bluuebunny/crossref_metadata_2025 huggingface dataset:
A recurrent homozygous LMNA missense variant p.Thr528Met causes atypical progeroid syndrome characterized by mandibuloacral dysostosis, severe muscular dystrophy, and skeletal deformities
Authors: Saadi Abdelkrim , Navarro Claire , Ozalp Ozge , Lourenco Charles Marques , Fayek Racha , Da Silva Nathalie , Chaouch Athmane , Benahmed Meryem , Kubisch Christian , Munnich Arnold , Lévy Nicolas , Roll Patrice , Pacha Lamia Ali , Chaouch Malika , Lessel Davor , De Sandre‐Giovannoli Annachiara
Published: June 2023

Atypical progeroid syndromes (APS) are premature aging syndromes caused by pathogenic LMNA missense variants, associated with unaltered expression levels of lamins A and C, without accumulation of wild‐type or deleted prelamin A isoforms, as observed in Hutchinson‐Gilford progeria syndrome (HGPS) or HGPS‐like syndromes. A specific LMNA missense variant, (p.Thr528Met), was previously identified in a compound heterozygous state in patients affected by APS and severe familial partial lipodystrophy, whereas heterozygosity was recently identified in patients affected by Type 2 familial partial lipodystrophy. Here, we report four unrelated boys harboring homozygosity for the p.Thr528Met, variant who presented with strikingly homogeneous APS clinical features, including osteolysis of mandibles, distal clavicles and phalanges, congenital muscular dystrophy with elevated creatine kinase levels, and major skeletal deformities. Immunofluorescence analyses of patient‐derived primary fibroblasts showed a high percentage of dysmorphic nuclei with nuclear blebs and typical honeycomb patterns devoid of lamin B1. Interestingly, in some protrusions emerin or LAP2α formed aberrant aggregates, suggesting pathophysiology‐associated clues. These four cases further confirm that a specific LMNA variant can lead to the development of strikingly homogeneous clinical phenotypes, in these particular cases a premature aging phenotype with major musculoskeletal involvement linked to the homozygous p.Thr528Met variant.
Mechanochemistry‐Amended Barbier Reaction as an Expedient Alternative to Grignard Synthesis**
Authors: Varma Nallaparaju Jagadeesh , Nikonovich Tatsiana , Jarg Tatsiana , Merzhyievskyi Danylo , Aav Riina , Kananovich Dzmitry G.
Published: June 2023

Organomagnesium halides (Grignard reagents) are essential carbanionic building blocks widely used in carbon‐carbon and carbon‐heteroatom bond‐forming reactions with various electrophiles. In the Barbier variant of the Grignard synthesis, the generation of air‐ and moisture‐sensitive Grignard reagents occurs concurrently with their reaction with an electrophile. Although operationally simpler, the classic Barbier approach suffers from low yields due to multiple side reactions, thereby limiting the scope of its application. Here, we report a mechanochemical adaptation of the Mg‐mediated Barbier reaction, which overcomes these limitations and facilitates the coupling of versatile organic halides (e.g., allylic, vinylic, aromatic, aliphatic) with a diverse range of electrophilic substrates (e.g., aromatic aldehydes, ketones, esters, amides, O‐benzoyl hydroxylamine, chlorosilane, borate ester) to assemble C−C, C−N, C−Si, and C−B bonds. The mechanochemical approach has the advantage of being essentially solvent‐free, operationally simple, immune to air, and surprisingly tolerant to water and some weak Brønsted acids. Notably, solid ammonium chloride was found to improve yields in the reactions of ketones. Mechanistic studies have clarified the role of mechanochemistry in the process, indicating the generation of transient organometallics facilitated by improved mass transfer and activation of the surface of magnesium metal.
Benzidine Derivatives: A Class of High Redox Potential Molecules for Aqueous Organic Flow Batteries
Authors: Liu Xianghui , Li Tianyu , Zhang Changkun , Li Xianfeng
Published: June 2023

The development of water‐soluble redox‐active molecules with high potentials is one of the effective ways to enhance the energy density of aqueous organic flow batteries (AOFBs). Herein, a series of promising N‐substituted benzidine analogues as water‐soluble catholyte candidates with controllable redox potentials (0.78–1.01 V vs. standard hydrogen electrode (SHE)) were obtained by the molecular engineering of aqueous irreversible benzidines. Theoretical calculations reveal that the redox potentials of these benzidine derivatives in acidic solution are determined by their electronic structure and alkalinity. Among these benzidine derivatives, N,N,N′,N′‐tetraethylbenzidine(TEB) shows both high redox potential (0.82 V vs. SHE) and good solubility (1.1 M). Pairing with H4[Si(W3O10)4] anolyte, the cell displayed discharge capacity retention of 99.4 % per cycle and a high coulombic efficiency (CE) of ∼100 % over 1200 cycles. The stable discharge capacity of 41.8 Ah L−1 was achieved at the 1.0 M TEB catholyte with a CE of 97.2 % and energy efficiency (EE) of 91.2 %, demonstrating that N‐substituted benzidines could be promising for AOFBs.
Contamination of the central medical air supply with water leading to mass ventilator failure
Authors: Chia J. J. X. , Goh M. H. , Goh M. M. , Teo C. W. S. , Tan K. H. , Sewa D. W. , Ng H. F.
Published: June 2023

