization effect on unit-cell geometrical structures is examined for different characteristics of LWA such as propagation constant behavior, leakage ratio, and scanning range variation. In addition, we demonstrate how the extrinsic characteristics of HSHs in a one-dimensional periodic LWA and its unit-cell analysis can provide design flexibility through two case studies. In the first case of demonstration, we show a dually polarized multi-beam LWA design with wide scanning capability in which we use both even and odd HSHs of n $\mathrm {\epsilon }$ [−1, −2]). The second antenna design case is concerned with a DP-LWA having the capability of fixed and scanning beams operation by using a combinatory set of PSC and four HSHs of n $\mathrm {\epsilon }$ [−1, −2, −3, −4]. Two DP-LWA prototypes are demonstrated as proofs of concept, and the obtained experimental results show a very good agreement with their simulation and analysis counterparts.

This paper introduces a coupling matrix synthesis method tailored for a class of tunable bandpass filters (BPFs) with reconfigurable filtering characteristics. The filter has symmetric transmission zeros (TZs) which can be reconfigured on the real or imaginary axis of the complex plane. Different filtering characteristics can be obtained by merely tuning resonant frequencies of certain resonators. The frequency tuning and reconfigurable states of the filter can be achieved simultaneously. The synthesis procedure mainly includes three steps. First, a transversal coupling matrix is synthesized according to the design specifications of one of the reconfigurable states. Second, the matrix is transformed to an intermediate configuration, in which some of the coupling coefficients on the mainline couplings are designated as the key coefficients. Tuning these key coefficients alone can change the locations of TZs to realize reconfigurable filtering characteristics. In this intermediate configuration, the coupling matrix is optimized based on the synthesized values to make all reconfigurable filter states meet their design requirements. Finally, the key coefficients are rotated to the diagonal entries of the coupling matrix, so that reconfigurable TZs are realized by merely tuning the resonant frequencies of certain resonators. The reconfigurable mechanism and the maximum number of TZs under different filter orders are investigated in detail for a variety of coupling diagrams. Two synthesis examples are presented to show the novel reconfigurable filter optimization procedure. For experimental verification, a fourth-order tunable BPF with a pair of reconfigurable symmetric TZs was fabricated and measured. The filter states with TZs on the real or imaginary axis of the complex plane can be switched solely by tuning resonant frequencies, which validates the proposed synthesis approach of tunable filters.

The Arctic is one of the most significant changing areas on the Earth under the climate change scenario. More regions in the Arctic are becoming ice-free oceans in the melting season or through the whole year. Therefore, ocean wind and wave, as the two most important parameters in the air–sea interface, are drawing significant attention to the Arctic Ocean. Scatterometer and radar altimeter are the two traditional remote sensing instruments for ocean wind and wave observations, while the former is limited by coarse spatial resolution and the latter has small spatial coverage. Wind and wave data in high spatial resolution and wide coverage by synthetic aperture radar (SAR) are currently lacking in the Arctic Ocean. We developed an ocean wind and wave dataset by Sentinel-1 SAR in the pan-Arctic Ocean (above 60°N), covering January 2017 to May 2021. By comparing with sea surface wind speed data of scatterometer, the SAR-retrieved wind data achieve an accuracy of 1.23 m/s, in terms of root mean square error (RMSE). Compared with significant wave height data of radar altimeter, the SAR retrievals have an RMSE of 0.66 m. The data records are in the standard NetCDF-4 format. The dataset is publicly available at: http://www.dx.doi.org/10.11922/sciencedb.00834.

Deep learning has achieved good performance in hyperspectral image classification (HSIC). Many methods based on deep learning use deep and complex network structures to extract rich spectral and spatial features of hyperspectral images (HSIs) with high accuracy. During the process, how to accurately extract the features and information from pixel blocks in HSIs is important. All of the spectral features are treated equally in classification, and the input of the network often contains much useless pixel information, leading to a low classification result. To solve this problem, an enhanced spectral-spatial residual attention network (ESSRAN) is proposed for HSIC in this article. In the proposed network, the spectral-spatial attention network (SSAN), residual network (ResNet) and long-short term memory (LSTM) are combined to extract more discriminative spectral and spatial features. More specifically, SSAN is first applied to extract image features by using the spectral attention module to emphasize useful bands and suppress useless bands. The spatial attention module is used to emphasize pixels that have same category with the central pixel. Then, these obtained features are fed into an improved ResNet, which adopts LSTM to learn representative high-level semantic features of the spectral sequences, since the use of ResNet can prevent gradient disappearance and explosion. The proposed ESSRAN model is implemented on three commonly used HSI datasets and compared to some state-of-the-art methods. The results confirm that ESSRAN effectively improves accuracy.

Current COVID-19 vaccines are effective countermeasures to control the SARS-CoV-2 virus pandemic by inducing systemic immune responses through intramuscular injection. However, respiratory mucosal immunization will be needed to elicit local sterilizing immunity to prevent virus replication in the nasopharynx, shedding, and transmission. In this study, we first compared the immunoprotective ability of a chimpanzee replication–deficient adenovirus–vectored COVID-19 vaccine expressing a stabilized pre–fusion spike glycoprotein from the ancestral SARS-CoV-2 strain Wuhan–Hu–1 (BV-AdCoV-1) administered through either aerosol inhalation, intranasal spray, or intramuscular injection in cynomolgus monkeys and rhesus macaques. Compared with intranasal administration, aerosol inhalation of BV-AdCoV-1 elicited stronger humoral and mucosal immunity that conferred excellent protection against SARS-CoV-2 infection in rhesus macaques. Importantly, aerosol inhalation induced immunity comparable to that obtained by intramuscular injection, although at a significantly lower dose. Furthermore, to address the problem of immune escape variants, we evaluated the merits of heterologous boosting with an adenovirus–based Omicron BA.1 vaccine (C68–COA04). Boosting rhesus macaques vaccinated with two doses of BV-AdCoV-1 with either the homologous or the heterologous C68–COA04 vector resulted in cross–neutralizing immunity against WT, Delta, and Omicron subvariants, including BA.4/5 stronger than that obtained by administering a bivalent BV-AdCoV-1/C68–COA04 vaccine. These results demonstrate that the administration of BV-AdCoV-1 or C68–COA04 via aerosol inhalation is a promising approach to prevent SARS-CoV-2 infection and transmission and curtail the pandemic spread.

Hu sheep, a locally bred species in China known for its high productivity, is currently suffering from pneumonia. Here, we combine high-throughput 16SrRNA gene sequencing and bacterial culturing to examine the bacterial community in pneumonic Hu Sheep lungs (p < 0.05). The results showed that the abundance and diversity of lung bacteria in healthy sheep were significantly higher than those in pneumonia sheep (p = 0.139), while there was no significant difference between moderate and severe pneumonia. Furthermore, the composition of the lung microbiota community underwent significant alterations between different levels of pneumonia severity. The application of LEfSe analysis revealed a notable enrichment of Mannheimiae within the lungs of sheep afflicted with moderate pneumonia (p < 0.01), surpassing the levels observed in their healthy counterparts. Additionally, Fusobacterium emerged as the prevailing bacterial group within the lungs of sheep suffering from severe pneumonia. Integrating the results of bacterial isolation and identification, we conclusively determined that Mannheimia haemolytica was the primary pathogenic bacterium within the lungs of sheep afflicted with moderate pneumonia. Furthermore, the exacerbation of pneumonia may be attributed to the synergistic interplay between Fusobacterium spp. and other bacterial species. Our results provide new insights for guiding preventive and therapeutic measures for pneumonia of different severities in sheep.

