Search Results (5,652)

Ionic liquids (ILs) are salts that are liquid at or below 100 °C. They are composed entirely of ions and have unique properties like negligible vapor pressure, high thermal stability, and tunable structures. These characteristics make them a promising alternative to traditional, often volatile and toxic organic solvents in the petrochemical industry. They have broad applications in chemical and petrochemical industry processes. Ionic liquids may be applied in the following processes: desulfurization, benzene toluene xylene (BTX) separation, alkylation, and carbon capture units. Two different ionic liquid-based process configurations have been evaluated for BTX separation. It has been found that the process configuration working with 1-ethyl-3methylimidazolium tricyanomethanide ([emim][TCM]) reduces the energy costs and capital expenditures associated with the Morphylane process by 67 and 63%, respectively. It also reduces solvent costs, confirming it as a cleaner alternative. The hydrodesulfurization (HDS) process is operated under harsh conditions, such as high temperature and high pressure and the requirement of a noble catalyst and hydrogen. High-Temperature Hydrogen Attack (HTHA) failure occurs at high temperatures between the gaseous molecular hydrogen contained inside the steel pressure vessel and the carbon atoms located in the steel matrix or in carbides. Methane molecules are produced during this reaction. This phenomenon can consequently lead to a loss of mechanical properties due to surface decarburization and to the formation of defects caused by methane bubbles mainly located at grain boundaries. The application of ionic liquids (ILs) in oil refineries offers significant advantages, such as safety, environmental sustainability, and process efficiency, primarily by serving as versatile alternatives to hazardous traditional solvents and catalysts. Across BTX extraction, carbon capture, and desulfurization/HDS-adjacent service, the recurring barriers are high viscosity, difficult regeneration, solvent cost/inventory and uncertain long-term stability. Full article

Centrosomal protein 152 (CEP152) is a key regulator of centriole architecture and function, essential for proper cell division and polarity. Pathogenic variants in CEP152 cause Seckel syndrome (SCKL), a systemic disorder characterized by microcephalic primordial dwarfism. However, the mechanisms underlying its multi-organ manifestations remain poorly understood. To investigate this, we utilized two mouse models harboring patient-derived CEP152 variants, Cep152^W105*/K897* and Cep152^Q32P/Q32P. While our previous work focused on neurodevelopmental defects, here we systematically analyzed extra-neuronal phenotypes. We identified impaired spermatogenesis, characterized by defective mitosis and increased apoptosis in spermatogonia, as well as hematological abnormalities indicative of macrocytic anemia. In addition, we found reduced expression of Opalin, a gene involved in oligodendrocyte differentiation, and decreased numbers of Olig2-positive oligodendrocytes, suggesting broader glial deficits beyond recently characterized neuronal abnormalities. Collectively, our results highlight the role of CEP152 dysfunction in multi-systemic abnormalities of SCKL and provide an integrative view of its impact on both neuronal and extra-neuronal development. Full article

Sustainable water remediation requires catalytic strategies that remove contaminants efficiently while reducing chemical input, byproduct formation, and ecological disturbance. Conventional radical-dominated advanced oxidation processes can rapidly degrade pollutants, but their reliance on high oxidant dosages and freely diffusing reactive oxygen species often causes matrix quenching, non-selective oxidation, low oxidant utilization, and potential ecological risks. Mild interfacial catalysis provides a materials-chemistry strategy to regulate oxidative intensity and direct contaminant transformation under environmentally relevant conditions. In this review, mild catalysts are defined by pathway-selective, interfacially confined, and environmentally compatible oxidation rather than by low dosage alone. Representative non-radical or low-intensity pathways, including singlet oxygen generation, surface-mediated electron transfer, high-valent metal–oxo species, and direct oxidative transfer processes, are discussed in relation to active-site structure, oxidant utilization, matrix tolerance, and byproduct control. We further summarize how coordination environments, defect chemistry, heteroatom configurations, nanoconfinement, and immobilized interfaces regulate reactive-species formation and interfacial charge transfer. Key material platforms, including single-atom catalysts, heteroatom-doped carbons, defect-engineered oxides, catalytic membranes, hydrogels, and floating or immobilized composites, are evaluated from mechanistic and application-oriented perspectives. Finally, catalyst regeneration, cost, microbial community responses, algae–bacteria balance, ecotoxicity, and long-term safety are discussed to guide sustainable aquatic ecosystem restoration. Full article

