Search

Introduction: Loeys‐Dietz syndrome (LDS) is a rare autosomal dominant disorder with extensive connective tissue involvement. The diagnosis of this disease is mainly based on clinical features combined with the detection of pathogenic gene mutations, mainly mutations in the transforming growth factor-beta (TGF-β) signaling pathway. Methods: The molecular pathogenesis of a LDS syndrome proband and his family members was analyzed using whole exome sequencing and validated using Sanger sequencing. Molecular dynamics simulations and in vitro cell experiments further analyzed the structural changes and functional abnormalities of the variation. Results: This study describes the case of a 6-month-old infant diagnosed with LDS with typical craniofacial abnormalities, developmental delay, and a dilated aortic sinus (19 mm; Z-score 3.5). Genetic analysis showed the patient carried a novel de novo TGF-β receptor 2 (TGFBR2) mutation (NM_003242: c.1005_1007delGTA (p.Glu335_Tyr336delinsAsp)). Molecular dynamics simulation showed that the TGFBR2 c.1005_1007delGTA mutation changed the protein conformation, making the protein conformation more stable. The p.Glu335_Tyr336delinsAsp mutation significantly reduced TGF-β-induced gene transcription and phosphorylation of SMAD Family Member 2 (SMAD2) in vitro. Conclusions: Our comprehensive genetic analysis suggested that the p.Glu335_Tyr336delinsAsp variant of TGFBR2 caused aberrant TGF-β signaling and contributed to LDS in the patient.

Background and objectivesLeft-behind children are characterized by prolonged separation from one or both parents, which exposes them to a constant lack of good parenting, leading to increased risk of internalizing and (or) externalizing problems. This study explored the effects of parental rejection on internalizing and externalizing problems, and examined the moderating role of interpersonal harmony in class.MethodsThe sample comprised 3,473 left-behind children (aged 6 ~ 15; 54.1% girls) in a rural area of southwest China. Self-reported measures including Parental Acceptance-Rejection Questionnaire, Class Interpersonal Harmony Questionnaire and Child Behavior Scale were administrated. Regression analysis was employed and Hayes PROCESS macro was utilized to test the moderation effect.ResultsThe analysis showed that parental rejection had a significant predictive effect on both internalizing problems (β = 0.33, p

IntroductionAdolescent mental health has been an issue of global concern, and the mental health of adolescents in economically disadvantaged areas may require additional research. The research delves into factors associated with economically disadvantaged areas’ adolescent mental health, employing family systems theory, self-determination theory, and psychological reactance theory. Specifically, the present study which was done in Jingzhou country, an economically disadvantaged area of Hunan Province, China, aimed to examine the link between parental psychological control and adolescent mental health, as well as the mediating roles of psychological reactance and problematic smartphone use in this relationship.MethodsA sample of 1300 Chinese middle school students (620 girls, mean age = 14.22 ± 1.29) completed self-reported measures of parental psychological control, psychological reactance, problematic smartphone use, and adolescent mental health.ResultsResults showed that parental psychological control was negatively associated with adolescent mental health. Psychological reactance and problematic smartphone use mediated the association between parental psychological control and adolescent mental health, separately and serially.DiscussionThe findings of the present study enrich the literature on parenting styles and adolescent mental health in economically disadvantaged areas, and this provides an intervention perspective to reduce the negative impact of poor parenting on adolescent mental health in economically disadvantaged areas.

In order to explore the quality changes of grass carp visceral fish oil under different storage temperatures, the grass carp visceral fish oil was extracted by enzymatic method. The visceral fish oil of grass carp was stored at cold storage (4 ℃), normal temperature (25 ℃) and 37 ℃ for 60 days at constant temperature, and its acid value and peroxide value were determined regularly. Fingerprint characteristic analysis, principal component analysis (PCA) and linear discriminant analysis (LDA) and load analysis and other electronic nose technologies regularly were used to analyze the changes of volatile components. The results showed that the changes of acid value, peroxide value and volatile odor components of grass carp viscera were not obvious during storage at low temperature (4 ℃); The acid value and peroxide value of grass carp visceral crude oil increased significantly at room temperature (25 ℃) and 37 ℃, and the volatile odor components changed significantly, especially at 37 ℃; Taking the acid value and peroxide value as the criteria for judging whether the fish oil was deteriorated, the shelf life was about 55 days at 4 ℃, 40 days at 25 ℃ and 30 days at 37 ℃; The volatile components of fish oil during storage were mainly nitrogen oxides, sulfides and aromatic components. This study provides a theoretical basis for the effective utilization of grass carp viscera.