Here, we present a case of mass ventilator failure due to contaminated medical air. Multiple ventilators failed routine tests, including almost all of the ventilators in our intensive care unit. A faulty air compressor had led to water contamination of our centre's supply of medical air. Water entered the pipeline supply of air and, hence the ventilators and anaesthetic machines. The disruption of the machines' proportional mixer valve resulted in unreliable delivery of fresh gas flow. This malfunction was discovered during routine pre‐use checks, and backup ventilators were available to replace the faulty ventilators. A shortage of equipment was averted due to a serendipitous availability of ventilator stockpiles prepared for the COVID‐19 pandemic. Ventilator shortages are commonly described in mass casualty and pandemic scenarios. While there are multiple strategies described in literature to augment and maximise equipment available for mechanical ventilation, stockpiling equipment remains an expensive but necessary component of disaster contingency planning.
Microcellular injection molding of foamed engineering plastic parts with high dimensional accuracy
Authors: Feng Yu‐Teng , Zhan Haiying , Mi Hao‐Yang , Antwi‐Afari Maxwell Fordjour , Chen Youfu , Gu Laifa , Dong Binbin , Liu Chuntai , Shen Changyu
Published: June 2023

Polyformaldehyde (POM) and Polyamide 66 (PA66) are engineering plastics with excellent mechanical properties and thermal stability. Producing microcellular injection molded POM and PA66 parts with high dimensional accuracy would be beneficial to reduce material cost and product quality. In this research, foamed POM and PA66 gear parts were fabricated by using microcellular injection molding with supercritical nitrogen as the blowing agent. Compared to conventional injection molded parts (parts that foaming is not involved), the foamed POM and PA66 gear parts achieved 5% and 10% average weight reduction, respectively. The foamed parts displayed a lower shrinkage ratio when compared to the solid counterparts, which was attributed to the cell expansion that offset part of the inward shrinkage stress. Moreover, POM gear parts with a higher crystallinity degree presented more serious shrinkage ratio compared to the PA66 gear parts, which contributed to the denser polymer molecular chains arrangement. The shrinkage ratio in both directions of PA66 foamed gear parts depended on the injection volume, and the lowest shrinkage ratio of 0.043‰ was obtained at the injection volume of 74 mm, when the polymer reached the maximum foaming ratio. The findings from this study could provide practical guidance for preparing microcellular injection molded products with high dimensional accuracy.
Influences of processing conditions on gradient structures in injection‐molded polyethylene caps and their property correlations
Authors: Rungswang Wonchalerm , Nantasetphong Wiroj , Jarumaneeroj Chatchai , Makkaroon Bharanabha , Juabrum Sawitree , Kaewsuwan Dechmongkhon , Soontaranon Siriwat , Rugmai Supagorn
Published: June 2023

In this study, the influences of processing conditions, that is, injection speed and holding pressure, on the gradient crystal structural formation in the injection‐molded cap specimens were investigated. Skin‐, shear‐ and core‐layers across the specimen thickness were investigated in detail by using small‐angle x‐ray scattering, wide‐angle x‐ray diffraction, and atomic force microscopy techniques. The results showed that high holding pressure caused high density and large weight of the cap specimens. This strongly contributed to high displacement resistant on applied pressure (doming resistant), while the lamellar thickness (Lc) of the skin layer also played a role. On the contrary, low holding pressure promoted the formation of the random‐oriented lamellae in the core layer, which contributed to high environmental cracking resistance (ESCR). Moreover, the lower injection speed caused the thicker Lc of the skin layer, and this improved the ESCR of the specimen. The results allowed us to propose its failure mechanism.
Metabolomics and Machine Learning Identify Metabolic Differences and Potential Biomarkers for Frequent Versus Infrequent Gout Flares
Authors: Wang Ming , Li Rui , Qi Han , Pang Lei , Cui Lingling , Liu Zhen , Lu Jie , Wang Rong , Hu Shuhui , Liang Ningning , Tao Yongzhen , Dalbeth Nicola , Merriman Tony R. , Terkeltaub Robert , Yin Huiyong , Li Changgui
Published: June 2023

Objective

The objective of this study was to discover differential metabolites and pathways underlying infrequent gout flares (InGF) and frequent gout flares (FrGF) using metabolomics and to establish a predictive model by machine learning (ML) algorithms.

Methods

Serum samples from a discovery cohort of 163 patients with InGF and 239 patients with FrGF were analyzed by mass spectrometry–based untargeted metabolomics to profile differential metabolites and explore dysregulated metabolic pathways using pathway enrichment analysis and network propagation–based algorithms. ML algorithms were performed to establish a predictive model based on selected metabolites, which was further optimized by a quantitative targeted metabolomics method and validated in an independent validation cohort with 97 participants with InGF and 139 participants with FrGF.

Results

A total of 439 differential metabolites between InGF and FrGF groups were identified. Top dysregulated pathways included carbohydrates, amino acids, bile acids, and nucleotide metabolism. Subnetworks with maximum disturbances in the global metabolic networks featured cross‐talk between purine metabolism and caffeine metabolism, as well as interactions among pathways involving primary bile acid biosynthesis, taurine and hypotaurine metabolism, alanine, aspartate, and glutamate metabolism, suggesting epigenetic modifications and gut microbiome in metabolic alterations underlying InGF and FrGF. Potential metabolite biomarkers were identified using ML‐based multivariable selection and further validated by targeted metabolomics. Area under receiver operating characteristics curve for differentiating InGF and FrGF achieved 0.88 and 0.67 for the discovery and validation cohorts, respectively.