To address surface quality defects caused by traditional mechanical polishing of gears, such as machining scratches and large surface waviness, this study proposes a novel immersed chemical-mechanical polishing (CMP) process for gear finishing. Numerical simulations were conducted in FLUENT to analyze the gear surface stress distribution and polishing fluid flow trajectories under different process conditions. The Euler–Euler method and RNG k–ε turbulence model were used to optimize process parameters and clarify the formation mechanism of ultra-smooth tooth surfaces. Experimental results for spiral bevel gears show that the proposed immersed CMP process effectively improves surface quality. The tooth profile roughness was reduced from Ra 1.531 μm to 0.509 μm, and surface scratches were significantly alleviated. These results confirm the feasibility and effectiveness of the proposed process. This study provides a reliable approach for efficient and precision polishing of complex-structured gears and extends the application of CMP technology to non-planar mechanical components. Full article

Two-dimensional (2D) materials have emerged as a key platform for next-generation electronics due to their atomic thickness and tunable properties. Iron disulfide (FeS₂), known as pyrite, with a bandgap of ~0.95 eV, is suitable for solar energy applications. However, its performance is limited by defects in bulk crystals. Reducing FeS₂ to a single layer eliminates bulk defects and enables strain engineering of the bandgap. In this study, First-principles density functional theory (DFT) calculations are performed using the CASTEP code and the PBEsol functional to examine the structural, electronic, and optical properties of a distorted 1T′-phase FeS₂ monolayer. Full geometry optimization yields lattice parameters a′ = 17.594 Å, b′ = 3.20231 Å, c′ = 5.28091 Å, and Fe–S bond angles of ~75.8° and ~98.2°, confirming symmetry-breaking distortion. The monolayer is dynamically stable, showing no imaginary modes in the phonon dispersion, and remains structurally intact up to 1000 K in molecular dynamics simulations. The unstrained system has an indirect bandgap of 0.70 eV, with the valence band maximum at the Γ point (dominated by S-p states) and conduction band minimum near the X point (Fe-d states). Under mechanical strain (±4%), the bandgap decreases significantly: from 0.70 eV to 0.44 eV under +4% tensile strain along the y-axis, and to 0.53 eV under −4% compressive strain. Biaxial strain causes weaker modulation, reducing the gap to 0.66 eV (+4%) and 0.62 eV (−4%). Optical absorption exceeds 10⁴ cm⁻¹ for photon energies above the bandgap, with tensile strain causing redshifts and compressive strain inducing blueshifts. These findings demonstrate that 2D FeS₂ is mechanically robust, electronically tunable, and optically active, making it a promising candidate material for flexible strain sensors and photovoltaic devices. This work is intended to motivate and inform future synthesis efforts. Full article

