Desminopathy is a rare heritable cardiac and skeletal muscle disease caused by variants in the DES gene, which encodes the primary muscle-specific intermediate filament protein, known as desmin. Childhood-onset is commonly associated with severe early-onset myopathy and early death. Here, we reported an 11-year-old Chinese girl presenting with complete atrioventricular block and cardiomyopathy, without skeletal muscle involvement. Genetic analysis identified a de novo variant (c.152C > T/p.Ser51Phe) in the DES gene.

Milk fat synthesis is tightly regulated by hormones and growth factors. Leptin is a versatile peptide hormone that exerts pleiotropic effects on metabolic pathways. In this study, we evaluated the expression and function of leptin and its long form receptor OB-Rb in dairy cow mammary tissues from different physiological stages and in cultured mammary epithelial cells. The results showed that the expression of leptin and OB-Rb were significantly higher in the mammary tissues of lactating cows as compared with dry cows, suggesting that they are related to milk component synthesis. In cultured dairy cow mammary epithelial cells, leptin treatment significantly increased OB-Rb expression and intracellular triacylglycerol content. Transcriptome analysis identified the difference in gene expression between leptin treated cells and control cells, and 317 differentially expressed genes were identified. Gene ontology and pathway mapping showed that lipid metabolism-related gene expression increased and signal transduction pathway-related genes were the most significantly enriched. Mechanistic studies showed that leptin stimulation enhanced sterol regulatory element-binding protein 1 expression via activating the phosphatidylinositol 3-kinase/protein kinase B/mammalian target of rapamycin (PI3K/AKT/mTOR) signaling pathway, which in turn up-regulated the expression of genes related to milk fat synthesis. Moreover, we found that fatty acid synthesis precursors, acetate and β-hydroxybutyrate, could positively regulate the expression of leptin and OB-Rb in bovine mammary epithelial cells, thereby potentially increasing milk fat synthesis. Our study provided novel evidence in the regulation of leptin on milk fat production in mammary glands of dairy cows, as well as experimental basis for artificial regulation of milk fat

This paper investigates the use of English within the linguistic landscape of Luang Prabang through a combination of photographs and interviews. Specifically, we examine the characteristics of English in the linguistic landscape of Luang Prabang, the roles played by English, and the perceptions of merchants and consumers towards it. The study finds that: first, English is frequently used alongside Lao, particularly in bilingual signs, and it is also used alongside other languages within multilingual sign combinations. English predominantly appears in areas heavily frequented by consumers, where it is utilized for economic benefit. Second, English primarily fulfills the following roles in the linguistic landscape: cultural heritage bearer, tourism information provider, cultural exchange facilitator, economic opportunities creator, educational resource provider, and international image broadcaster. Third, shop owners and consumers generally express satisfaction with the presence of English in Luang Prabang's linguistic landscape, believing that English promotes economic development, cultural exchange, and brand establishment. However, some interviewees also highlight shortcomings, such as concerns over traditional culture and unfriendliness towards non-native English speakers. This study underscores the pivotal role of English in bridging cultural and economic divides in Luang Prabang, offering insights for policymakers on language policy and tourism management.

Determining the insdel distance of linear codes is a very challenging problem. Recently, Ji et al. [‘Strict half-Singleton bound, strict direct upper bound for linear insertion-deletion codes and optimal codes’, IEEE Trans. Inform. Theory 69(5) (2023), 2900–2910] proposed their strict half-Singleton bound for linear codes that do not include the all-one vector. A natural question asks for linear codes with both large Hamming distance and insdel distance. Almost maximum distance separable (MDS) codes are a special kind of linear code with good Hamming error-correcting capability. We present a sufficient condition for the insdel distance of almost MDS codes to be bounded by the strict half-Singleton bound. In addition, we construct a class of two-dimensional near MDS codes that achieve the strict half-Singleton bound using twisted Reed–Solomon codes. Finally, we present a construction of optimal almost MDS insdel codes from Reed–Solomon codes for large dimensions.

The effect of the polarizations of two counter-propagating relativistic laser pulses interacting with subwavelength thin solid-density foil is investigated. Three-dimensional particle-in-cell simulations and analytical modelling show that the interaction and resulting transverse instability depend strongly on the polarization directions as well as the intensity distribution of the resultant light field in the foil. The left- and right-handed circularly polarized laser pair with the same phase at the common focal spot in the ultrathin foil leads to the strongest distortion of the foil. The fastest growing mode and maximum growth rate depend mainly on the laser intensity. For all polarization and phase-difference combinations, the instability is weakest when the two laser pulses are exactly out of phase at the common focusing point in the foil.

