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Abstract Background Dentatorubral-pallidoluysian atrophy is a rare autosomal dominant neurodegenerative disease. It is a rare disease in the world. Therefore, sharing clinical encounters of this case can deepen global awareness and understanding of the disease. Case presentation The patient was a 34-year-old male of Han nationality who was unmarried. The patient was admitted owing to weakness of the left lower limb with walking instability for 2 months and aggravation for 1 month. There was no dizziness, headache, numbness of limbs, convulsions, nausea, vomiting, abdominal pain, ataxia, nausea, vomiting, or abdominal pain. No nausea, vomiting, diarrhea, abdominal distension, tinnitus, hearing loss, fever, cough, expectoration. Personal history: worked in Cambodia 5 years ago, worked in Dubai 3 years ago, engaged in computer work, smoking or drinking habits. The patient was unmarried. Family history: the mother had symptoms similar to walking unsteadily (undiagnosed). Positive signs include a wide-base gait with a rotatory nystagmus that jumps upward in both eyes. Bilateral finger-nose instability test was quasi-positive, rapid alternating test was negative, and eye closure tolerance test was positive. Tendon reflexes were active in both upper limbs and hyperreflexia in both lower limbs. Stability of the heel, knee, and tibia. Genetic testing showed that the number of repeats in the dentatorubral-pallidoluysian atrophy ATN1 gene was 18 and 62, and the (CAG)n repeat sequence in the ATN1 gene was abnormal, with a repeat number of 62, and the patient was a pathogenic variant. The patient was diagnosed with dentatorubral-pallidoluysian atrophy. Dentatorubral-pallidoluysian atrophy remains a progressive neurodegenerative disease with no effective treatment. At present, the proband is taking 5 mg of buspirone three times a day, which has been reported to improve the symptoms. The patient was followed up for 6 months after taking buspirone, and there was no significant improvement in the temporary symptoms. At present, there are few cases of dentatorubral-pallidoluysian atrophy, and the characteristics of nystagmus in this disease have not been proposed in the past. This case reported the unusual presentation of nystagmus. Conclusion Dentatorubral-pallidoluygur atrophy is a rare neurodegenerative disease with autosomal dominant inheritance. To the best of our knowledge, our present case report is the first case report of dentatorubral-pallidoluygur atrophy with specific nystagmus. We describe the special eye shake and its positive signs to increase dentatorubral-pallidoluysian atrophy clinical positive signs.

Abstract P2X receptors, a subfamily of ligand-gated ion channels activated by extracellular ATP, are implicated in various physiopathological processes, including inflammation, pain perception, and immune and respiratory regulations. Structural determinations using crystallography and cryo-EM have revealed that the extracellular three-dimensional architectures of different P2X subtypes across various species are remarkably identical, greatly advancing our understanding of P2X activation mechanisms. However, structural studies yield paradoxical architectures of the intracellular domain (ICD) of different subtypes (e.g., P2X3 and P2X7) at the apo state, and the role of the ICD in P2X functional regulation remains unclear. Here, we propose that the P2X3 receptor’s ICD has an apo state conformation similar to the open state but with a less tense architecture, containing allosteric sites that influence P2X3’s physiological and pathological roles. Using covalent occupancy, engineered disulfide bonds and voltage-clamp fluorometry, we suggested that the ICD can undergo coordinated motions with the transmembrane domain of P2X3, thereby facilitating channel activation. Additionally, we identified a novel P2X3 enhancer, PSFL77, and uncovered its potential allosteric site located in the 1α3β domain of the ICD. PSFL77 modulated pain perception in P2rx3 +/+, but not in P2rx3 −/−, mice, indicating that the 1α3β, a “tunable” region implicated in the regulation of P2X3 functions. Thus, when P2X3 is in its apo state, its ICD architecture is fairly ordered rather than an unstructured outward folding, enabling allosteric modulation of the signaling of P2X3 receptors.

P2X receptors (P2X1–7) are non-selective cation channels involved in many physiological activities such as synaptic transmission, immunological modulation, and cardiovascular function. These receptors share a conserved mechanism to sense extracellular ATP. TNP-ATP is an ATP derivative acting as a nonselective competitive P2X antagonist. Understanding how it occupies the orthosteric site in the absence of agonism may help reveal the key allostery during P2X gating. However, TNP-ATP/P2X complexes (TNP-ATP/human P2X3 (hP2X3) and TNP-ATP/chicken P2X7 (ckP2X7)) with distinct conformations and different mechanisms of action have been proposed. Whether these represent species and subtype variations or experimental differences remains unclear. Here, we show that a common mechanism of TNP-ATP recognition exists for the P2X family members by combining enhanced conformation sampling, engineered disulfide bond analysis, and covalent occupancy. In this model, the polar triphosphate moiety of TNP-ATP interacts with the orthosteric site, while its TNP-moiety is deeply embedded in the head and dorsal fin (DF) interface, creating a restrictive allostery in these two domains that results in a partly enlarged yet ion-impermeable pore. Similar results were obtained from multiple P2X subtypes of different species, including ckP2X7, hP2X3, rat P2X2 (rP2X2), and human P2X1 (hP2X1). Thus, TNP-ATP uses a common mechanism for P2X recognition and modulation by restricting the movements of the head and DF domains which are essential for P2X activation. This knowledge is applicable to the development of new P2X inhibitors.