Conclusion

Systematic metabolic alterations underlie InGF and FrGF, and distinct profiles are associated with differences in gout flare frequencies. Predictive modeling based on selected metabolites from metabolomics can differentiate InGF and FrGF.
Terminal sliding mode fault‐tolerant control for aviation redundant hydraulic actuation system based on robust fault reconstruction
Authors: Li Ting , Yang Ting , Cao Yuyan , Li Yang , Wang Xinmin
Published: June 2023

This paper investigates the fault‐tolerant control problem for a dual‐redundant hydraulic actuation system on active/active (A/A) mode subject to servovalve leakage and disturbances. The change in system dynamics caused by servovalve leakage is modeled as an additive time‐varying fault. Then, an enhanced iterative learning observer with improved robustness against abrupt unknown input is designed for fault reconstruction. In view of the high relative degree of the plant, an auxiliary variable is adopted to facilitate the controller design. Combined with the fault reconstruction results, an adaptive continuous nonsingular fast terminal sliding mode (NFTSM) fault‐tolerant controller is developed, in which an NFTSM manifold is constructed based on the auxiliary variable to achieve fast convergence of trajectory tracking errors. An adaptive continuous reaching law with less chattering is designed to compensate for the influence of the lumped disturbance. Lyapunov stability analysis demonstrates that this method can ensure finite‐time convergence of sliding variable and can guarantee the trajectory tracking errors converge to the neighborhood of the origin exponentially. Finally, the effectiveness of the proposed method is verified through a comparative simulation study.
Cell membrane biomimetic nanoparticles in drug delivery
Authors: Mohammad‐Rafiei Fatemeh , Khojini Javad Yaghmoorian , Ghazvinian Fatemeh , Alimardan Sajad , Norioun Hamid , Tahershamsi Zahra , Tajbakhsh Amir , Gheibihayat Seyed Mohammad
Published: June 2023

Despite the efficiency of nanoparticle (NP) therapy, in vivo investigations have shown that it does not perform as well as in vitro. In this case, NP confronts many defensive hurdles once they enter the body. The delivery of NP to sick tissue is inhibited by these immune‐mediated clearance mechanisms. Hence, using a cell membrane to hide NP for active distribution offers up a new path for focused treatment. These NPs are better able to reach the disease's target location, leading to enhanced therapeutic efficacy. In this emerging class of drug delivery vehicles, the inherent relation between the NPs and the biological components obtained from the human body was utilized, which mimic the properties and activities of native cells. This new technology has shown the viability of using biomimicry to evade immune system‐provided biological barriers, with an emphasis on restricting clearance from the body before reaching its intended target. Furthermore, by providing signaling cues and transplanted biological components that favorably change the intrinsic immune response at the disease site, the NPs would be capable interacting with immune cells regarding the biomimetic method. Thus, we aimed to provide a current landscape and future trends of biomimetic NPs in drug delivery.
Time‐dependent sorption behavior of lentiviral vectors during anion‐exchange chromatography
Authors: Pamenter George , Davies Lee , Knevelman Carol , Miskin James , Mitrophanous Kyriacos , Dikicioglu Duygu , Bracewell Daniel G.
Published: June 2023

Use of lentiviral vectors (LVs) in clinical Cell and Gene Therapy applications is growing. However, functional product loss during capture chromatography, typically anion‐exchange (AIEX), remains a significant unresolved challenge for the design of economic processes. Despite AIEX's extensive use, variable performance and generally low recovery is reported. This poor understanding of product loss mechanisms highlights a significant gap in our knowledge of LV adsorption and other types of vector delivery systems. This work demonstrates HIV‐1‐LV recovery over quaternary‐amine membrane adsorbents is a function of time in the adsorbed state. Kinetic data for product loss in the column bound state was generated. Fitting a second order‐like rate model, we observed a rapid drop in functional recovery due to increased irreversible binding for vectors encoding two separate transgenes ( = 12.7 and 18.7 min). Upon gradient elution, a two‐peak elution profile implicating the presence of two distinct binding subpopulations is observed. Characterizing the loss kinetics of these two subpopulations showed a higher rate of vector loss in the weaker binding peak. This work highlights time spent in the adsorbed state as a critical factor impacting LV product loss and the need for consideration in LV AIEX process development workflows.
Engineering and characterization of carbohydrate‐binding modules for imaging cellulose fibrils biosynthesis in plant protoplasts
Authors: Jayachandran Dharanidaran , Smith Peter , Irfan Mohammad , Sun Junhong , Yarborough John M. , Bomble Yannick J. , Lam Eric , Chundawat Shishir P. S.
Published: June 2023