Sunscald in kiwifruit, an environmental stress caused by solar radiation during the summer, reduces fruit quality and yields and causes economic losses. The efficient and timely detection of sunscald and similar diseases is a challenging task but helps to implement measures to control stress. This study provides high-precision detection models and relevant spectral information on kiwifruit physiology for similar statuses, including early-stage sunscald, late-stage sunscald, anthracnose, and healthy. Primarily, in the laboratory, 429 groups of spectral reflectance data for leaves of four statuses were collected and analyzed using a hyperspectral reflection acquisition system. Then, multiple modeling approaches, including combined preprocessing methods, feature extraction algorithms, and classification algorithms, were designed to extract bands and evaluate the performance of the models to detect the statuses of kiwifruit. Finally, the detection of different stages of kiwifruit sunscald under anthracnose interference was accomplished. As influential bands, 694–713 nm, 758–777 nm, 780–799 nm, and 1303–1322 nm were extracted. The overall accuracy, precision, recall, and F1-score values of the models reached 100%, demonstrating an ability to detect all statuses with 100% accuracy. It was concluded that the combined processing of moving average and standard normal variable transformations (MS) could significantly improve the data; the near-infrared support vector machine and visible convolutional neural network with MS (NIR-MS-SVM and VIS-MS-CNN) were established as high-precision detection techniques for the classification of similar kiwifruit statuses, demonstrating 25.58% higher accuracy than the single support vector machine. The VIS-MS-CNN model reached convergence with a stable cross-entropy loss of 0.75 in training and 0.77 in validation. The techniques developed in this study will improve orchard management efficiency and yields and increase researchers’ understanding of kiwifruit physiology.

We present a novel fast synthetic aperture radar (SAR) image simulation method based on the echo matrix cell algorithm including multiple scattering. To improve the efficiency of SAR image simulation while ensuring the fidelity of the simulated results, we first discretized the target facets set in the SAR beams footprint into lattice targets using the range-Doppler (RD) imaging geometry model and provided the basis for simulating electromagnetic wave transmission. Based on the simulation of electromagnetic waves transmission, we used the ray tracing algorithm to calculate the multiple backscattering field including multi-polarimetric information and various material properties. Then, based on the echo matrix cell algorithm, we set the echo matrix cell as the subfield of the target backscattering field and designed the CUDA kernel function to implement a computation parallelization for SAR echo generation. All the echo matrix cells are traversed in parallel to obtain the total backscattering field of the target, reproducing the time-varying characteristic of the backscatter coefficient for each lattice target within the synthetic aperture time. The echo signal is processed using the RD imaging algorithm to obtain the simulated SAR image. Finally, we select some targets including aircraft carrier and airplane models for simulation tests. The computation efficiency is improved over 170-fold by comparing the computations of the proposed method and CPU single-thread. We also performed some qualitative and quantitative evaluations on the fidelity of the simulated SAR images. The experimental results verify the effectiveness of the proposed method.

Abstract Introduction The development of pancreatic ductal adenocarcinoma (PDAC) is closely tied with the immune system. C‐C motif chemokine ligands (CCL) were proved to lead to immune recruitment and training. Thus, we reckoned CCL2 to be the kernel of immune suppression in PDAC tissues. Methods We compared normal pancreatic tissues with PDAC tissues according to The Cancer Genome Atlas (TCGA) and clinical samples. Flow cytometry was used to identify M‐MDSCs. We further demonstrated immune suppression of M‐MDSCs according to proliferation rates of CD8+ T cells/CD4+ T cells. Levels of reactive oxygen species (ROS) and Arginase were also tested by flow cytometry, enzyme‐linked immunosorbent assay, and western blot analysis. We also analyzed the specific mechanisms by cluster analysis after CCL2 stimulating M‐MDSCs. Results We found that CCL2 highly increased in PDAC tissues. CCL2 is positively related to CD33 and CD14, markers of monocytic myeloid‐derived suppressor cells (M‐MDSCs). We have demonstrated that CCL2 recruited M‐MDSCs into PDAC tissues both in vitro and in vivo. M‐MDSCs recruitment is accompanied by sustained immune suppression. Furthermore, we have found that M‐MDSCs impeded T cell proliferation and produced high levels of ROS and Arginase, which can be enhanced by CCL2. Mechanistically, CCL2 stimulated M‐MDSCs led to a significant upregulation of genes, a large part of which accumulated in the mitogen‐activated protein kinase signaling pathway. Treatment of aloesin, MAPK signaling inhibitor, relieved the associated immunosuppressive phenotype induced by CCL2. Conclusions Our study indicates that PDAC cells produced CCL2, which promoted localized M‐MDSC recruitment and immune suppression, thereby promoting tumor progression.

Abstract Background Bainbridge-Ropers syndrome (BRPS) [OMIM#615485] is a neurodevelopmental disorder, characterized by delayed psychomotor development with generalized hypotonia, moderate to severe intellectual disability, poor or absent speech, feeding difficulties, growth failure, dysmorphic craniofacial features and minor skeletal features. The aim of this study was to investigate the genetic etiology of a Sudanese boy with severe developmental delay, intellectual disability, and craniofacial phenotype using trio-based whole-exome sequencing. To our knowledge, no patients with ASXL3 gene variant c.3043C>T have been reported detailedly in literature. Case presentation The patient (male, 3 years 6 months) was the first born of a healthy non-consanguineous couple originating from Sudan, treated for “psychomotor retardation” for more than 8 months in Yiwu. The patient exhibited severely delayed milestones in physiological and intellectual developmental stages, language impairment, poor eye-contact, lack of subtle motions of fingers, fear of claustrophobic space, hypotonia, clinodactyly, autistic features. Peripheral blood samples were collected from the patient and his parents. Trio-based whole-exome sequencing(Trio-WES) identified a de novo heterozygous ASXL3 gene variant c.3043C>T;p.Q1015X. Sanger sequencing verified variants of this family. Conclusion Trio-WES analysis identified a de novo nonsense variant (c.3043C>T) of ASXL3 gene in a Sudanese boy. To our knowledge, the patient with this variant has not been reported previously in literature. This study presents a new case for ASXL3 gene variants, which expanded the mutational and phenotypic spectrum.

Metamaterials (MTMs) based on a periodic array of resonant coils have been shown to behave as μ-negative (MNG), enabling the focusing of magnetic flux. The phenomenon has been deployed by designers to boost the efficiency of many inductively coupled systems, such as magnetic resonance imaging, underwater and underground communications, and charging base stations (CBS) for consumer electronics and implanted devices. However, due to their dependency on high-Q unit cells, linear MNG-like MTMs have limited bandwidth, restricting their use in many applications, notably in near-field simultaneous wireless information and power transmission (NF-SWIPT) systems. To improve the tight constraints of the amplitude-bandwidth trade-off of artificial magnetic lenses, this paper presents a theoretical analysis of nonlinear MTMs based on a lattice of Duffing resonators (DRs). Additionally, it introduces a criterium for the quantification and evaluation of the amplitude-bandwidth enhancement. The analytical results are based on a circuit model and further verified by numerical simulations using commercial software. The preliminary findings in this paper open up possibilities for nonlinear MTM lenses and can be applied to enhance the linear amplitude-bandwidth limit.