Bronze mou vessels appear in Shu tombs in south-west China during the Eastern Zhou period (c. 771–256 BC). Examination of these vessels reveals major changes in the supply of metal and alloying technology in the Shu State, throwing new light on the social impact of the Qin conquest and later unification of China.

The previous study indicated that ubiquitination is involved in the freezing tolerance of hydrated seeds. Parthenolide (PN), inducing the ubiquitination of MDM2, an E3 ring-finger ubiquitin ligase, adversely affects the freezing tolerance of hydrated lettuce seeds. Therefore, a proteomics analysis was conducted to identify PN's targets in hydrated seeds exposed to cooling conditions. Several pathways, including oxidative phosphorylation (KEGG00190), amino sugar and nucleotide sugar metabolism (KEGG00520), and biosynthesis of nucleotide sugars (KEGG01250), were enriched in the PN treatment under slow-cooling conditions (3°C h−1, P < 0.05). Among the proteins in oxidative phosphorylation, the expression of NADH dehydrogenases and ATP synthases (ATPsyn) decreased in PN treatment. In contrast, uncoupling proteins increased after PN treatment, which led to the dissociation of the electron transport chain from ATP synthesis. Treatments with rotenone, dicoumarol, and oligomycin (i.e., oxidative phosphorylation inhibitors) decreased the survival rate of hydrated seeds under freezing conditions, which indicated that energy metabolism was related to the freezing tolerance of hydrated seeds. The predicted interactions between PN and MDM2-like proteins of Lactuca indicated that LsMDM2-5 forms two potential hydrogen bonds with PN. Furthermore, based on AlphaFold predictions and yeast 2-hybrid results, MDM2-5 might interact directly with NADH2. The knockdown of MDM2-5 by RNAi caused a higher level of NADH2 and ATPsyn and a higher freezing tolerance of hydrated seeds. This indicated that MDM2 played negative roles in regulating ATP synthesis and freezing tolerance of hydrated seeds.

Rhopalosiphum padi is an important grain pest, causing severe losses during crop production. As a systemic insecticide, flonicamid can control piercing-sucking pests efficiently. In our study, the lethal effects of flonicamid on the biological traits of R. padi were investigated via a life table approach. Flonicamid is highly efficiently toxic to R. padi, with an LC50 of 9.068 mg L−1. The adult longevity and fecundity of the R. padi F0 generation were markedly reduced under the LC25 and LC50 concentrations of flonicamid exposure. In addition, negative transgenerational effects on R. padi were observed under exposure to lethal concentrations of flonicamid, with noticeable decreases in the reproductive period, adult longevity, total longevity, and total fecundity of the F1 generation under the LC25 concentration of flonicamid. Furthermore, the third nymph stage (N3), preadult stage, duration of the adult pre-reproductive period, duration of the total pre-reproductive period, reproductive period, adult longevity, total longevity, and total fecundity of the F1 generation were significantly lower under treatment with the LC50 concentration of flonicamid. The life table parameters were subsequently analysed, revealing that the intrinsic rate of increase (rm) and the net reproductive rate (R0) were significantly lower but that the finite rate of increase (λ) and the mean generation time (T) were not significantly different under the LC25 and LC50 concentrations of flonicamid. These data are beneficial for grain aphid control and are critical for exploring the role of flonicamid in the integrated management of this key pest.

Using the syntactic priming paradigm, this study investigated abstract syntactic knowledge of Chinese transitive structures (i.e., subject-verb-object [SVO], BA, and BEI) in deaf children with cochlear implants (CIs). Specifically, we focused on the differences in the development of various syntactic structures (within CI children and compared with their typically hearing children) and the possible individual differences during this process. Results showed that both CI and hearing children exhibited structural priming for all syntactic structures (i.e., SVO, SbaOV structure [agent-patient ordering], and ObeiSV structure [patient-agent ordering]) after comprehending and repeating the prime sentence regardless of verb repetition. However, verb repetition induced an intense abstract priming effect in CI children but not hearing children, with the lexical boost effect more significant for SVO and BA structures. In addition, CI children’s working memory capability modulated the production of the BA structure but not SVO and BEI structures.