Carbohydrate binding modules (CBMs) are noncatalytic domains that assist tethered catalytic domains in substrate targeting. CBMs have therefore been used to visualize distinct polysaccharides present in the cell wall of plant cells and tissues. However, most previous studies provide a qualitative analysis of CBM‐polysaccharide interactions, with limited characterization of engineered tandem CBM designs for recognizing polysaccharides like cellulose and limited application of CBM‐based probes to visualize cellulose fibrils synthesis in model plant protoplasts with regenerating cell walls. Here, we examine the dynamic interactions of engineered type‐A CBMs from families 3a and 64 with crystalline cellulose‐I and phosphoric acid swollen cellulose. We generated tandem CBM designs to determine various characteristic properties including binding reversibility toward cellulose‐I using equilibrium binding assays. To compute the adsorption (nkon) and desorption (koff) rate constants of single versus tandem CBM designs toward nanocrystalline cellulose, we employed dynamic kinetic binding assays using quartz crystal microbalance with dissipation. Our results indicate that tandem CBM3a exhibited the highest adsorption rate to cellulose and displayed reversible binding to both crystalline/amorphous cellulose, unlike other CBM designs, making tandem CBM3a better suited for live plant cell wall biosynthesis imaging applications. We used several engineered CBMs to visualize Arabidopsis thaliana protoplasts with regenerated cell walls using confocal laser scanning microscopy and wide‐field fluorescence microscopy. Lastly, we also demonstrated how CBMs as probe reagents can enable in situ visualization of cellulose fibrils during cell wall regeneration in Arabidopsis protoplasts.
Attribute imbalances and innovation implementation based on grounded theory: A case of Chinese enterprises in Gansu Province
Authors: Wang Hui , Jiang Anyin , Ahmad Fayyaz , Abid Nabila , Chandio Abbas Ali
Published: June 2023

The purpose of this study is to explore what are the determinant influencing factors of the dilemma faced by state‐owned enterprises (SOEs) in implementing innovation‐driven development strategy (IDDS), to propose a suitable localized theory. It also suggests specific actions to break the post‐effects dilemma with the support of this theory. Based on the innovation‐driven survey data of provincial SOEs in Gansu Province, this study adopts grounded theory to conduct exploratory research. This paper constructs a behavior process model of “pre‐motivation, innovation path, and post‐effects dilemma” to drive SOE's innovation and expounds on the incidence relation among the three. The results show that the external administrative force and institutional mechanism drive have both indirect and direct impacts on the innovation path choice of SOEs. The endogenous drive is distorted by external administrative force and institutional mechanism drive, leading to the limited innovation path choice and post‐effects dilemma. This paper puts forward the theory of “Attribute Imbalance–Weakened Autonomy–Sluggish Innovation” and explains why it is sometimes difficult to implement the IDDS of SOEs. However, there are some regional limitations in the application scenarios of the theory. Whether it has broader implications need to further explore. Future theoretical testing should be conducted with a sample of SOEs in developed market regions. It is possible to study the differences in the degree of government intervention in SOEs, the ways of intervention, and the impact of these differences on SOEs' balancing dual attribute conflicts.
Network Pharmacology and Molecular Docking Reveal the Mechanism of Corydalis saxicola Bunting Total Alkaloids in Treating Radiation‐Induced Oral Mucositis
Authors: Lai Yiyuan , Yong Xiangzhi , Wei Yongzheng , Wei Shanni , Su Zhiheng , Tao Renchuan
Published: June 2023

The study aims to explore the effect and mechanism of total alkaloids of Corydalis saxicola Bunting (CSBTA) in the treatment of radiation induced oral mucositis (RIOM) through network pharmacology and molecular docking. The components and corresponding targets of Corydalis saxicola Bunting were screened by literature review. RIOM related targets were obtained in GeneCards. Cytoscape software was used to construct the component‐target‐pathway network. Protein‐Protein Interaction (PPI) networks was constructed by String database. GO and KEGG enrichment analyses were performed by Metascape. AutoDock Vina 4.2 software was used for molecular docking. There were 26 components of CSBTA targeting 61 genes related to RIOM. Through Cytoscape and PPI analysis, 15 core target genes of CSBTA for treating RIOM were identified. GO functional analysis indicated that CSBTA might play a role through kinase binding and protein kinase activation. KEGG pathway analysis showed that the core targets of CSBTA were mainly focused on cancer and reactive oxygen species (ROS) pathway. The results of molecular docking showed that CSBTA had strong binding energy with target protein including SRC, AKT and EGFR. The study demonstrates that CSBTA may treat RIOM by affecting SRC, AKT and EGFR through ROS pathway.
Noncovalent Association Thermodynamics of Turn‐On Fluorescent Probes with Human Serum Albumin: Dual‐Concentration Ratio Method
Authors: Chen Han‐Yu , Teng Chung‐Siang , Lin Pin‐Han , Liu Ching‐Ping , Liu Wei‐Min , Chu Li‐Kang
Published: June 2023

Efficient quantification of the affinity of a drug and the targeted protein is critical for strategic drug design. Among the various molecules, turn‐on fluorescent probes are the most promising signal transducers to reveal the binding strength and site‐specificity of designed drugs. However, the conventional method of measuring the binding ability of turn‐on fluorescent probes by using the fractional occupancy under the law of mass action is time‐consuming and a massive sample is required. Here, we report a new method, called dual‐concentration ratio method, for quantifying the binding affinity of fluorescent probes and human serum albumin (HSA). Temperature‐dependent fluorescence intensity ratios of a one‐to‐one complex (L ⋅ HSA) for a turn‐on fluorescent probe (L), e. g., ThT (thioflavin T) or DG (dansylglycine), with HSA at two different values of [L]0/[HSA]0 under the constraint [HSA]0>[L]0 were collected. The van't Hoff analysis on these association constants further resulted in the thermodynamic properties. Since only two samples at different [L]0/[HSA]0 are required without the need of [L]0/[HSA]0 at a wide range, the dual‐concentration ratio method is an easy way to greatly reduce the amounts of fluorescent probes and proteins, as well as the acquisition time.
[99m Tc] Tc‐MDP bone SPECT/CT diagnosing unstable slipped capital femoral epiphysis with secondary AVN in a patient with misleading knee pain
Authors: Ebrahimi Marzieh , Ziaee Vahid , Nemati Maryam , Paymani Zeinab
Published: June 2023

