Search

Background: Hereditary angioedema (HAE) is a rare autosomal dominant genetic disease characterized by recurrent edema and a potentially fatal risk. Despite its severity, there is a notable lack of effective methods for predicting and preventing HAE attacks. This study aims to thoroughly investigate the underlying pathological mechanisms of HAE and identify potential biomarkers that could aid in its prediction and prevention. Results: In our investigation, we have discovered a novel pathogenic variant of the SERPING1 gene, specifically c.708T > G, in a Han family affected by HAE. Our observations indicate that this variant leads to an increase in the accumulation of C1-INH within the endoplasmic reticulum (ER), resulting in the upregulation of GRP75 protein expression. This cascade of events resulted in Ca2+ overload, disruption of mitochondrial structure and function, and eventually triggered apoptosis. Using siRNA to knock down GRP75 mitigates cellular calcium overload and mitochondrial damage induced by the SERPING1 mutation. Conclusion: Based on our findings, we propose that the detection of intracellular Ca2+ concentration could serve as a valuable biomarker for predicting acute attacks of HAE in patients. This discovery holds significant implications for the development of more targeted and effective strategies in the management of HAE. [ABSTRACT FROM AUTHOR]

The construction industry plays a pivotal role in China's achievement of its "dual carbon" goals. This study conducts a decomposition analysis of the carbon emissions from the construction industry (CECI) at both national and provincial levels for the period 2010–2020 and employs the ARIMA model to predict the short-term peak trends at the provincial level. The findings are as follows. (1) Inner Mongolia, Shandong, Sichuan, and Chongqing exhibit an N-shaped trend in CECI, while the northeast region shows an inverted U-shaped trend. (2) Labor productivity and energy intensity are identified as the largest and smallest drivers of national CECI growth, respectively, with the driving force of the study's identified factors fluctuating between 1% and 60%. (3) Energy intensity significantly contributes to the growth of CECI in Tianjin and Zhejiang, while it aids in reducing CECI in western provinces. The "rebound effect" of building energy efficiency is particularly pronounced in provinces with strong resource endowments, such as Ningxia. (4) Between 2021 and 2025, CECI is predicted to decrease in the northern and economically developed provinces, while it is expected to increase in central and western provinces, with Heilongjiang, Shandong, Guangdong, Shanghai, and Shaanxi potentially reaching their peaks within the forecast period. The paper concludes with several recommendations. [ABSTRACT FROM AUTHOR]

Zhoushan Archipelago, the largest archipelago in China, harbors a significant tidal stream resource due to robust tidal currents coursing through numerous channels between its islands. Nevertheless, the tidal energy resource in the Zhoushan Archipelago has not been systematically characterized considering both topography and energy flow density. This study presents a modeling study aimed at characterizing the tidal energy resource in the whole Zhejiang coast, including Zhoushan Archipelago, employing a high-resolution tidal hydrodynamic model, i.e. MIKE 21. Through a comparison of the model output with observed data from two tidal stations and three current stations, the accuracy of the numerical model was confirmed. The study identified areas with robust currents and suitable water depths, both essential factors for the advancement of tidal current energy. The findings showed that many channels in Zhoushan Archipelago and Wenzhou Bay in Zhejiang experience the highest tidal power density, reaching nearly 5500 W/m2. Furthermore, the study estimated the theoretical tidal current energy production for the whole area, highlighting Loutou channel, Cezi channel, Taohuagang, and the channel between Xiushan island and Daishan island as regions with high potential. [ABSTRACT FROM AUTHOR]

Interprovincial disparities in carbon emissions from the construction industry (CECI) are an important challenge for future emissions reductions. Based on the CECI data of 30 provinces in China from 2010 to 2020, the interprovincial equity and decoupling of CECI were examined in this study. The conclusions are as follows: (1): The main CECI growth regions were the eastern Bohai Rim region and the Pearl River Delta region. Heilongjiang basically achieved a CECI carbon peak in 2016. (2) The three northeastern provinces and western provinces were the main high–high and low–low aggregation areas, respectively. The greatest degree of inequality was found in the western provinces. The inequality between the eastern and western areas was the largest, while the inequality between the central and western areas was the smallest. The inequality in CECI in the north–south region decreased year by year. (3) The decoupling status of Shandong and some western provinces has significantly worsened, while the decoupling status of Sichuan, Yunnan, and some eastern developed provinces has been improving. These conclusions will contribute to the improvement of regional emission reduction strategies. [ABSTRACT FROM AUTHOR]

Dexmedetomidine (Dex) has been used in surgery to improve patients' postoperative cognitive function. However, the role of Dex in stress-induced anxiety-like behaviors and cognitive impairment is still unclear. In this study, we tested the role of Dex in anxiety-like behavior and cognitive impairment induced by acute restrictive stress and analyzed the alterations of the intestinal flora to explore the possible mechanism. Behavioral and cognitive tests, including open field test, elevated plus-maze test, novel object recognition test, and Barnes maze test, were performed. Intestinal gut Microbe 16S rRNA sequencing was analyzed. We found that intraperitoneal injection of Dex significantly improved acute restrictive stress-induced anxiety-like behavior, recognition, and memory impairment. After habituation in the environment, mice (male, 8 weeks, 18–23 g) were randomly divided into a control group (control, N = 10), dexmedetomidine group (Dex, N = 10), AS with normal saline group (AS + NS, N = 10) and AS with dexmedetomidine group (AS + Dex, N = 10). By the analysis of intestinal flora, we found that acute stress caused intestinal flora disorder in mice. Dex intervention changed the composition of the intestinal flora of acute stress mice, stabilized the ecology of the intestinal flora, and significantly increased the levels of Blautia (A genus of anaerobic bacteria) and Coprobacillus. These findings suggest that Dex attenuates acute stress-impaired learning and memory in mice by maintaining the homeostasis of intestinal flora. [ABSTRACT FROM AUTHOR]

The future fusion reactor devices built by Institute of Plasma Physics, Chinese Academy of Science will be a compact burning plasma facility to fill the gap between ITER and the China Fusion Engineering Test Reactor, with the mission of studying the deuterium–tritium plasma in steady-state operation. The winding-package (WP) of its toroidal field coil is graded into high-field WP and low-field WP, and the high critical current density (J c) Nb3Sn strand will be applied to the high-field WP based on the current design. The thermo-magnetic instability is the main issue in the application of high- J c Nb3Sn strand, which may induce premature quenching in the low field regions. This issue can be improved by reducing the effective filament diameter (d eff) to suppress flux jumps and increasing the residual resistivity ratio (RRR) to enhance the release of heat generated by flux jumps. However, in the conductor manufacturing process, local plastic deformations (indentation) of strands can impact the sub-element layout and degrade the RRR of the strand, which would increase again the risk on instability. In this study, magnetization measurements and V – I tests were performed on samples with different indentation depths. The hysteresis loss and d eff of the indented sample was obtained from the test results. The impact of local plastic deformation on the thermo-magnetic instability of two types of Nb3Sn strand was confirmed by cross-sectional metallographic observation and quantitative microstructure analysis. It was shown that flux jumps were suppressed at indentation depths below 0.3 mm. Further indentation increase leads to severe flux jumps. [ABSTRACT FROM AUTHOR]