Abstract The acoustic black hole (ABH) structure exhibits remarkable energy focalization above a given cut-on frequency, offering potential for broadband vibration suppression in structures. However, its energy focusing properties diminish significantly below this cut-on frequency. Therefore, it is crucial to enhance the vibration attenuation capabilities of ABH structures within the low frequency range. This study presents a numerical investigation into the impact of thin-walled structures with embedded ABHs and distributed dynamic vibration absorbers (DVAs) on low frequency broadband vibration reduction. Initially, the focusing characteristics of the ABH thin-walled structure is analyzed, aiding in the attached position of DVAs. Furthermore, the influence of the design parameters and attached position of DVA on the broadband damping effect of the structure is explored. The findings indicate that DVAs designed for low frequencies can achieve significant vibration attenuation across the entire frequency spectrum, including low frequencies, when installed at specific focusing positions. When compared to the position with the maximum vibration response, while the attenuation of the low frequency common amplitude value is slightly reduced, greater vibration attenuation across the entire frequency band is achieved. This research offers valuable insights into optimizing the integration of DVAs with ABHs in thin-walled structures for enhanced broadband vibration attenuation.

Abstract Star anise (Illicium verum), a valuable spice tree, faces significant threats from fungal diseases, particularly Alternaria leaf spot. This study investigates the potential of a soil-derived actinomycete strain, YG-5, as a biocontrol agent against Alternaria tenuissima, the causative pathogen on Alternaria leaf spot in star anise. Through comprehensive morphology, physiology, biochemistry, and genetic analyses, we identified the isolate as Streptomyces sp. YG-5. The strain exhibited broad-spectrum antimicrobial activity against several plant pathogens, with inhibition rates ranging between 36.47 to 80.34%. We systematically optimized the fermentation conditions for YG-5, including medium composition and cultivation parameters. The optimized process resulted in an 89.56% inhibition rate against A. tenuissima, a 14.72% improvement over non-optimized conditions. Notably, the antimicrobial compounds produced by YG-5 demonstrated stability across various temperatures, pH levels, and UV irradiation. In vivo efficacy trials showed promising results, with YG-5 fermentation broth reducing Alternaria leaf spot incidence on star anise leaves by 56.95%. These findings suggest that Streptomyces sp. YG-5 holds significant potential as a biocontrol agent against Alternaria leaf spot in star anise cultivation, offering a sustainable approach to disease management in this valuable crop.

In modern ship degaussing systems, degaussing windings are mainly distributed based on the shape of ship bulkhead, which is difficult to ensure the degaussing effect of magnetic induction intensity of unit winding of each degaussing winding. In order to solve this problem, this paper introduces a tilted correlation screening in high-dimensional variable filter, which splits and recombines the original coils, and re-divides the original degaussing sections, so as to improve the degaussing efficiency of each coil. Aiming at the problem of sparse parameter vectors and multiple collinearity in the calculation of degaussing current after winding restructuring, this paper proposes a slant correlation screening and partial ridge regression algorithm. Through simulation, when the threshold is 0.73 and 0.91, the algorithm reduces 10.08% and 17.59% respectively compared with the least square method, while the residual root mean square error decreases by 10.45% and 12.17%. The simulation results show that the degaussing effect is significantly improved after the algorithm is adopted.