Bone scan is highly sensitive whole‐body imaging with relative low radiation in patients with non‐localized skeletal symptoms. Patient is 12‐year‐old boy with Down syndrome, suffering recent claudication and exacerbated left knee pain unable to walk even with crutches. Three‐dimensional Single photon emission computed tomography/Computed tomography (SPECT/CT) detected left slipped capital femoral epiphysis (SCFE) and secondary Avascular necrosis (AVN).
Highly Selective Conversion of Carbon Dioxide to Methanol through a Cu−ZnO−Al2O3−ZrO2/Cu−MOR Tandem Catalyst
Authors: Wang Yuxin , Wei Yayu , Li Yanhong , Chen Xiaofang , Caro Jürgen , Huang Aisheng
Published: June 2023

Methanol formation from CO2 hydrogenation attracts great attention in view of utilization of carbon resources. However, CO2 transformation to methanol is challenging because of the thermodynamic equilibrium restriction and water‐caused catalyst deactivation. It is desired, therefore, to develop highly active, selective and stable catalysts for CO2 hydrogenation to methanol. Herein, we propose a novel tandem catalyst composed of Cu−ZnO−Al2O3−ZrO2 (CZAZ) and Cu−MOR for highly selective conversion of CO2 to methanol. During CO2 hydrogenation by the CZAZ catalyst, the by‐product methane is continuously transformed to methanol through reaction with water via the Cu−MOR catalyst, thus enhancing CO2 conversion and methanol selectivity. Under mild reaction conditions (200 °C and 3.0 MPa), high CO2 conversion (40.7 %) and methanol selectivity (97.6 %) are achieved, outperforming state‐of‐the‐art CO2 hydrogenation catalysts. Further, water‐caused deactivation of the catalyst through aggregation and densification is suppressed owing to water consumption via methane oxidation to methanol, validating a high CZAZ/Cu−MOR tandem catalyst stability.
Dem Regen trotzen
Authors: Hübner Karl
Published: June 2023

Vor 200 Jahren begann der Schotte Charles Macintosh, Textilgewebe mit einer Schicht aus Naturkautschuk wasserdicht zu machen und fertigte daraus auch Regenmäntel. Im 20. Jahrhundert sorgten zunehmend Kunststoffe wie PVC oder PU für regenfeste Bekleidung. Seit über 40 Jahren tragen darüber hinaus atmungsaktive Membranen zu neuem Tragekomfort bei. Inzwischen arbeiten Hersteller verstärkt daran, für den Regenschutz möglichst umweltfreundliche und nachhaltige Materialien zu verwenden.
Sturge–Weber syndrome: Updates in pathogenesis, diagnosis, and treatment
Authors: Valery Chelsea B. , Comi Anne M.
Published: June 2023

Objectives

We summarize the current knowledge of Sturge–Weber syndrome (SWS) including genetic involvement, difficulties in diagnosis, symptoms caused by the vascular malformations, treatments, and future areas of research.

Methods

PubMed searches were completed between October and December of 2022 including the following search terms: Sturge–Weber neuroimaging, Sturge–Weber ocular involvement, Sturge–Weber G‐protein alpha q subunit (GNAQ), Sturge–Weber presymptomatic treatment, and Sturge–Weber quantitative EEG. Clinically relevant articles and case reports were reviewed and summarized, with emphasis placed on reports from the last 20 years.

Results

Literature suggests that early identification of brain involvement is essential for optimal medical care. Infants with a port‐wine birthmark on the forehead, temple, or eyelids are at risk for SWS brain and eye involvement. Neuroimaging findings include leptomeningeal enhancements, cortical calcifications, and brain atrophy, and diagnosis requires magnetic resonance imaging with and without contrast. Before 1 year of age, neuroimaging has low sensitivity and may underestimate the extent of involvement; imaging after 1 year of age is needed to exclude brain involvement. The most common underlying cause for SWS is a somatic mosaic mutation inGNAQ. Neurological symptoms include seizures, stroke or stroke‐like episodes, headaches, and cognitive deficits. Recommended treatment for SWS brain involvement includes aggressive seizure control with antiepileptic medications; low‐dose aspirin is also frequently but not universally utilized. Current literature suggests that children with SWS may benefit from presymptomatic treatment; further study of this approach is ongoing.