This paper considers the guidance issue for attackers against aircraft with active defense in a two-on-two engagement, which includes an attacker, a protector, a defender and a target. A cooperative line-of-sight guidance scheme with prescribed performance and input saturation is proposed utilising the sliding mode control and line-of-sight guidance theories, which guarantees that the attacker is able to capture the target with the assistance of the protector remaining on the line-of-sight between the defender and the attacker in order to intercept the defender. A fixed-time prescribed performance function and first-order anti-saturation auxiliary variable are designed in the game guidance strategy to constrain the overshoot of the guidance variable and satisfy the requirement of an overload manoeuver. The proposed guidance strategy alleviates the influence of external disturbance by implementing a fixed-time observer and the chattering phenomenon caused by the sign function. Finally, nonlinear numerical simulations verify the cooperative guidance strategies.

With the escalating laser peak power, modulating and detecting the intensity, duration, phase and polarization of ultra-intense laser pulses progressively becomes increasingly arduous due to the limited damage thresholds of conventional optical components. In particular, the generation and detection of ultra-intense vortex lasers pose great challenges for current laser technologies, which has limited the widely potential applications of relativistic vortex lasers in various domains. In this study, we propose to reconstruct the vortex phase and generate and amplify the relativistic vortex lasers via surface plasma holograms (SPHs). By interfering with the object laser and reference laser, SPHs are formed on the target and the phase of the interfering laser is imprinted through the modulation of surface plasma density. In particular, using the quadrature phase-shift interference, the vortex phase of the object laser can be well reconstructed. The generated vortex lasers can be focused and enhanced further by one order of magnitude, up to $1.7\times {10}^{21}$ W/cm${}^2$, which has been demonstrated by full three-dimensional particle-in-cell simulations. For the first time, we provide a practical way to detect the phase of relativistic vortex lasers, which can be applied in large 1–10 PW laser facilities. This will promote future experimental research of vortex-laser–plasma interaction and open a new avenue of plasma optics in the ultra-relativistic regime.

Traditional bulky and complex control devices such as remote control and ground station cannot meet the requirement of fast and flexible control of unmanned aerial vehicles (UAVs) in complex environments. Therefore, a data glove based on multi-sensor fusion is designed in this paper. In order to achieve the goal of gesture control of UAVs, the method can accurately recognize various gestures and convert them into corresponding UAV control commands. First, the wireless data glove fuses flexible fiber optic sensors and inertial sensors to construct a gesture dataset. Then, the trained neural network model is deployed to the STM32 microcontroller-based data glove for real-time gesture recognition, in which the convolutional neural network-Attention mechanism (CNN-Attention) network is used for static gesture recognition, and the convolutional neural network-bidirectional long and short-term memory (CNN-Bi-LSTM) network is used for dynamic gesture recognition. Finally, the gestures are converted into control commands and sent to the vehicle terminal to control the UAV. Through the UAV simulation test on the simulation platform, the average recognition accuracy of 32 static gestures reaches 99.7%, and the average recognition accuracy of 13 dynamic gestures reaches 99.9%, which indicates that the system’s gesture recognition effect is perfect. The task test in the scene constructed in the real environment shows that the UAV can respond to the gestures quickly, and the method proposed in this paper can realize the real-time stable control of the UAV on the terminal side.

This study employs direct numerical simulations to examine the effects of varying backpressure conditions on the turbulent atomisation of impinging liquid jets. Using the incompressible Navier–Stokes equations, and a volume-of-fluid approach enhanced by adaptive mesh refinement and an isoface-based interface reconstruction algorithm, we analyse spray characteristics in the environments with ambient gas densities ranging from 1 to 40 times the atmospheric pressure under five different backpressure scenarios. We investigate the behaviour of turbulent jets, incorporate realistic orifice geometries and identify significant variations in the atomisation patterns depending on backpressure. Two distinct atomisation types emerge, namely jet-sheet-ligament-droplet at lower backpressures and jet-sheet-fragment-droplet at higher ones, alongside a transition from dilute to dense spray patterns. This variation affects the droplet size distribution and spray dynamics, with increased backpressure reducing the spray's spreading angle and breakup length, while increasing the droplet size variation. Furthermore, these conditions promote distributions that induce rapid, nonlinear wavy motion in liquid sheets. Topological analysis of the atomisation field using velocity-gradient tensor invariants reveals significant variations in topology volume fractions across different regions. Downstream, the droplet Sauter mean diameter increases and then stabilises, reflecting the continuous breakup and coalescence processes, notably under higher backpressures. This research underscores the substantial impact of backpressure on impinging-jet atomisation and provides essential insights for nozzle design to optimise droplet distributions.