The morphological and structural state of rough solid electrodes usually has a complex effect on the kinetics of an electrochemical process. In order to correctly distinguish the influence of different factors on the rate of an electrode reaction, it is necessary to first separate a purely geometric current rise caused by the surface area increase. At the same time, it is necessary to take into account that surface roughness itself often not only leads to a geometric rise in the electrode area, but also contributes to a change in the kinetic parameters of the electrochemical process. As a consequence, the conclusion regarding an electrocatalytic effect will be reasonable only if the roughness effect is correctly taken into account. The most difficult problem is to establish the role of roughness when experimental electrochemical data are obtained under mixed diffusion–kinetic control of the electrode process. However, the use of appropriate theoretical approaches is required to correctly determine the kinetic characteristics of the electrochemical stage, i.e., of the charge transfer stage. This paper establishes the influence of the morphology and structure of electrodeposited copper coatings on the kinetics of the cathodic reduction of nitrate ion, which occurs in a mixed diffusion–kinetic mode, using the theoretical model of chronoamperometry of an electrochemical process on a rough electrode developed earlier by the authors. Several Cu-electrodes with roughness and structure, the parameters of which vary widely enough, were obtained by cathodic deposition from sulfate solutions of different compositions. The integral (roughness factor) and local (average roughness) characteristics of the surface morphology were determined by methods of underpotential deposition and atomic force microscopy, respectively. Structural investigation of the electrodeposited coatings was carried out by X-ray diffraction to determine their crystallographic structure and average crystallite size. The methods of voltammetry and a rotating disk electrode revealed the mixed kinetics of the electroreduction of ${\mathrm{NO}}_3^{-}$ ions. The kinetic parameters of the charge transfer stage on the copper coatings with a roughness factor of f_r ≤ 3.5 are determined for the first time in this paper by treatment of the experimental current decay curves with the non-linear theoretical equation obtained by the authors for the chronoamperogram of the process on rough electrodes. It was found that the rate constant of the charge transfer stage and the exchange current density of the nitrate ion electroreduction increase by about 50%, with an increase in the average surface roughness from 25 to 120 nm. Considering that this effect is not caused by a purely geometric increase in the true surface area of the electrode, and that the average crystallite size is approximately the same (25 ± 2 nm) for all investigated coatings, it can be concluded that the electrocatalytic activity of copper increases in the reaction of the cathodic reduction of nitrate ions during the transition to copper electrodes with the higher average surface roughness. Taking into account XRD data, the role of the structural and morphological state in the kinetics of the electroreduction of nitrate ions has been established. The smoothest polycrystalline coating was found to be the least electrocatalytically active in this reaction. On the contrary, the roughest coatings with the most prominent plane (220) show the highest activity, which increases with increasing average roughness, possibly due to the growth of defects and excess energy of such curved surfaces. Full article

Micro-structured SAE H13 tool steel inserts for polymer injection molding require accurate replication of sub-millimeter features while retaining adequate densification and heat-treatment response. This study compared two powder-based routes on the same hemispherical insert containing pyramidal features of approximately 0.145 mm base width: hot embossing (HE) of water-atomized SAE H13 powder (supplier d50 = 5.7 µm, irregular morphology) compounded with a commercial M1 binder, and material extrusion (MEX) of a commercial gas-atomized SAE H13 filament processed on a Markforged Metal X. Rheological screening selected a 57:43 vol% powder-to-binder ratio for the in-house HE feedstock, and DSC/TGA measurements defined two-step debinding windows. The best HE conditions were 220 °C, 8 MPa, and 45 min for the in-house mixture, and 210 °C, 8 MPa, and 30 min for the granulated commercial filament; the latter showed a 0.15% linear deviation from the silicone replica diameter among the best-rated samples. Under the tested commercial MEX configuration, the pyramidal features were not resolved because the 0.40 mm deposition line width exceeded the target feature base width, causing the slicer to omit the sub-line-width geometry. The defect populations differed qualitatively: HE specimens showed porosity and local cracking associated with powder morphology and pressureless sintering, whereas MEX specimens showed build-direction-aligned inter-raster voids associated with the toolpath. Microhardness and tensile data are therefore interpreted as process-history-specific results rather than as a direct route ranking, because sintering conditions were not uniform across all specimens. The study defines an experimentally bound process-selection limit for SAE H13 micro-tooling: HE remains preferable for sub-nozzle surface features, whereas MEX remains attractive for macro-scale geometric freedom, if resolution, densification, and post-sintering consolidation are addressed. Full article