Conclusions

SWS is a rare neurovascular disorder usually signaled by a facial port‐wine birthmark. Early diagnosis and appropriate treatment may improve outcomes.
Automatic performance tuning using the ATMathCoreLib tool: Two experimental studies related to dense symmetric eigensolvers
Authors: Kobayashi Masato , Hirota Yusuke , Kudo Shuhei , Hoshi Takeo , Yamamoto Yusaku
Published: June 2023

We consider automatic performance tuning of dense symmetric eigenvalue problems using ATMathCoreLib, which is a library to assist automatic tuning. We deal with two problems, namely, automatic code selection for the symmetric generalized eigenvalue problem in distributed‐memory parallel environments and automatic parameter tuning in tridiagonalization of dense symmetric matrices on multicore processors. As for the first problem, numerical experiments show that ATMathCoreLib can choose the fastest solver for a given computing environment and problem size quickly even if the fluctuation in the execution time is as high as 40%. As for the second problem, ATMathCoreLib was able to select nearly optimal combinations of the algorithm and its parameter reliably and efficiently for various computing environments and matrix sizes. The performance of auto‐tuning was further enhanced by incorporating a user‐provided execution‐time model into ATMathCoreLib.
Comparison of the marginal and internal fit of PMMA interim crowns printed with different layer thicknesses in 3D‐printing technique
Authors: Hasanzade Mahya , Yaghoobi Negin , Nematollahi Parsa , Ghazanfari Rezvaneh
Published: June 2023

Objective

The aim of this in vitro study was to compare the effect of printing layer thickness on the marginal and internal fit of interim crowns.

Material and methods

A maxillary first molar model was prepared for ceramic restoration. Thirty‐six crowns were printed with three different layer thicknesses using a digital light processing‐based three‐dimensional printer (25, 50, and 100 µm [LT 25, LT 50, and LT 100]). The marginal and internal gaps of the crowns were measured with replica technique. An analysis of variance was conducted to determine if there were significant differences between the groups (ɑ = .05).

Results

The marginal gap of LT 100 group was significantly higher than that LT 25 (p = .002) and LT 50 groups (p ≤ .001). The LT 25 group has significantly larger axial gaps than LT 50 group (p = .013); however, there were no statistically significant differences between other groups. The LT 50 group showed the smallest axio‐occlusal gap. The mean occlusal gap differed significantly by printing layer thickness (p ≤ .001), with the largest gap occurring for LT 100.

Conclusions

Provisional crowns printed with 50 µm layer thickness provided the best marginal and internal fit.

Clinical significance

It is recommended that provisional crowns be printed with a 50 µm layer thickness to ensure optimal marginal and internal fit.
A low power external capacitor‐less low drop‐out regulator with low over/undershoot voltage and high PSR
Authors: Ahmadi Mohammad , Ebrahimi Emad
Published: June 2023

This paper presents a new architecture for improving power supply rejection (PSR) and load transient response in a capacitor‐less low drop‐out (LDO) voltage regulator. In the proposed architecture, inserting a leading path from the power supply to the gate of the pass transistor keeps the pass transistor gate‐source voltage constant in the presence of the supply voltage ripples that results in the PSR enhancement of the LDO. In addition, by using a frequency compensation technique, the amount of undershoot and overshoot voltages in the presence of a sudden change of load current are reduced. Because of the creation of a zero in the LDO's transfer function, the effect of non‐dominant pole on the phase of LDO is reduced, and a 58° phase margin is achieved without any off‐chip capacitor. As reducing the power consumption as much as possible increases the battery life, therefore, in addition to using the current‐reused technique, the transistors of control circuit are biased in the subthreshold region. The proposed regulator with a total of only 3.1 pF on‐chip capacitor is designed and simulated in 180 nm complementary metal‐oxide semiconductor (CMOS) technology for an output regulated voltage of 1.6 V and a load current of 50 μA to 50 mA. Post‐layout simulation results show that the proposed circuit with an active area of 0.0145 mm2 and the input voltage of 1.8 to 2.4 V has only 1.504 μW power dissipation and −80 and −64 dB PSR at 1 and 10 kHz frequencies, respectively.
Prmt5 deficient mouse B cells display RNA processing complexity and slower colorectal tumor progression
Authors: Zhou Bingqian , Chen Ningdai , Chen Zheyi , Chen Shiyu , Yang Junyao , Zheng Yingxia , Shen Lisong
Published: June 2023

Protein arginine methyltransferase 5 (Prmt5) is essential for normal B‐cell development; however, the roles of Prmt5 in tumor‐infiltrating B cells in tumor therapy have not been well elucidated. Here, we revealed that CD19‐cre‐Prmt5fl/fl (Prmt5cko) mice showed smaller tumor weights and volumes in the colorectal cancer mouse model; B cells expressed higher levels of Ccl22 and Il12a, which attracted T cells to the tumor site. Furthermore, we used direct RNA sequencing to comprehensively profile RNA processes in Prmt5 deletion B cells to explore underline mechanisms. We found significantly differentially expressed isoforms, mRNA splicing, poly(A) tail lengths, and m6A modification changes between the Prmt5cko and control groups. Cd74 isoform expressions might be regulated by mRNA splicing; the expression of two novel Cd74 isoforms was decreased, while one isoform was elevated in the Prmt5cko group, but the Cd74 gene expression showed no changes. We observed Ccl22, Ighg1, and Il12a expression was significantly increased in the Prmt5cko group, whereas Jak3 and Stat5b expression was decreased. Ccl22 and Ighg1 expression might be associated with poly(A) tail length, Jak3, Stat5b, and Il12a expression might be modulated by m6A modification. Our study demonstrated that Prmt5 regulates B‐cell function through different mechanisms and supported the development of Prmt5‐targeted antitumor treatments.
Blue‐LED Synthesis of Pummerer's Ketones by Peroxidase‐Lignin‐Catalyzed Oxidative Coupling of Substituted Phenols
Authors: Piccinino Davide , Ceccotti Vlas Natalia , Gabellone Sofia , Capecchi Eliana , Saladino Raffaele
Published: June 2023