As a set of new technologies to enhance life-cycle construction management processes, Building Information Modelling (BIM) has been introduced to China’s Architecture, Engineering, and Construction (AEC) industries. Adopting the framework of Technology-Organization-Environment (TOE) and the theory of innovation diffusion, this study provides an empirical analysis of BIM adoption and its impact on corporation performance, using a time series panel sample of 1109 observations of AEC enterprises listed in China’s A-share stock market. This study reveals that the decision of BIM adoption could be driven by corporate innovation strategy and government policy pressure. Overall, BIM implementation brings positive financial performance to the BIM adopted enterprises, but the differences of BIM performance between innovation strategy-led and policy-driven adoption are only significant in enterprises of state-controlled. This paper contributes to the body of knowledge in enriching the TOE framework by adding innovation strategy motivations in the organization context and government policy forces to the environment context. In addition, the findings of this paper might help policy makers around the world improve the acceptance of BIM technology and evaluate the effects of BIM policy.

Background: Chronic kidney disease (CKD) with known valve calcification (VC) places individuals at high risk of cardiovascular disease. The study of VC in CKD is challenging due to the lack of a suitable research model. Here, we established a rat model of multivalve calcification induced by subtotal nephrectomy and a high-phosphate (HP) diet and analyzed the valve characteristics. Methods: We established a CKD model in Sprague-Dawley rats by performing 5/6 nephrectomy (5/6Nx) followed by feeding with chow containing different phosphate concentrations for 8, 12, or 16 weeks. The rats were divided into 4 groups: sham+normal phosphate (NP, 0.9% P), sham+high phosphate (HP, 2.0% P), 5/6Nx+NP, and 5/6Nx+HP. Serum creatinine (Scr), blood urea nitrogen (BUN), parathyroid hormone (PTH), calcium, phosphorus, and 24-h urine protein levels were investigated. Pathological examinations included histological characterization, safranin staining, Alcian blue staining, and von Kossa staining at different time points. Using nanoanalytical electron microscopy, we examined valves from rats in the 5/6Nx+HP and sham+HP groups and detected spherical particles using energy-dispersive spectroscopy (EDS) to observe microscopic changes in the valves. In addition, the calcified tissues were analyzed for phase and crystallization properties using an X-ray powder diffractometer. Results: The rats in the 5/6Nx+HP and 5/6Nx+NP groups presented with increased levels of Scr, BUN, and 24-h urine protein compared with those of the rats in the sham+HP and sham+NP groups. High levels of PTH were observed, and hematoxylin and eosin staining and immunohistochemistry for proliferating cell nuclear antigen showed parathyroid hyperplasia in rats in the 5/6Nx+HP group but not in the 5/6Nx+NP group. In rats in the 5/6Nx+HP group, extracellular matrix glycosylation was observed in the aortic valve in the 12th week and the mitral valve in the 16th week. In the 16th week, chondrocytes appeared in the aortic valve, as confirmed by immunofluorescence and Western blotting. Calcified particles mainly composed of phosphorus and calcium were observed in both the aortic and mitral valves by transmission electron microscopy and scanning electron microscopy (SEM). The main mineral component of the calcified aortic valve particles was hydroxyapatite [Ca5(PO4)3(OH)], as shown by X-ray diffraction. However, there were no obvious differences in heart function between rats in the 5/6Nx+HP and sham+HP groups. Conclusions: Our findings demonstrate that multivalve calcification is involved in CKD following 16-week HP and that hydroxyapatite [Ca5(PO4)3(OH)] is the main component of the calcified aortic valve particles of rats in the 5/6Nx+HP group.