Quantitative prediction of nitrate contents in different industrial wastewater was carried out using Fourier transform infrared attenuated total reflectance (FTIR-ATR) spectroscopy. The algorithm of Gaussian deconvolution was applied in the spectral range of 1500–1200 cm−1 to eliminate the background interferences on target information of nitrate, and partial least squares regression (PLSR) model and support vector machine (SVR) model were developed for the prediction of nitrate. The results showed that the PLSR model (Rv2 = 0.921, RMSEv = 0.351 mg/L, RPDv = 3.56) and SVR model (Rv2 = 0.856, RMSEv = 0.473 mg/L, RPDv = 3.15) reached excellent prediction accuracy and robustness for electroplating wastewater, and for metallurgical wastewater the SVR model (Rv2 = 0.916, RMSEv = 1.38 mg/L, RPDv = 3.26) showed a better prediction performance. The PLSR and SVR models exhibited poor prediction accuracy of nitrate for pesticide wastewater and dyeing wastewater due to the strongly interference by carbonate. The spectra pretreatment by deconvolution dramatically improved the prediction models. Therefore, combined with deconvolution spectra pretreatment and chemometrics methods, FTIR-ATR could achieve a fast and effective in-situ monitoring of nitrate in industrial wastewater.

We evaluated the immunogenicity and protective ability of a chimpanzee replication-deficient adenovirus vectored COVID-19 vaccine (BV-AdCoV-1) expressing a stabilized pre-fusion SARS-CoV-2 spike glycoprotein in golden Syrian hamsters. Intranasal administration of BV-AdCoV-1 elicited strong humoral and cellular immunity in the animals. Furthermore, vaccination prevented weight loss, reduced SARS-CoV-2 infectious virus titers in the lungs as well as lung pathology and provided protection against SARS-CoV-2 live challenge. In addition, there was no vaccine-induced enhanced disease nor immunopathological exacerbation in BV-AdCoV-1-vaccinated animals. Furthermore, the vaccine induced cross-neutralizing antibody responses against the ancestral strain and the B.1.617.2, Omicron(BA.1), Omicron(BA.2.75) and Omicron(BA.4/5) variants of concern. These results demonstrate that BV-AdCoV-1 is potentially a promising candidate vaccine to prevent SARS-CoV-2 infection, and to curtail pandemic spread in humans.

In this paper, we define one kind of new Jack polynomials denoted by Jkλ, which equal the standard Jack polynomials Pλα multiplied by a coefficient. We show that the structure constant Cλμν in Jkλ⋅Jkμ=∑νCλμνJkν can be given from affine Yangian of gl(1). Then we give the Boson-Fermion correspondence for Jack polynomials Jkλ and show that 3-Schur functions Sπ equal Jack polynomials Jkλ in the special case of h1=h, h2=−h−1, h3=−h+h−1.

BackgroundPathogenic variants in the FIG4 gene have been described to be associated with a diverse spectrum of syndromes, such as autosomal recessive bilateral temporooccipital polymicrogyria (OMIM 612691), autosomal dominant amyotrophic lateral sclerosis-11 (ALS11; OMIM 612577), autosomal recessive Charcot-Marie-Tooth disease, type 4J (CMT4J; OMIM 611228), and autosomal recessive Yunis-Varon syndrome (YVS; OMIM 216340). Heterozygous FIG4 variants are responsible for ALS11 characterized by progressive muscular weakness, atrophy, and bulbar palsy. CMT4J is a disorder of peripheral nervous system defects mainly presenting with a highly variable onset of proximal and/or distal muscle weakness. YVS is a disorder of severe neurological involvement with central nervous system (CNS) dysfunction and extensive skeletal anomalies.Case PresentationWe reported two Chinese siblings born with a weakness in all limbs. They experienced rapidly progressive weakness in distal limbs. At the age of 6 years, the elder brother presented with severe scoliosis and cervical kyphosis. They both had global developmental delay and a CNS involvement with cognitive deficits and swallowing problems. Genetic screening in the patients' family for inherited diseases was recommended. Novel compound heterozygous variants in the FIG4 gene (c.2148delTinsAA and c.317A > G) were found by whole-exome sequencing in the patients. These variants were confirmed by Sanger sequencing in family members.ConclusionsHerein, we reported two Chinese male patients with CMT4J who presented with abnormal CNS features. CMT4J with CNS involvement has been very rarely reported. We hoped this study could expand the phenotypic and genetic spectrum of FIG4-related diseases. And we helped physicians to understand the genotype–phenotype correlation.

Cistanche deserticola Y.C. Ma (CD) possesses hepatoprotective activity, while the active ingredients and involved mechanisms have not been fully explored. The objective of this study was to investigate the chemical composition and hepatoprotective mechanisms of CD. We primarily used ultra-performance liquid chromatography with quadrupole time-of-flight tandem mass spectrometry (UPLC-Q-TOF-MS/MS) to identify the phenylethanoid glycoside (PhG) components of CD. Then, network analysis was used to correlate and predict the pharmacology of the identified active components of PhGs with hepatoprotection. Next, the mechanisms of the core components and targets of action were explored by cellular assays and toll-like receptor 4 (TLR4) target competition assays. Finally, its hepatoprotective effects were further validated in in vivo experiments. The results showed that a total of 34 PhGs were identified based on the UPLC-Q-TOF-MS/MS method. Echinacoside (ECH) was identified as the key ingredient, and TLR4 and nuclear factor-kappa B (NF-κB) were speculated as the core targets of the hepatoprotective effect of CD via network analysis. The cellular assays confirmed that PhGs had significant anti-inflammatory activity. In addition, the real-time quantitative polymerase chain reaction (RT-qPCR) and Western blot indicated that ECH notably reduced the levels of interleukin 6 (IL-6) and tumor necrosis factor alpha (TNF-α), as well as the mRNA expression of TLR4, TNF-α, and IL-6, and decreased the high expression of the TLR4 protein, which in turn downregulated the myeloid differentiation factor 88 (MyD88), p-P65 and TNF-α proteins in the inflammatory model. The target competition experiments suggested that ECH and LPS could competitively bind to the TLR4 receptor, thereby reducing the expression of TLR4 downstream proteins. The results of in vivo studies showed that ECH significantly ameliorated LPS-induced hepatic inflammatory infiltration and liver tissue damage and reduced serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels in mice. Moreover, ECH remarkably inhibited the release of inflammatory factors such as TNF-α, IL-6, IL-1β, and MCP-1 in the serum of mice, exerting the hepatoprotective effect by the TLR4/NF-κB signaling pathway. More importantly, ECH could act as a potential inhibitor of TLR4 and deserves further in-depth study. Our results could provide a basis for exploring the hepatoprotective properties of CD.

Water mist plays an indispensable role in various fire extinguishing scenarios, but its efficiency is restricted by the interaction area of droplets and the flame. With the characteristics of simple operation and limited environmental pollution, acoustic waves can deliver water droplets to flame and have the potential to enhance the fire extinguishing efficiency of water mist. However, our understanding of the synergistic effect of acoustics and water mist on suppressing flame is still limited. In this study, the enhancement mechanisms of acoustic waves in extinguishing ethanol fueled flames are experimentally investigated. The effects of critical parameters related to sound pressure and acoustic-induced air speed on flame behaviors are comprehensively discussed. Initially, the flames cannot be extinguished by the pure water mist. Coupling with acoustic waves, the response of the flames significantly changes. For the acoustic wave of a sine signal, the frequency of 30 Hz induces a greater air speed, which is superior to the frequency of 40 Hz and 50 Hz in suppressing the flame. The acoustically enhanced mechanism is perhaps related to the acoustic-induced wind, which causes the flame oscillation. The flame and the wick expose the larger area to be cooled directly by the water mist when the flame is “pulled” and “pushed” by the acoustic-induced wind. A dimensionless number θ is finally estimated for determining the critical flame extinction condition for this collaborative system. These results could advance our understanding of the underlying enhancement mechanisms in coupling acoustics with water mist and provide scientific insights into applying this novel suppression strategy.