Background/Objectives: White–Sutton syndrome (WHSUS; OMIM 616364) is a rare neurodevelopmental disorder caused by pathogenic variants in the POGZ gene and characterized by developmental delay, intellectual disability, speech impairment, autism spectrum features, and dysmorphic traits. Although most reported cases are sporadic, inherited forms are exceptionally rare. We describe a familial case of WHSUS involving an affected mother and two children carrying a heterozygous POGZ nonsense variant, highlighting marked intra-familial phenotypic variability and expanding the clinical spectrum of the disorder. Methods: Clinical evaluation included multidisciplinary assessments. Genetic testing was performed using clinical exome sequencing (CES) with a virtual neurodevelopmental disorder (NDD) gene panel, followed by Sanger confirmation and segregation analysis in family members. The POGZ transcript reference NM_015100.3 was used for variant nomenclature and verified with the Mutalyzer tool. CNV detection from NGS data was performed using the Alissa CNV caller (Agilent) and visualized via IGV; the Xp11.22 microduplication was confirmed by chromosomal microarray (aCGH) and parental segregation analyses. Results: CES identified the heterozygous pathogenic POGZ variant c.1522C>T (p.Arg508*) in the female proband (III₆), an infant presenting with global developmental delay, hypotonia, speech impairment, gait abnormalities, and characteristic dysmorphic features. Segregation analysis demonstrated maternal inheritance and confirmed the presence of the variant in her affected brother (III₄), who also carries a de novo 1.79 kb microduplication at Xp11.22, while the maternal grandparents tested negative, indicating a de novo origin in the mother. The mother exhibited an attenuated phenotype, including mild neuropsychiatric and gastrointestinal manifestations. The variant is predicted to undergo nonsense-mediated decay (NMD), consistent with a moderate clinical presentation; however, experimental validation was not performed. Conclusions: This report documents a rare familial occurrence of WHSUS with highly variable expressivity. Our findings broaden the phenotypic and molecular characterization of POGZ-related disorders and emphasize the importance of comprehensive segregation studies and early genomic diagnosis. While experimental data link POGZ deficiency to DNA repair defects, no longitudinal clinical studies have demonstrated increased cancer risk in WHSUS; therefore, formal malignancy screening guidelines cannot be established at present, and this issue deserves future study in larger cohorts or registries. Full article

Drainage pipe defect detection is essential for maintaining the normal operation of urban infrastructure. In recent years, deep learning-based object detection methods have provided an effective technical solution for drainage pipe defect recognition. Among them, YOLO-series models have demonstrated strong potential in visual detection tasks due to their end-to-end architecture and high inference efficiency. However, directly applying baseline YOLO models may still face challenges such as limited detection accuracy, relatively high model complexity, and insufficient adaptability for lightweight deployment scenarios. To address these issues, this paper proposes a lightweight drainage pipe defect detection method based on an improved YOLO11 network. Rather than treating detection enhancement and model compression as two separate procedures, the proposed method integrates feature enhancement, adaptive pruning, and distillation-based recovery into a unified lightweight detection framework. Specifically, an improved SimAM attention mechanism is introduced into the backbone and integrated with the C3k2 module to construct the C3K2_SWS module, aiming to enhance the representation capability of critical defect features. In the neck network, a focused diffusion pyramid network with a dimension-aware selective fusion structure, termed FDPN-DASI, is designed to strengthen multi-scale feature interactions. In addition, an adaptive-threshold focal loss (ATFL) is introduced to improve the learning capability for hard samples. For efficient deployment, the LAMP pruning algorithm is further improved, and an entropy-guided entropy-adaptive magnitude-based pruning method (EA-LAMP) is proposed to enable adaptive allocation of pruning ratios across different network layers. Moreover, BCKD knowledge distillation is applied after pruning to mitigate the accuracy degradation caused by model compression. Experimental results indicate that the proposed lightweight YOLO11-SFA+EA+BCKD framework achieves a precision of 92.4%, a recall of 88.5%, and an mAP50 of 93.3%, while maintaining a compact model size of 1.6 M parameters and 4.5 G FLOPs. Compared with the baseline model, the proposed method improves precision, recall, and mAP50 by 5.9%, 5.0%, and 4.7%, respectively, while reducing the number of parameters, FLOPs, and model size by 1.0 M, 1.8 G, and 2.1 M, respectively. These results suggest that the proposed framework can improve detection performance while reducing model complexity under the current experimental setting, indicating its potential for lightweight drainage pipe defect detection tasks. Full article