Abstract Background Theoretically, paralogous genes generated through whole genome duplications should share identical expression levels due to their identical sequences and chromatin environments. However, functional divergences and expression differences have arisen due to selective pressures throughout evolution. A comprehensive investigation of the expression patterns of paralogous gene pairs in response to various stresses and a study of correlations between the expression levels and sequence divergences of the paralogs are needed. Results In this study, we analyzed the expression patterns of paralogous genes under different types of stress and investigated the correlations between the expression levels and sequence divergences of the paralogs. We analyzed the differential expression patterns of the paralogs under four different types of stress (drought, cold, infection, and herbivory) and classified them into three main types according to their expression patterns. We then further analyzed the differential expression patterns under various degrees of stress and constructed corresponding co-expression networks of differentially expressed paralogs and transcription factors. Finally, we investigated the correlations between the expression levels and sequence divergences of the paralogs and identified positive correlations between expression level and sequence divergence. With regard to sequence divergence, we identified correlations between selective pressures and phylogenetic relationships. Conclusions These results shed light on differential expression patterns of paralogs in response to environmental stresses and are helpful for understanding the relationships between expression levels and sequences divergences.

Select 180 primary school students from a city primary school in Shanghai, a developed area in eastern China, and 146 primary school students from a rural primary school in Jingzhou, a centrally underdeveloped area, as subjects. The method of scale is used to explore the influence of family socioeconomic status on the emotional intelligence of primary school students, and the mediating role of parenting styles in this influence and the difference in this effect in the two regions. The results show that: (1) The socioeconomic status and emotional intelligence of primary school students in Jingzhou are significantly lower than those of primary school students in Shanghai. In terms of parenting style, the emotional warmth and understanding of the fathers and mothers of Jingzhou’s primary school students are both significantly lower than those of Shanghai’s primary school students; (2) the socioeconomic status and emotional intelligence of the primary school students in Jingzhou are significantly and positively correlated with the parents’ emotional warmth and understanding parenting styles, while the socioeconomic status and emotional intelligence of the primary school students in Shanghai are only significantly positively correlated with the father’s emotional warmth and understanding parenting style; and (3) parenting style has a mediating effect between family socioeconomic status and emotional intelligence, but this effect has regional differences. The specific performance is as follows: The parents’ emotional warmth and understanding parenting styles of the primary school students in Jingzhou play a partial mediating effect between the family socioeconomic status and emotional intelligence, while the Shanghai primary school students’ fathers’ emotional warmth and understanding parenting style plays a complete mediating effect in family socioeconomic status and emotional intelligence.

Based on Chinese and Arabic literature, this paper summarizes the spread of Laozi’s text and thought in the Arab world. The specific course of dissemination can be divided into two stages: from 1966 to 2000, the Dao De Jing was disseminated on the basis of indirect translations; from 2000 to today, the spread of the Dao De Jing in the Arab world reached a climax when the first direct translation was published. Laozi’s text and thought have gained acceptance and popularity largely because of their soothing function and similarity to the Sufi spirit found in Arab culture. The spread of Laozi’s text and thought in the Arab world presents three features: a long and friendly history of cultural exchange has served as a foundation; languages such as English and French have functioned as intermediaries; and similarities between Sufism and Daoism have provided points of connection.

In the beverage, food and drug industry, more and more machine vision systems are being used for the defect detection of Polyethylene Terephthalate (PET) bottle caps. In this paper, in order to address the result of cylindrical distortions that influence the subsequent defect detection in the imaging process, a very fast image stitching algorithm is proposed to generate a panorama planar image of the surface of PET bottle caps. Firstly, the three-dimensional model of the bottle cap is established. Secondly, the relative poses among the four cameras and the bottle cap in the three-dimensional space are calculated to obtain the mapping relationship between three-dimensional points on the side surface of the bottle cap and image pixels taken by the camera. Finally, the side images of the bottle cap are unfolded and stitched to generate a planar image. The experimental results demonstrate that the proposed algorithm unfolds the side images of the bottle cap correctly and very fast. The average unfolding and stitching time for 1.6-megapixel color caps image can reach almost 123.6 ms.