Pummerer's ketones resembling the tricyclic scaffold of bioactive natural substances were synthesized by blue‐LED driven Horseradish Peroxidase oxidative coupling of substituted phenols in 2‐methyltetrahydrofuran by using meso‐tetraphenylporphyrin as photosensitizer and dioxygen as primary oxidant. The application of functionalized lignin nanoparticles as a renewable and efficient platform for the immobilization of the enzyme extended the effectiveness of the overall process to heterogeneous catalysis under buffer limiting conditions.
Dry Methane Reforming in a Piston Engine for Chemical Energy Storage and Carbon Dioxide Utilization: Kinetic Modeling and Thermodynamic Evaluation
Authors: Rudolph Charlotte , Atakan Burak
Published: June 2023

The flexible energy conversion in piston engines offers one possibility for storing energy from renewable sources. This work theoretically explores the engine‐based dry methane reforming converting mechanical energy into chemical energy. The endothermic, endergonic reforming process is activated by the temperature increase during the compression stroke, assisted by a reduction of the heat capacity through dilution with argon. This leads to an increase in chemical exergy, as higher‐exergy species are produced with small exergy losses while simultaneously consuming CO2. The engine‐based homogenous dry reforming serves as a flexible power‐to‐gas process and energy storage solution, presenting an alternative to catalytic processes. The piston engine is simulated using a time‐dependent single‐zone model with detailed chemical kinetics, followed by an analysis of thermodynamics and kinetics. With inlet temperatures ranging from 423–473 K and argon dilutions of 91–94 mol%, CH4 and CO2 conversion are between 50%–90% and 30%–80%, respectively, resulting in synthesis gas yields of 45–55%. Additionally, higher hydrocarbons such as C2H2, C2H4, and C6H6 are produced with yields of up to 20%, 10%, and 10%. So, this power‐to‐gas process allows for exergy storage of up to 3.35 kW L−1 per cycle with an efficiency of up to 75%.
Insights into the Application of 2D Phosphorene in Dye‐Sensitized Solar Cells
Authors: Derbali Sarah , Moudam Omar
Published: June 2023

With simpler manufacturing processes and production costs lower than by one‐fifth of those for silicon solar cells, dye‐sensitized solar cells (DSSCs) are among the most promising technologies of third‐generation cells possessing the capability of producing electricity under cloudy conditions and low or artificial light in comparison to other technologies. Although large‐scale commercialization of DSSCs is inhibited by their low power conversion efficiency. To boost the efficiency, several approaches have been implemented, including 2D phosphorene material, which has attracted interest due to its unique characteristics. Since its synthesis in 2014, the use of 2D phosphorene in various optoelectronic devices, including solar cells, has been widely reviewed in the literature. The deficiency in the availability of full reviews on the integration of 2D phosphorene in DSSCs propels this review to focus on research studies relating to the application of 2D phosphorene in DSSCs.
Optimizing the Efficiency of Lead‐Free Cs2TiI6‐Based Double Halide Perovskite Solar Cells Using SCAPS‐1D
Authors: Rehman Ubaid ur , Almousa Nouf , Sahar Kashaf ul , Ashfaq Arslan , Mahmood Khalid , Shokralla Elsammani Ali , Al-Buriahi Mohammed S. , Alrowaili Ziyad A. , Capangpangan Rey Y. , Alguno Arnold C.
Published: June 2023

Conventional lead halides perovskites have attracted much attention to become a next‐generation solar technology. Besides their low cost and excellent efficiency, high toxic nature and device instability become the major issue to limit their wide applications. Ti‐based (Cs2TiI6) double halide perovskite solar cells (PSCs) have emerged as a potential candidate to become the best alternate. However, poor device efficiency is still a restriction. Therefore, proper optimization and deep device analysis are needed to explore the full potential of Cs2TiI6‐based PSCs. In the present research work, the open‐circuit voltage (Voc), current density (Jsc), fill factor (FF), and power conversion efficiency of Cs2TiI6‐based PSC are successfully optimized. The device optimization is performed using the different combinations of electron transport layers and hole transport layers along with absorber thickness, acceptor doping density, and interface defect density. The effect of Rseries and Rshunt is explored and identified the best device efficiency of 28.07% along with other photovoltaic (PV) parameters; Voc = 1.41 V, Jsc = 22.44 mA cm−2, and FF = 88.15%, respectively. The supportive analysis of C–V and Mott–Schottky characteristics helps to get deep insight into the device's performance. Furthermore, the resultant PV parameters are compared to the previous studies as well.
The pharmacological treatment of epilepsy in adults
Authors: Tomson Torbjörn , Zelano Johan , Dang Yew Li , Perucca Piero
Published: June 2023