Background: Coffin–Lowry syndrome (CLS) [OMIM#303600] is a rare X-linked dominant syndrome. CLS is caused by highly heterogeneous loss-of-function mutations in the RPS6KA3 gene (OMIM*300,075). CLS is characterized by intellectual disability (ID), short stature, tapered fingers, characteristic facial features, and progressive skeletal changes. Distal 22q11.2 microdeletion syndrome (OMIM#611867) is an autosomal dominant and recurrent genomic disorder. It mainly includes three types [distal type I (D–E/F), type II (E–F), and type III (F–G)] and exhibits variable clinical phenotypes (mild, moderate, or even normal): preterm birth, pre- and/or postnatal growth restriction, development delay, ID, behavioral problems, cardiovascular defects, skeletal anomalies, and dysmorphic facial features. We investigated the genetic etiology of a Chinese pedigree with ID, short stature, digit abnormalities, facial dysmorphism, and menstrual disorder. A heterozygous RPS6KA3 gene variant c.898C>T (p.R300X) was identified in this familial case. Two female CLS patients with distal 22q11.2 microdeletion presented with more severe clinical phenotypes. We provided clinical characteristics of these Chinese female CLS patients.Case presentation: We described a Chinese family with three affected females (the mother, the elder sister, and the proband). The mother and the elder sister had more severe clinical phenotypes (moderate facial dysmorphism, more severe cognitive impairment, and shorter stature). The common characteristic phenotypes are ID, short stature, facial dysmorphism, irregular menstruation, and cardiovascular disorders. Peripheral blood samples were collected from the pedigree. Whole-exome sequencing (WES) identified a heterozygous nonsense RPS6KA3 gene variant c.898C>T (p.R300X). It was verified by Sanger sequencing. Copy number variation sequencing (CNV-seq) showed that both the mother and the elder sister carried a CNVseq [hg19] del (22) (q11.22-q11.23) (22997582–23637176)×0.5. RNA from peripheral blood samples was used for measuring the relative quantification of mRNA (expressed by exon 14 of RPS6KA3). The levels of mRNA relative expressions were significantly lower in the mother’s and the elder sister’s blood samples. The levels of mRNA relative expressions were significantly higher in the proband’s blood sample. X-chromosome inactivation (XCI) studies demonstrated that the proband showed extremely skewed XCI, and the XCI pattern of the elder sister was random.Conclusion: Herein, we reported three Chinese female patients with a heterozygous nonsense RPS6KA3 gene variant c.898C>T. Further genetic studies were performed. To our knowledge, Chinese patients with this variant have not been previously reported in the literature. The three female patients presented with variable degrees of severity. The clinical characteristics of these Chinese female CLS patients could expand the phenotypic spectrum of CLS. We helped physicians to understand the genotype–phenotype correlation further.

BackgroundFocal segmental glomerulosclerosis (FSGS) is a histopathological diagnosis of the sclerosis of glomeruli and the damage to renal podocytes. FSGS affects the filtration function of the kidneys and results in nephrotic syndrome (NS) in children and adults. FSGS is a clinically and genetically heterogeneous disorder. FSGS-1 [OMIM #603278] is one of the progressive hereditary renal diseases. It is caused by heterozygous variants of the actinin alpha 4 (ACTN4) [OMIM*604638] gene on chromosome 19q13.2 in a dominant inheritance (AD) manner. With the recent development of whole-exome sequencing (WES), 22 (including our case) pathogenic or likely pathogenic variants have been identified in ACTN4 gene.Case presentationWe reported a 17-year-old Chinese girl who was hospitalized with foamy urine, nausea and vomiting. Laboratory tests revealed increased levels of serum creatinine and urea nitrogen. Ultrasonography demonstrated bilaterally reduced size of kidneys. The primary diagnoses were NS and chronic kidney disease stage 5 (CKD5). The hemodialysis was initiated in 48 h after admission. After 4 months of treatment, the patient received an allogeneic kidney transplantation from her father. A novel heterozygous missense variant c.494C > T (p.A165V) in the ACTN4 gene was found by WES in the patient. This variant was confirmed by Sanger sequencing. The computational simulation of the stability of mutant protein (p.A165V) was decreased. Interatomic interactions of the p.A165V site were increased, and it might be associated with the increased ubiquitylation in the vicinity of the mutant site.ConclusionAs per the guidelines of the American College of Medical Genetics and Genomics for interpreting sequence variants, the novel heterozygous missense variant was pathogenic (PS2 + PM1 + PM2 + PP3 + PP4). It should be noted that the early onset of severe proteinuria with a poor prognosis is an important and universal symptom for most genetic FSGS. If necessary, genetic screening is recommended.

Background: Orthodontic tooth movement mainly depends on biological and mechanical reactions in the periodontium, such as the indispensable reconstruction process of the periodontal ligament and alveolar bone. To explore whether orthodontic compressive force can induce bone resorption during orthodontic tooth movement by regulating the GSK-3β/β-catenin pathway. Methods: We established orthodontic tooth movement models in Sprague-Dawley rats. In addition, compressive force-induced bone resorption that occurred during orthodontic tooth movement was analyzed by HE staining and micro-CT. The number and distribution of osteoclasts were observed by TRAP staining. Furthermore, pressure-induced bone resorption mediated by the GSK-3β/β-catenin signaling pathway was analyzed by immunohistochemistry. Results: As shown by the micro-CT results, bone parameters, such as bone mineral density (BMD), the bone volume fraction (BV/TV), and trabecular thickness (Tb. Th), were significantly decreased under orthodontic compressive force stimulation, in contrast with the dramatically increased trabecular spacing (Tb. Sp). During the process of tooth movement, the compressive force can induce bone resorption on the side with the force, which increases the expression of phosphorylated Ser-GSK-3β and activation of the β-catenin signaling pathway. Additionally, downregulation of the GSK-3β activity further caused the downregulation of bone parameters, leading to bone loss. The TRAP staining and immunohistochemistry staining results indicated that orthodontic compressive force influenced osteoclast formation and the secretion of osteoclast-related cytokines, matrix metallopeptidase 9 (MMP-9) and receptor activator of nuclear factor-κB ligands (RANKLs), which is also related to the duration of orthodontic force. Conclusions: These results indicated that the GSK-3β inhibitor can promote osteoclast formation on the side with orthodontic compressive force. In addition, the activation of the GSK-3β/β-catenin signaling pathway contributes to bone reconstruction caused by orthodontic compressive force. Therefore, the GSK-3β/β-catenin signaling pathway can be a potential target for further clinical applications.

BackgroundRecently, the combination of immunotherapy with chemotherapy has been recommended as first-line treatment of metastatic gastric/gastroesophageal junction (G/GEJ) in the clinical guidelines of many countries; the therapeutic potential of this application needs to be further investigated for neoadjuvant therapy of advanced G/GEJ cancer patients.MethodsWe performed a prospective, single-arm, open-label, phase 2 trial of the PD-1 inhibitor tislelizumab combined with S-1 plus oxaliplatin (SOX) in patients with advanced LAG/GEJ cancer. All patients underwent the three-cycle (21 days/cycle) treatment except for one patient who underwent two cycles. The primary endpoints were tumor major pathology response (MPR) and other events of tumor response assessed by the RECIST 1.1 and Becker criteria. Moreover, we constructed a few-shot learning model to predict the probability of MPR, which could screen those patients who might benefit from the neoadjuvant immunotherapy–chemotherapy scheme. This study was registered at https://clinicaltrials.gov/ct2/show/NCT0-4890392.ResultsThirty-two patients were enrolled; 17 patients (53.1%) achieved MPR (≤10% viable tumor cells) after treatment, and among them, 8 (25.0%) had a pathological complete response (pCR). The 1-year overall survival (OS) rate was 91.4% and the 1-year recurrence-free survival (RFS) rate was 90.0%. Adverse events occurred in 24 patients (65.6%) and grade III–IV adverse events were observed in 4 patients (12.5%) during the neoadjuvant period. Furthermore, we found commonly used preoperative assessment tools such as CT and EUS, which presented limited accuracy of tumor therapeutic response in this study; thus, we developed a therapeutic response predictive model that consisted of TNFα, IFNγ, IL-10, CD4, and age of patient, and the AUC of this FSL model was 0.856 (95% CI: 0.823–0.884).DiscussionOur study showed that the neoadjuvant PD-1 inhibitor tislelizumab combined with SOX had promising application potential and presented no increasing treatment-related adverse events in patients with advanced G/GEJ cancer. Moreover, the predictive model could help therapists to evaluate the therapeutic response of this scheme accurately.Clinical Trial Registrationhttps://clinicaltrials.gov/ct2/show/NCT0-4890392, identifier [NCT04890392].