Background and Clinical Significance: Left ventricular free-wall rupture (LVFWR) is a rare but devastating mechanical complication of acute myocardial infarction (AMI), with reported in-hospital mortality approaching 90% without surgical intervention. Although its incidence has declined in the contemporary primary percutaneous coronary intervention (PCI) era, LVFWR remains an important cause of early post-infarction death, particularly after delayed reperfusion or fibrinolytic therapy. Subacute or contained “oozing” ruptures pose a unique diagnostic challenge because hemodynamic stability and nonspecific symptoms can mask the underlying catastrophe, and standard transthoracic echocardiography may fail to visualize a sealed defect. Contrast-enhanced cardiac computed tomography (CT) has emerged as a valuable adjunct in this setting, enabling early recognition and surgical planning. Case Presentation: We report a case of a 51-year-old male, a heavy smoker, with acute lateral ST-segment elevation myocardial infarction (STEMI) treated with thrombolysis at a referring hospital, followed by percutaneous coronary intervention (PCI) to the obtuse marginal branch. Despite reperfusion, he developed persistent pleuritic chest pain and a small pericardial effusion. Cardiac computed tomography (CT) demonstrated a contained (sealed) lateral-wall oozing-type left ventricular free-wall rupture (LVFWR) with thrombus sealing the defect. A multidisciplinary heart team initially opted for diligent observation with frequent echocardiography. Within the first 24 h, the pericardial effusion increased, and echocardiography showed circumferential effusion with lateral wall thickening and hematoma, prompting emergent sternotomy. Intraoperatively, a large posterolateral infarct with an oozing-type LV free-wall rupture was identified. Surgical repair was performed using interrupted pledgeted sutures, native pericardial patch, BioGlue, and an overlying Teflon patch, with intra-aortic balloon pump (IABP) support. This case demonstrates the complementary diagnostic value of multimodality imaging—echocardiography for serial monitoring of the pericardial effusion and regional wall changes, and cardiac CT for direct characterization of the contained (sealed) defect—and the timely transition from conservative to surgical management in oozing-type rupture. The patient recovered uneventfully and was discharged in stable condition. Conclusions: This case highlights the diagnostic value of multimodality imaging—particularly cardiac CT—in detecting contained (sealed) LVFWR when echocardiography is inconclusive. Early recognition and prompt surgical intervention enabled a successful outcome in this otherwise frequently fatal complication. Full article

Accurate reconstruction of complete tree point clouds is essential for estimating ecosystem structural characteristics from LiDAR data. In urban forestry environments, however, terrestrial laser scanning (TLS) and mobile laser scanning (MLS) frequently produce incomplete observations. Occlusion caused by neighboring trees, together with interference from surrounding urban objects such as buildings and vehicles, often leads to missing regions within scanned point clouds. These defects may further affect the reliability of tree structural analysis and parameter estimation. Although recent learning-based point cloud completion methods have improved reconstruction performance, several limitations remain when they are applied to complex tree structures. Many existing networks depend on farthest point sampling (FPS) for feature extraction, which can result in the loss of fine-scale branching information. Furthermore, local feature aggregation methods based on the traditional k-nearest neighbor (KNN) strategy are highly sensitive to regions with uneven point cloud distribution, such as the canopy region where density variations are significant in tree point clouds. To alleviate these issues, this study proposes GS-TreeAttn, an attention-guided framework specifically for tree point cloud completion. This network models density and structural representation as a coupled problem and employs a structure-guided density-adaptive attention mechanism to jointly capture global structural dependencies and local geometric features. We comprehensively evaluate the proposed method using publicly available datasets and urban forestry data collected under real-world scanning conditions. Experimental results show that even in complex scenarios with severe occlusion and uneven sampling density, GS-TreeAttn generates more complete reconstruction results. This improvement is particularly evident in regions where the canopy and branches mutually occlude each other, where information loss is very common in real-world urban forestry. Full article

LRRK1 is essential for osteoclast-mediated bone resorption, and loss of LRRK1 function causes osteopetrosis in mice and humans. However, the mechanisms by which LRRK1 regulates osteoclast activity remain incompletely defined. We previously identified that phosphorylation of OSTM1 at threonine 328 and serine 329 was compromised in LRRK1-deficient osteoclasts. To test the role for OSTM1 phosphorylation in LRRK1 regulation of osteoclast functions, we expressed a phosphomimetic OSTM1 variant in LRRK1-null osteoclasts. Overexpression of phosphomimetic, but not a dephosphomimetic variant, partially restored resorptive activity in LRRK1-deficient osteoclasts in vitro. To test OSTM1’s role in rescuing defective bone resorption in Lrrk1-null mice, we generated Ostm1-T328E/S329D knock-in (KI) mice and crossed them onto the Lrrk1-deficient background. Ostm1-T328E/S329D KI mice displayed normal skeletal development and bone remodeling. When crossed to the Lrrk1-deficient background, OSTM1-T328E/S329D expression increased osteoclast resorptive activity and bone formation and partially improved trabecular architecture, although bone volume remained unchanged. These findings demonstrate that OSTM1 phosphorylation contributes to LRRK1-dependent regulation of osteoclast function and identify the LRRK1–OSTM1 pathway as a mechanistic node controlling bone resorption. Our work provides new insight into the molecular basis of LRRK1-mediated osteoclast function and highlights OSTM1 phosphorylation as a potential therapeutic target for metabolic bone diseases. Full article