Background Chronic atrophic gastritis (CAG) with intestinal metaplasia (IM) is an independent risk factor for gastric cancer. Long-term inflammation and oxidative stress response stimulate the physical and mental state of patients. Under the modern medical model, proton pump inhibitors and gastric mucosal protective agents are increasingly unable to meet the high drug resistance of patients. It is urgent to seek effective new Chinese medicine treatments and multiple methods to treat CAG and IM. Objective To evaluate the clinical efficacy and safety of modified Piwei Peiyuan Decoction combined with acupuncture in the treatment of CAG with IM. Methods From January 2022 to September 2023, 202 patients with CAG and IM diagnosed by gastroscopy and pathological examination in the Department of Spleen and Stomach, the Center for Preventive Treatment of Disease, the Second Affiliated Hospital of Anhui University of Traditional Chinese Medicine and the Third Affiliated Hospital of Beijing University of Traditional Chinese Medicine were selected. The patients were divided into control group (n=67), treatment group A (n=68) and treatment group B (n=67) by random number table method. All patients participated in 6 weeks of treatment: (1) Control group: aluminum magnesium suspension combined with folic acid tablets (3 times/d) ; (2) Treatment group A: to add and subtract syndrome types of Piwei Peiyuan Decoction (2 times/d) ; (3) Treatment group B: Piwei Peiyuan Decoction (the same as group A) combined with acupuncture (1 time/d), with Zusanli, Liangqiu, Gongsun, Neiguan, Zhongwan as the main points, according to the syndrome type selection of acupoints. Before and 6 weeks after treatment, OLGA, OLGIM staging, gastric mucosal pathological efficacy, gastric mucosal pathological score, clinical symptom score, PRO scale score, drug-related adverse events (AE) and adverse drug reactions (ADR) were recorded. Results A total of 192 patients completed the 6-week course of treatment (62 in the control group, 66 in the treatment group A, and 64 in the treatment group B). The effective rate of the control group was 48.39% (30/62), the effective rate of the treatment group A was 69.70% (46/66), and the effective rate of the treatment group B was 71.88% (46/64). There was a statistically significant difference in the effective rate among the three groups (χ2=9.144, P=0.01). After treatment, the gastric mucosal pathological score, clinical symptom score and PRO scale score in the three groups were lower than those in the same group before treatment (P0.05) . Conclusion The overall clinical efficacy of Piwei Peiyuan Decoction combined with acupuncture is better than that of aluminum magnesium suspension combined with folic acid tablets, which is better than that of traditional antacids and gastric mucosal protective agents.

Wind farm wakes have a significant impact on momentum and turbulence fluxes within the atmospheric boundary layer， thereby influencing the local climate and environment.Mesoscale numerical models incorporating wind farm parameterizations are powerful tools for studying the climate and environmental impacts of wind farms.In this study， the wind speed and turbulence kinetic energy profiles of the Fitch wind farm parameterization scheme in the WRF mesoscale model are evaluated in the turbine and wake regions using high-resolution Large Eddy Simulations （LES） as “true values” and a method based on the relation derived from classical momentum theory is proposed to correct the grid-inflow wind speed.The method takes into account the blocking effect caused by the grid equivalent thrust and the corrected wind speed is closer to the free-stream wind speed.Results show that the difference between the grid-inflow wind speed from the original Fitch parameterization scheme and the LES is significant and sensitive to the model horizontal resolution.The Fitch-new parameterization with corrected grid-inflow wind speed reduces the relative error in absolute value between grid-inflow and free-stream wind speed to less than 1% across different horizontal resolutions （1000 m， 500 m， and 250 m）.The spatially averaged thrust and output power are consistent with LES results.The Fitch-new parameterization improves the simulated wind speed deficit in the wake zone of wind turbine， especially in the grid with turbine under high resolution.Although the simulated increase in turbulent kinetic energy and its vertical distribution in the wake zone is improved compared to the original Fitch scheme， there are certain issues that require further investigation.

Abstract Aging and obesity pose significant threats to public health and are major contributors to muscle atrophy. The trends in muscle fiber types under these conditions and the transcriptional differences between different muscle fiber types remain unclear. Here, we demonstrate distinct responses of fast/glycolytic fibers and slow/oxidative fibers to aging and obesity. We found that in muscles dominated by oxidative fibers, the proportion of oxidative fibers remains unchanged during aging and obesity. However, in muscles dominated by glycolytic fibers, despite the low content of oxidative fibers, a significant decrease in proportion of oxidative fibers was observed. Consistently, our study uncovered that during aging and obesity, fast/glycolytic fibers specifically increased the expression of genes associated with muscle atrophy and inflammation, including Dkk3, Ccl8, Cxcl10, Cxcl13, Fbxo32, Depp1, and Chac1, while slow/oxidative fibers exhibit elevated expression of antioxidant protein Nqo-1 and downregulation of Tfrc. Additionally, we noted substantial differences in the expression of calcium-related signaling pathways between fast/glycolytic fibers and slow/oxidative fibers in response to aging and obesity. Treatment with a calcium channel inhibitor thapsigargin significantly increased the abundance of oxidative fibers. Our study provides additional evidence to support the transcriptomic differences in muscle fiber types under pathophysiological conditions, thereby establishing a theoretical basis for modulating muscle fiber types in disease treatment.