Precipitation variability and nitrogen (N) deposition caused by anthropogenic activities could profoundly impact ecosystem productivity and carbon cycling. In desert ecosystems, vegetation is sensitive to changes in precipitation and N deposition. However, the impacts of large changes in precipitation, especially with a concurrent increase in N content, on plant community remain unclear. In this study, we carried out experiments to monitor the impacts of five precipitation levels and two N levels on the plant community function and composition from the Junggar desert in Central Asia during the period 2018–2019. Our results showed that: (1) Aboveground net primary production (ANPP) significantly increased with increasing precipitation, it followed a positive linear model under normal precipitation range, and nonlinear mode under extreme precipitation events; (2) N application led to an increase in ANPP, but did not significantly improve the sensitivity of ANPP to precipitation change; (3) Changes in N content and precipitation, and their impacts on ANPP were mainly driven by plant density. These results provide a theoretical basis for predict the future dynamics of terrestrial vegetation more accurately under climate change and increasing nitrogen deposition.

In this study, superhydrophobic ZnO/Polytetrafluoroethylene (ZnO/PTFE) films with water droplet contact angles (CA) observed as high as 165° and water droplet sliding angles of (SA) E. coli, the cell viability count of the ZnO/PTFE sample (3.5 log reduction, 99.96%) was conspicuously lower than that of the ZnO/HDTMS sample (1.2 log reduction, 93.87%) under 1 h illumination of UV light, which showed that the ZnO/PTFE sample has a better photocatalytic property than the ZnO/ HDTMS films. The ZnO/PTFE films also showed good mechanical robustness, which is an important consideration in their widespread real-world adoption.

This study aimed to investigate the distribution of microplastics (MPs) within the Shiwuli River in Hefei, a Chinese inland city. Water and sediment samples were collected during flood season (from May to September) and non-flood season (from October to April) at 10 representative points along the truck stream. The electron microscope, the laser direct infrared chemical imaging system (LDIR), and the scanning electron microscope (SEM) were used to observe and quantify the colour and shape of the MPs, to identify the number, size, and polymer composition of the MPs, and to observe the microstructures of typical MP particles, respectively. The polymer risk index (RI) model and the pollution load index (PLI) model were used to assess the polymer-related risks and the overall extent of MP pollution in the river, respectively. Analysis of MP abundance for different sampling points showed that the water of Shiwuli River had an average abundance of MPs of 8.4 ± 2.5 particles/L during the flood season and 5.8 ± 1.7 particles/L during the non-flood season; the sediment had an average abundance of MPs of 78.9 ± 8.3 particles/kg during the flood season and 63.9 ± 7.1 particles/kg during the non-flood season. The abundance of MPs of different points was investigated. Result show that the more abundances of MPs were found at confluences with tributaries (S4, S5, and S6), where they are also close to the residential and industrial development, while lower values were found in agricultural areas (S8) and wetland ecological regions (S9 and S10). In water, the maximum appeared at S5 with 21.7 ± 4.6 particles/L during the flood season and 15.9 ± 4.2 particles/L during the non-flood season, respectively; the minimum appeared at S9 with 1.8 ± 1.0 particles/L during the flood season and 2.2 ± 0.4 particles/L during the non-flood season, respectively. In sediment, the maximum appeared at S5 with 174.1 ± 10.1 particles/kg during the flood season and 143.6 ± 10.4 particles/kg during the non-flood season, respectively; the minimum appeared at S8 with 10.3 ± 2.8 particles/kg during the flood season and at S9 with 12.1 ± 3.2 particles/kg during the non-flood season, respectively. MP characteristics were also studied. Results show that the MPs mainly exhibited a fibroid morphology (27.90–34%), and red-coloured particles (19.10%) within the smaller size less than 500 μm (38.60%) were more prevalent. Additionally, the result of LDIR scanning shows that a total of eleven types of MP polymers were found in the river water and sediment, including acrylates (ACR), chlorinated polyethylene (CPE), ethylene vinyl acetate (EVA), polyethylene (PE), polyethylene terephthalate (PET), polypropylene (PP), polystyrene (PS), polyurethane (PU), polyvinylchloride (PVC), polyamide (PA), and silicon. The most common particle was PE (19.3–21.6%). Furthermore, the environmental risk assessment demonstrated that the PS polymer posed a Level-III risk in the water samples and a Level-II risk in the sediment samples from the Shiwuli River. The remaining polymer types exhibited Level-I risk. The PLIzone value for water was 2.24 during the flood season, indicating heavy pollution, and 1.66 during the non-flood season, indicating moderate pollution. Similarly, the PLIzone value for sediments was 2.34 during the flood season and 1.91 during the non-flood season, both suggesting a heavy pollution. These findings highlight the potential risk posed by MP pollution in the Shiwuli River to the quality of drinking water sources in Chaohu Lake in Hefei. They provide valuable insights into management, pollution control, and integrated management strategies pertaining to MPs in urban inland rivers in Hefei.

The increasing demand for fine and high-value-added chemicals has motivated the lasting exploration of high-performance catalysts with desirable activity, selectivity, and durability [...]

Due to the existing coupling loop between the motor drive system and the ground, when switching devices in the drive system operate at a high speed, common-mode (CM)-conducted electromagnetic interference (EMI) is generated in the loop. The electromagnetic environment quality of the motor and peripheral equipment is reduced. As a small and lightweight EMI suppression device, the active EMI filter (AEF) can be used in the motor drive system to suppress the CM-conducted EMI. As the unity gain bandwidth of the operational amplifier (OPAMP) in the AEF is fixed, the CM-conducted EMI suppression effect of the AEF is poor. To solve this problem, the multiple-amplification-channel method is used to spread the frequency range of the CM voltage that the AEF can sense; therefore, the frequency of the compensation current output using the AEF is more complete, and a better CM-conducted EMI attenuation effect is obtained. At the same time, a combination of the period frequency carrier modulation (PCFM) and the multi-amplification-channel AEF is adopted so that the CM-conducted EMI in the whole EMI frequency range of the motor drive system is effectively suppressed.

Triple-negative breast cancer (TNBC) lacks estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2 expressions, making targeted therapies ineffective. Mesenchymal stem cells (MSCs) have emerged as a promising approach for TNBC treatment by modulating the tumor microenvironment (TME) and interacting with cancer cells. This review aims to comprehensively overview the role of MSCs in TNBC treatment, including their mechanisms of action and application strategies. We analyze the interactions between MSC and TNBC cells, including the impact of MSCs on TNBC cell proliferation, migration, invasion, metastasis, angiogenesis, and drug resistance, along with the signaling pathways and molecular mechanisms involved. We also explore the impact of MSCs on other components of the TME, such as immune and stromal cells, and the underlying mechanisms. The review discusses the application strategies of MSCs in TNBC treatment, including their use as cell or drug carriers and the advantages and limitations of different types and sources of MSCs in terms of safety and efficacy. Finally, we discuss the challenges and prospects of MSCs in TNBC treatment and propose potential solutions or improvement methods. Overall, this review provides valuable insights into the potential of MSCs as a novel therapeutic approach for TNBC treatment.