Renal tubular disorders are often overlooked causes of acquired or inherited bone hypomineralization and fragility fractures in adults. The proximal tubule reabsorbs glucose, phosphate, low-molecular-weight proteins, amino acids, bicarbonate, and much of the sodium, potassium, chloride, and calcium. The distal nephron—the thick ascending limb of the loop of Henle, the distal convoluted tubule, and the collecting duct—regulates urine concentration and dilution, maintains acid-base balance via urinary proton secretion, and controls electrolytes, including sodium, potassium, magnesium, and calcium. Tubular defects may cause hyperphosphaturia (high urinary phosphate), hypercalciuria (high urinary calcium), or chronic metabolic acidosis (renal tubular acidosis, RTA). These changes weaken bone mineralization, disrupt bone turnover, and raise the risk of muscle weakness and fractures. This review summarizes acquired and genetic tubulopathies linked to hyperphosphaturia, hypercalciuria, and RTA and outlines a practical diagnostic approach for outpatients with bone fragility and suspected renal tubulopathy. Full article

Defect detection in manufacturing has evolved from manual inspection to deep learning-based Automated Visual Inspection (AVI) systems; however, acquiring sufficient defect samples in real industrial environments remains challenging, causing severe data sparsity and class imbalance. We propose CASDA (Context-Aware Steel Defect Augmentation), a five-stage framework that classifies defect morphology and background surface properties, constructs a compatibility matrix encoding their contextual relationship, and synthesizes defect images via a ControlNet pipeline conditioned on a three-channel hint image. Experiments on the Severstal steel dataset demonstrate that CASDA achieves an 83.0% quality validation pass rate. Under multi-seed evaluation (seeds 42 and 456), CASDA improved EB-YOLOv8’s overall mAP@0.5 by 2.60 pp over the raw baseline and achieved a Class 2 AP gain of 22.09 pp over Copy-Paste, suggesting that context-aware synthesis produces more discriminative minority-class training samples than simple patch reuse under the tested settings. Performance gains are architecture-dependent; YOLO-MFD did not show overall improvement, indicating that augmentation sensitivity varies with backbone feature representation. Full article

Background/Objectives: As the most prevalent supraventricular arrhythmia, affecting around 1% of people worldwide, atrial fibrillation (AF) is implicated with a multitude of detrimental clinical sequelae, encompassing congestive failure, thromboembolic cerebral stroke, and premature death. Accumulating epidemiological evidence convincingly demonstrates genetic defects as a cornerstone in the pathogenesis of idiopathic AF. Despite significant genetic underpinnings responsible for AF, owing to substantial genetic heterogeneity, the predisposing genetic substrates for AF in most individuals remain to be ascertained. Methods: A four-generation pedigree suffering from familial AF and a cohort of 238 subjects affected with idiopathic AF, together with 266 healthy people, were enrolled prospectively. Pan-exome sequencing analysis was implemented on the chosen AF pedigree members, and Sanger sequencing assay was performed on all research subjects. The functional effects of the detected HAND2 variations were examined via an in vitro dual-reporter gene measurement. Results: Two novel heterozygous truncating HAND2 variations, NM_021973.3: c.138G>A; p.(Trp46*) and NM_021973.3: c.337C>T; p.(Gln113*), were discovered in the AF pedigree and one of the 238 AF cases, respectively. The two HAND2 variants co-segregated with the AF phenotype and were absent from the 532 control chromosomes of 266 healthy individuals. Quantitative reporter gene assays unveiled that both Trp46*- and Gln113*-mutant HAND2 failed to transcriptionally activate HCN4 and NPPA, two AF-causative genes. Additionally, the two variations nullified the synergistic transcriptional activation of NPPA by HAND2 and GATA4, another recognized gene predisposing to AF. Conclusions: These findings support HAND2 as a strong candidate gene contributing to AF susceptibility, which unravels novel etiopathogenesis underpinning the occurrence and perpetuation of AF and offers a potential molecular target for individualized medicine. Full article