The COVID-19 pandemic has brought about significant influences to the world, including tunnel construction. Based on the analysis of 12 tunnel construction projects, this paper identifies the specific risk factors related to the COVID-19 pandemic, e.g., men, materials, machines, methods, social environment, and political epidemic prevention pressure. Among these risk factors, worker availability, site accessibility, shortage of construction materials, and inadequate epidemic prevention materials caused by the lockdown policy are the most fundamental challenges encountered by the projects. Social panic and epidemic prevention policy requirements are key issues needed to be addressed before the resumption of construction work. The special circumstances caused by the COVID-19 pandemic called for flexible project management and coordination skills to raise suitable and effective response strategies, while local governments make substantial contributions in solving the difficulties. Although these measures have resulted in higher project costs, their effectiveness in catching up with project schedules is worthy of recognition. The findings of this study enrich the risk categories of tunnel construction and the risk response strategies from the perspective of a global pandemic. It implies that future construction schemes including design, budget, supply chain, and project management should consider the possible influence of an epidemic.

Background RNA-binding proteins (RBPs) play important roles in cellular homeostasis by regulating the expression of thousands of transcripts, which have been reported to be involved in human tumorigenesis. Despite previous reports of the dysregulation of RBPs in cancers, the degree of dysregulation of RBPs in cancers and the intrinsic relevance between dysregulated RBPs and clinical TNM information remains unknown. Furthermore, the co-expressed networks of dysregulated RBPs with transcriptional factors and lncRNAs also require further investigation. Results Here, we firstly analyzed the deviations of expression levels of 1,542 RBPs from 20 cancer types and found that (1) RBPs are dysregulated in almost all 20 cancer types, especially in BLCA, COAD, READ, STAD, LUAD, LUSC and GBM with proportion of deviation larger than 300% compared with non-RBPs in normal tissues. (2) Up- and down-regulated RBPs also show opposed patterns of differential expression in cancers and normal tissues. In addition, down-regulated RBPs show a greater degree of dysregulated expression than up-regulated RBPs do. Secondly, we analyzed the intrinsic relevance between dysregulated RBPs and clinical TNM information and found that (3) Clinical TNM information for two cancer types—CHOL and KICH—is shown to be closely related to patterns of differentially expressed RBPs (DE RBPs) by co-expression cluster analysis. Thirdly, we identified ten key RBPs (seven down-regulated and three up-regulated) in CHOL and seven key RBPs (five down-regulated and two up-regulated) in KICH by analyzing co-expression correlation networks. Fourthly, we constructed the co-expression networks of key RBPs between 1,570 TFs and 4,147 lncRNAs for CHOL and KICH, respectively. Conclusions These results may provide an insight into the understanding of the functions of RBPs in human carcinogenesis. Furthermore, key RBPs and the co-expressed networks offer useful information for potential prognostic biomarkers and therapeutic targets for patients with cancers at the N and M stages in two cancer types CHOL and KICH.

The capillary action between two solid surfaces has drawn significant attention in micro-objects manipulation. The axisymmetric capillary bridges and capillary forces between a spherical concave gripper and a spherical particle are investigated in the present study. A numerical procedure based on a shooting method, which consists of double iterative loops, was employed to obtain the capillary bridge profile and bring the capillary force subject to a constant volume condition. Capillary bridge rupture was characterized using the parameters of the neck radius, pressure difference, half-filling angle, and capillary force. The effects of various parameters, such as the contact angle of the spherical concave gripper, the radius ratio, and the liquid bridge volume on the dimensionless capillary force, are discussed. The results show that the radius ratio has a significant influence on the dimensionless capillary force for the dimensionless liquid bridge volumes of 0.01, 0.05, and 0.1 when the radius ratio value is smaller than 10. The effectiveness of the theorical approach was verified using simulation model and experiments.