Abstract Background Identifying patients who may develop severe coronavirus disease 2019 (COVID‐19) will facilitate personalized treatment and optimize the distribution of medical resources. Methods In this study, 590 COVID‐19 patients during hospitalization were enrolled (Training set: n = 285; Internal validation set: n = 127; Prospective set: n = 178). After filtered by two machine learning methods in the training set, 5 out of 31 clinical features were selected into the model building to predict the risk of developing severe COVID‐19 disease. Multivariate logistic regression was applied to build the prediction nomogram and validated in two different sets. Receiver operating characteristic (ROC) analysis and decision curve analysis (DCA) were used to evaluate its performance. Results From 31 potential predictors in the training set, 5 independent predictive factors were identified and included in the risk score: C‐reactive protein (CRP), lactate dehydrogenase (LDH), Age, Charlson/Deyo comorbidity score (CDCS), and erythrocyte sedimentation rate (ESR). Subsequently, we generated the nomogram based on the above features for predicting severe COVID‐19. In the training cohort, the area under curves (AUCs) were 0.822 (95% CI, 0.765–0.875) and the internal validation cohort was 0.762 (95% CI, 0.768–0.844). Further, we validated it in a prospective cohort with the AUCs of 0.705 (95% CI, 0.627–0.778). The internally bootstrapped calibration curve showed favorable consistency between prediction by nomogram and the actual situation. And DCA analysis also conferred high clinical net benefit. Conclusion In this study, our predicting model based on five clinical characteristics of COVID‐19 patients will enable clinicians to predict the potential risk of developing critical illness and thus optimize medical management.

Leaf nitrogen (N) concentration plays an important role in biochemical and physiological functions, and N availability directly influences rice yield. However, excessive N fertilization is considered to be a root cause of environmental issues and low nitrogen use efficiency. Therefore, the selection of appropriate nutrient management practices and organic amendments is key to maximizing nitrogen uptake and maintaining high and sustainable rice production. Here, we evaluated the effects of different 15N-labelled nitrogen sources (urea, ammonium nitrate, and ammonium sulfate at 315 kg ha−1) with or without biochar (30 t ha−1) on paddy soil properties, root growth, leaf gas exchange, N metabolism enzymes, and N uptake in the early and late seasons of 2019. We found significant differences among N fertilizer sources applied with or without biochar (P

Despite advances in screening and treatment, colon cancer remains one of the leading causes of cancer-related death. Finding novel and useful drug treatment targets is also an urgent need for clinical applications. Tetrandrine (Tet) is extracted from the Chinese medicinal herbal medicine, which is a well-known calcium blocker with a variety of pharmacological activities, including anti-cancer. In this study, we recruited cell viability assay, flow cytometry analysis, cloning formation to confirm that Tet can inhibit the proliferation of SW620 cells, and induce apoptosis. Mechanically, we confirmed that Tet up-regulates the mRNA and protein level of BMP9 in SW620 cells. Over-expression BMP9 enhances the anti-cancer effects of Tet in SW620 cells, but these effects can be partly reversed by silencing BMP9. Also, Tet reduces phosphorylation of Aktl/2/3 in SW620 cells, which could be elevated by overexpressed BMP9 and impaired by silencing BMP9. Furthermore, we demonstrated that Tet reduces phosphorylated PTEN, which can be promoted by overexpressed BMP9, analogously also be attenuated through silencing BMP9. Finally, we introduced a xenograft tumor model to investigate the anti-proliferative effect of Tet, further to explore the effects of BMP9 and PTEN in SW620 cells. Our findings suggested that the anti-cancer activity of Tet in SW620 cells may be mediated partly by up-regulating BMP9, followed by inactivation PI3K/Akt through up-regulating PTEN at least.

Abstract We analyze the rainbow tensor model and present the Virasoro constraints, where the constraint operators obey the Witt algebra and null 3-algebra. We generalize the method of W-representation in matrix model to the rainbow tensor model, where the operators preserving and increasing the grading play a crucial role. It is shown that the rainbow tensor model can be realized by acting on elementary function with exponent of the operator increasing the grading. We derive the compact expression of correlators and apply it to several models, i.e., the red tensor model, Aristotelian tensor model and r = 4 rainbow tensor model. Furthermore, we discuss the case of the non-Gaussian red tensor model and present a dual expression for partition function through differentiation.

Abstract Background Anastomosis-related complications are common after the radical resection of colon cancer. Among such complications, severe stenosis or completely occluded anastomosis (COA) are uncommon in clinical practice, and the separation of the anastomosis is even rarer. For such difficult problems as COA or anastomotic separation, clinicians tend to adopt surgical interventions, and few clinicians try to solve them through endoscopic operations. Case presentation In this article, we present a case of endoscopic treatment of anastomotic closure and separation after radical resection for sigmoid carcinoma. After imaging examination and endoscopic evaluation, we found that the patient had a COA accompanied by a 3–4 cm anastomotic separation. With the aid of fluoroscopy, we attempted to use the titanium clip marker as a guide to perform an endoscopic incision and successfully achieved recanalization. We used a self-expanding covered metal stent to bridge the intestinal canal to resolve the anastomotic separation. Finally, the patient underwent ileostomy takedown, and the postoperative recovery was smooth. The follow-up evaluation results showed that the anastomotic stoma was unobstructed. Conclusions We reported the successful application of endoscopic technique in a rare case of COA and separation after colon cancer surgery, which is worth exploring and verifying through more clinical studies in the future.

After completing the thio-substitution with Lawesson’s reagent, ethanol was found to be effective in the decomposition of the inherent stoichiometric six-membered-ring byproduct from the Lawesson’s reagent to a highly polarized diethyl thiophosphonate. The treatment significantly simplified the following chromatography purification of the desired thioamide in a small scale preparation. As scaling up the preparation of two pincer-type thioamides, we have successfully developed a convenient process with ethylene glycol to replace ethanol during the workup, including a traditional phase separation, extraction, and recrystallization. The newly developed chromatography-free procedure did not generate P-containing aqueous waste, and only organic effluents were discharged. It is believed that the optimized procedure offers the great opportunity of applying the Lawesson’s reagent for various thio-substitution reactions on a large scale.

Research into single crystal organic-inorganic halide perovskites have gained momentum due to the potential applications, yet the growth is still a challenge. Here, the authors demonstrate a universal method based on polymer controlled nucleation process to achieve large-size and high-quality perovskite single crystals.

Abstract An efficient modes analyses technique for isotropic or anisotropic material filled 2D metallic waveguides with an arbitrary contour using the multifilament current method (MFCM) is presented. The ideal PEC boundary of a 2D waveguide is replaced by a shell with a high conductivity and electrical small thickness. The thin lossy shell not only can well approximate the boundary condition of PEC waveguide wall therefore without altering the initial waveguide modes, but also can let the external excitation penetrate through to excite the inside modes, resulting in a high internal field intensity at the frequency of each mode. In this case, the modes are revealed by the peaks of field intensity responses, and the spurious modes which existed in traditional source‐free modes determination techniques can be completely avoided. Based on this idea, a generalized impedance boundary condition (GIBC) is formulated to represent the lossy waveguide wall and further utilized in the MFCM for simulating the internal field intensity over frequency. Three different configurations of a 2D waveguide are considered. The computed modes are compared with that obtained from commercial software, and an excellent agreement is achieved, yet an competitive advantage on simulation performances is observed by using the proposed technique.

PurposeTo investigate the deficits in contrast sensitivity in patients with Fuchs uveitis syndrome (FUS) and to explore the potential relationship between contrast sensitivity and ocular structure.MethodsIn this prospective study, 25 patients with FUS and 30 healthy volunteers were recruited. Eyes were divided into three groups: FUS-affected eyes (AE), fellow eyes (FE), and healthy eyes. The contrast sensitivity function (CSF) of all participants was evaluated using the quick CSF (qCSF) method. Fundus photographs were collected for the analysis of refractive media, and vascular density (VD) was assessed using optical coherence tomography angiography (OCTA). Data were analyzed and compared using the generalized estimating equation (GEE).ResultsThe CSF of AE was significantly lower than that of FE and controls, while no significant difference was observed between FE and controls. Contrast sensitivity was negatively correlated with the grade of haze. No significant correlation was found between visual function and VDs in FUS eyes.ConclusionsWe found that the CSF of FUS-affected eyes was significantly reduced, and the visual impairment was predominantly caused by the refractive media turbidity.

Tight sandstone reservoirs have smaller pore throats, complex structures, strong heterogeneity in the pore throat system, and significant differences in fluid distribution. To reveal the occurrence characteristics of movable fluids in tight sandstone reservoirs, the typical tight sandstone core samples from the Chang 4+5 reservoir of the Upper Triassic Yanchang Formation of the Ordos Basin were selected and the micro pore throat classification standard was established by using casting thin slices, field emission scanning electron microscopy (FE-SEM), high-pressure mercury injection (HPMI), and nuclear magnetic resonance (NMR) technology. The test analysis results showed that the Chang 4+5 tight sandstone reservoir can be divided into Type I, II, and III reservoirs by the different pore throat structure. Type I reservoirs have well-developed pore throats, good connectivity, low threshold pressure (0.42 MPa on average), high movable fluid saturation (46.82% on average), and a minimum pore throat radius of movable fluid (0.056 μm on average). The pore throat structure and fluid production degree of the other two reservoir types gradually deteriorated. Note that the tight sandstone reservoirs of the Chang 4+5 reservoirs have smaller pore throats but high movable fluid content in smaller pore throats (17% on average). With the deterioration of the pore throat structure in reservoir, the degree of fluid utilization in larger pore throats decreases (74.33%–57.33%), whereas the degree of fluid utilization in smaller pore throats does not change significantly (25.67%–13.82%). Many factors affect movable fluid parameters. The movable fluid parameters of tight sandstone reservoirs in the Chang 4+5 reservoir have a good positive correlation with permeability (R2 = 0.85) and sorting coefficients (R2 = 0.88). The movable fluid parameters of smaller and larger pore throats have no obvious correlation with a single factor. Multiple factors affect the fluid occurrence characteristics of different scale pore throats.

BackgroundAlpha-thalassemia/intellectual disability syndrome (ATR-X) (OMIM # 301040) was first described by Wilkie et al. (1). Several studies found that children who presented with significantly consistent clinical phenotypes of hemoglobin H (Hb H) disease and profound mental handicap carried ATRX chromatin remodeler (ATRX, OMIM*300032) gene variants. With the recent development of exome sequencing (ES), ATRX gene variants of severe to profound intellectual disability without alpha-thalassemia have been implicated in intellectual disability-hypotonic facies syndrome, X-linked, 1(MRXHF1, OMIM #309580). These two diseases present similar clinical manifestations and the same pattern of inheritance.Case PresentationWe reported a 3-year-old boy with intellectual disability, language impairment, hypotonia, and mild craniofacial abnormalities (flat nasal bridge, small and triangular nose, anteverted nostrils, and widely spaced incisors) and reviewed MRXHF1 cases. At an early stage, the patient developed global developmental delay (GDD). After 6 months of rehabilitation therapy, the patient's motor ability did not make big progress, as well as his speech or nonverbal communication. We performed whole-genome sequencing (WGS), Sanger sequencing, reverse transcription-polymerase chain reaction (RT-PCR), and X-inactivation studies. A novel hemizygous intronic variant in ATRX (c.5786+4A>G; NM_000489.6) was identified, which led to exon 24 skipping. The carrier mother showed extremely skewed X-chromosome inactivation (XCI). These results may contribute to the patient's phenotypes.ConclusionsThe novel hemizygous intronic variant in ATRX is the genetic etiology of the boy. Identification of this variant is helpful for parents to take prenatal diagnostic tests. Also, this new case expands the phenotypes of MRXHF1 and the mutational spectrum of the ATRX gene.

RimK-like family member B (RIMKLB) is an enzyme that post-translationally modulates ribosomal protein S6, which can affect the development of immune cells. Some studies have suggested its role in tumor progression. However, the relationships among RIMKLB expression, survival outcomes, and tumor-infiltrating immune cells (TIICs) in colorectal cancer (CRC) are still unknown. Therefore, we analyzed RIMKLB expression levels in CRC and normal tissues and investigated the correlations between RIMKLB and TIICs as well as the impact of RIMKLB expression on clinical prognosis in CRC using multiple databases, including the Tumor Immune Estimation Resource (TIMER), Gene Expression Profiling Interactive Analysis (GEPIA), PrognoScan, and UALCAN databases. Enrichment analysis was conducted with the cluster Profiler package in R software to explore the RIMKLB-related biological processes involved in CRC. The RIMKLB expression was significantly decreased in CRC compared to normal tissues, and correlated with histology, stage, lymphatic metastasis, and tumor status (p < 0.05). Patients with CRC with high expression of RIMKLB showed poorer overall survival (OS) (HR = 2.5,p = 0.00,042), and inferior disease-free survival (DFS) (HR = 1.9,p = 0.19) than those with low expression of RIMKLB. TIMER analysis indicated that RIMKLB transcription was closely related with several TIICs, including CD4+ and CD8+ T cells, B cells, tumor-associated macrophages (TAMs), monocytes, neutrophils, natural killer cells, dendritic cells, and subsets of T cells. Moreover, the expression of RIMKLB showed significant positive correlations with infiltrating levels of PD1 (r = 0.223, p = 1.31e-06; r = 0.249, p = 1.25e-03), PDL1 (r = 0.223, p = 6.03e-07; r = 0.41, p = 5.45e-08), and CTLA4 (r = 0.325, p = 9.68e-13; r = 0.41, p = 5.45e-08) in colon and rectum cancer, respectively. Enrichment analysis showed that the RIMKLB expression was positively related to extracellular matrix and immune inflammation-related pathways. In conclusion, RIMKLB expression is associated with survival outcomes and TIICs levels in patients with CRC, and therefore, might be a potential novel prognostic biomarker that reflects the immune infiltration status.

Improving grain filling in the presernt farming systems is crucial where grain filling is a concern due to the extreme use of chemical fertilizers (CF). A field experiment was conducted at the experimental station of Guangxi University, China in 2019 to test the hypothesis that cattle manure (CM) and poultry manure (PM) combined with CF could improve rice grain filling rate, yield, biochemical and qualitative attributes. A total of six treatments, i.e., no fertilizer (T1), 100% CF (T2), 60% CM + 40% CF (T3), 30% CM + 70% CF (T4), 60% PM + 40% CF (T5), and 30% PM + 70% CF (T6) were used in this study. Results showed that the combined treatment T6increased starch metabolizing enzymes activity (SMEs), such as ADP-glucose phosphorylase (ADGPase) by 8 and 12%, soluble starch synthase (SSS) by 7 and 10%, granule bound starch synthesis (GBSS) by 7 and 9%, and starch branching enzyme (SBE) by 14 and 21% in the early and late seasons, respectively, compared with T2. Similarly, higher rice grain yield, grain filling rate, starch, and amylose content were also recorded in combined treatments. In terms of seasons, higher activity of SMEs , grain starch, and amylose content was noted in the late-season compared to the early season. The increment in these traits was mainly attributed to a lower temperature in the late season during the grain filling period. Furthermore, our results suggested that an increment in starch accumulation and grain filling rate were mainly associated with the enhanced sink capacity by regulating key enzyme activities involved in Suc-to-starch conversion. In-addition, RT-qPCR analysis showed higher expression levels of AGPS2b, SSS1, GBSS1, and GBSE11b genes, which resultantly increased the activities of SMEs during the grain filling period under combined treatments. Linear regression analysis revealed that the activity of ADGPase, SSS, GBSS, and SBE were highly positively correlated with starch and amylose accumulation. Thus, we concluded that a combination of 30% N from PM or CM with 70% N from CF is a promising option in terms of improving rice grain yield and quality. Our study provides a sustainable fertilizer management strategy to enhance rice grain yield and quality at the lowest environmental cost.

Abstract For the purpose of assessing the radiation dose of the victims involved in the nuclear emergency or radiation accident, a new type of X-band EPR resonant cavity for in vivo fingernail EPR dosimetry was designed and a homemade EPR spectrometer for in vivo fingernail detection was constructed. The microwave resonant mode of the cavity was rectangular TE101, and there was a narrow aperture for fingernail detection opened on the cavity’s wall at the position of high detection sensitivity. The DPPH dot sample and the fingernail samples were measured based on the in vivo fingernail EPR spectrometer. The measurements of the DPPH dot sample verified the preliminary functional applicable of the EPR spectrometer and illustrated the microwave power and modulation response features. The fingernails after irradiation by gamma-ray were measured and the radiation-induced signal was acquired. The results indicated that the cavity and the in vivo EPR dosimeter instrument was able to detect the radiation-induced signal in irradiated fingernail, and preliminarily verified the basic function of the instrument and its potential for emergency dose estimate after a radiation accident.

Abstract A novel design for a TE10‐mode rectangular waveguide to a TE11‐mode circular waveguide mode converter based on the use of an intermediate one‐step double‐cruciform section is proposed. The converter achieves compactness and broadband by employing the one‐step double‐cruciform transition instead of the conventional multi‐step transitions or smooth transitions. Due to the one‐step structure, the converter achieves an extremely short‐length (around 0.3λ, where λ is the wavelength at the centre frequency), which makes it suitable for highly compact systems. For verification purpose, a back‐to‐back connection rectangular‐to‐circular mode converter was fabricated and measured. The measured results show that the return loss is around 15 dB and the insertion loss is around 0.4 dB in the operating frequency band. Thus, we can infer that the return loss and insertion loss of a single mode converter are around 19 and 0.2 dB, respectively. The experimental results reasonably agree with the simulated results.

In this paper, a class of single-layered multifunctional leaky-wave antennas (LWAs) with flexibly engineered radiation and filtering characteristics are proposed and demonstrated for microwave and millimeter-wave applications. Radiating discontinuities (RDs) exhibiting multiple resonances while particularly possessing flexible model-control capability are exploited to accomplish such design freedoms and multifunctionalities of LWAs. By properly engineering the resonance characteristics of RDs under the mode-control principle, the attenuation constant of relevant LWAs can not only be freely tailored for diverse beamwidth/directivity requirements, but also simultaneously maintain a flat frequency response for radiation stability. Meanwhile, controllable filtering behaviors can be obtained as well by the LWAs thanks to the transmission zeros introduced by resonances. Consequently, both the radiation and filtering performances of LWAs can be adequately tailored by taking advantage of the mode-control capability of RDs. Under this design concept, two types of LWAs based on substrate-integrated waveguide and microstrip techniques are respectively developed for different system integration platforms. The substrate-integrated waveguide LWA whose unit cells consist of different longitudinal slots is firstly examined. Additionally, the microstrip LWA, which depends on stub-loaded resonators, is further investigated. The proposed two LWAs are all with flexible engineered electrical behaviors, single-layer, low-cost, and easy integration; they may be a potential candidate for various system applications such as 5G communication and Internet of Vehicles.

Hyperspectral anomaly detection involves in many practical applications. Traditional anomaly detection methods are mainly proposed based on statistical models and geometrical models. This article proposes an Otsu-based isolation forest method, which applies the assumption that anomaly pixels are more sensitive to be isolated from the alternative pixels. The proposed article trains an isolation forest by assembling multiple binary trees. To construct a more discriminative binary tree, Otsu-based splitting criterion is applied to split subsamples into two groups at each division. Then, it feeds each tested pixel into isolation forest and obtains its anomaly score via the average path length throughout isolation forest. Considering the pixels with anomaly attribute values, path length refinement strategy based on distance weight is applied to better distinguish anomaly scores of tested pixels. Experimental results on three datasets reveal that the proposed method can effectively separate anomalies from backgrounds compared with other anomaly detection methods.

This paper presents a general overview of substrate integrated transmission lines, from the perspective of historical background and progress of guided-wave structures and their impacts on the development of microwave circuits and integration solutions. This is highlighted through a technology roadmap involving the categorized five generations of microwave circuits. In particular, the substrate integration technologies are reviewed and discussed with focus on technical features, design highlights, component developments, structures evolution, and systems integration. A number of examples are presented to showcase some of the selected milestone research and development activities and accomplishments in connection with substrate integrated transmission line technologies, with particular focus on substrate integrated waveguide (SIW) techniques. Practical applications and industrial interests are also presented with key references and technical results, which show more and more product developments in the end-user sectors. It can be found that the popularity of SIW techniques is closely related to the achieved seamless integration of planar and non-planar structures into a unified design space, thereby allowing the possibility of combining major advantages of all the structures while alleviating their potential drawbacks. The future perspectives of the substrate integration technology are discussed through five major research directions, which suggest potential impacts in the development of future generations of circuits and systems such as system-on-substrate (SoS).

A composite cavity-based antenna is proposed and presented in this work. Three kinds of modes of the composite structure are involved to obtain a wide bandwidth: the ${\mathrm {TE}}_{11 {\scriptstyle {}^{\scriptstyle 1}}\hspace {-0.224em}/\hspace {-0.112em}{\scriptstyle 2} }$ cavity mode of a cylindrical open-ended substrate integrated waveguide cavity, the odd TEM mode generated by loading two rectangular via holes inside the cavity, and the higher-order loop modes (L3 and L5) of a circular ring detached from the cavity by a circumferential slot located at the cavity’s side wall. In the implementation of the antenna, slot coupling feeding methodology is applied, which also introduces a resonance for the bandwidth enhancement. A simulated fractional impedance bandwidth of 57.3% with $|{\mathrm{ S}}_{11}| < -10$ dB from 24.9 GHz to 44.9 GHz is achieved ( $|{\mathrm{ S}}_{11}|= -9.7$ dB at $f=27.7$ GHz is ignored). The antenna has a broadside radiation pattern within the operating frequency band and a maximum simulated broadside gain of 10.6 dBi at ${f}=27$ GHz. Multi-layer technique and metallized via holes processing, which become commercial nowadays, are two critical techniques of the presented antenna. The simple structure is benefit of the fabrication and the integration with other planar front-end circuits. The antenna is fabricated and experimentally verified. A small difference is observed between the measurement and the simulation. A measured fractional bandwidth of 52% from 27 GHz to 46 GHz ( $|{\mathrm{ S}}_{11}|= -9$ dB at ${f}=29$ GHz is ignored) and a measured maximum broadside gain of 9.5 dBi are achieved.