The pharmacological treatment of epilepsy entails several critical decisions that need to be based on an individual careful risk–benefit analysis. These include when to initiate treatment and with which antiseizure medication (ASM). With more than 25 ASMs on the market, physicians have opportunities to tailor the treatment to individual patients´ needs. ASM selection is primarily based on the patient's type of epilepsy and spectrum of ASM efficacy, but several other factors must be considered. These include age, sex, comorbidities, and concomitant medications to mention the most important. Individual susceptibility to adverse drug effects, ease of use, costs, and personal preferences should also be taken into account. Once an ASM has been selected, the next step is to decide on an individual target maintenance dose and a titration scheme to reach this dose. When the clinical circumstances permit, a slow titration is generally preferred since it is associated with improved tolerability. The maintenance dose is adjusted based on the clinical response aiming at the lowest effective dose. Therapeutic drug monitoring can be of value in efforts to establish the optimal dose. If the first monotherapy fails to control seizures without significant adverse effects, the next step will be to gradually switch to an alternative monotherapy, or sometimes to add another ASM. If an add‐on is considered, combining ASMs with different modes of action is usually recommended. Misdiagnosis of epilepsy, non‐adherence and suboptimal dosing are frequent causes of treatment failure and should be excluded before a patient is regarded as drug‐resistant. Other treatment modalities, including epilepsy surgery, neuromodulation, and dietary therapies, should be considered for truly drug‐resistant patients. After some years of seizure freedom, the question of ASM withdrawal often arises. Although successful in many, withdrawal is also associated with risks and the decision needs to be based on careful risk–benefit analysis.
Quantitative reconstruction of competing sources reveals spatial heterogeneity and complex aeolian–fluvial interactions in the Horqin Sandy Land, NE China
Authors: Wu Peng , Xie Yuanyun , Kang Chunguo , Chi Yunping , Wang Yehui , Sun Lei , Wei Zhenyu
Published: June 2023

The provenance and aeolian–fluvial interaction are essential for understanding the formation and evolution of sandy land. However, the dune sediment source in the Horqin Sandy Land (HQSL), the largest semi‐stable sandy land in China, has been debated. Here, we collected 900 coarse (i.e., >63 μm) and 750 fine (i.e., <63 μm) detrital zircon (DZ) grains from surface sand in different spatial locations for U–Pb age analysis. The visual comparison largely shows remarkably similar zircon age characteristics throughout the HQSL, and the quantitative results of the inverse Monte Carlo modelling show the primary detrital contribution of the Central Asian orogenic belt (~50.5%–61.3%) to the HQSL. In contrast, the southeast of the HQSL is characterized by the absolute dominance of the North China Craton (~75.8%). The DZ age spectra in the western and northern HQSL are extremely similar but significantly different from the south, indicating the spatial heterogeneity of the HQSL. The DZ ages are largely unaffected by particle size sorting, except in the southeastern HQSL (e.g., the dramatically different detrital contribution of the North China Craton to the fine [52.4%] and coarse fractions [75.8%]). We argue that the aeolian–fluvial interaction and the resulting sedimentary sorting and recycling are responsible for the DZ age characteristics of the HQSL. We confirm that the DZ ages of the HQSL are a faithful record of the tectonic‐magmatic events, for example, the North China Craton massif collision and collusion, the Paleo‐Asian Ocean subduction and closure, the Mongolian‐Okhotsk Ocean plate subduction and the paleo‐Pacific Ocean subduction and retreat. Finally, this study suggests that caution must be exercised in interpreting the paleoclimatic record and the aeolian deposit source investigations due to the spatial heterogeneity and the complex wind‐fluvial interactions in the HQSL.
Daily tourism forecasting through a novel method based on principal component analysis, grey wolf optimizer, and extreme learning machine
Authors: Zhang Chuan , Hu Ao‐Yun , Tian Yu‐Xin
Published: June 2023

Accurate forecasting tourism demand is crucial for improving the economic benefits of tourist attractions, but it is a challenging task. In this paper, we propose an effective daily tourism forecast model, principal component analysis‐grey wolf optimizer‐extreme learning machine (PCA‐GWO‐ELM), based on Baidu index data, holiday data, and weather data. Our model uses PCA to reduce the dimensionality of the data and employs the GWO to optimize the number of neural networks in the hidden layer of the ELM model, improving its forecast performance. We conduct an empirical study using the collected tourist data of Mount Siguniang. The results show that the proposed hybrid forecasting model outperforms other models in daily tourism demand forecasting, making it a potential candidate method for practitioners and researchers studying tourism demand forecasting.
Petrogenesis of the Palaeoproterozoic (1.87 Ga) Damyang amphibolites in the north‐western margin of the Yeongnam Massif and tectonic implications of the Korean Peninsula
Authors: Wang Xin Ping , Lee Byung Choon , Oh Chang Whan
Published: June 2023

The Yeongnam Massif is located in the southern part of the Korean Peninsula and holds key elements in understanding the Palaeoproterozoic tectonic evolution of north‐east Asia during the amalgamation of the Columbia supercontinent. In this study, we have conducted geochronological and geochemical studies on the mafic intrusive along the northern margin of the Yeongnam Massif. Sensitive high‐resolution ion microprobe (SHRIMP) U–Pb zircon dating on two amphibolite and one gabbro samples in the Damyang area show that the amphibolites crystallized at ~1.87 Ga and the gabbro crystallized at ~87.6 Ma. The 1.87 Ga Damyang amphibolites are simultaneous with the Muju and Okbang amphibolites along the northern margin of the Yeongnam Massif. They also show similar geochemical characteristics, including tholeiitic basalt composition, enriched Light Rare Earth Elements (LREE) and large ion lithosphile elements (LILEs), depleted high field strength elements (HFSEs), and slightly enriched but various Hf isotopes, indicating that they originated from an enriched but heterogeneous mantle source with fluid addition. Thus, the 1.87 Ga amphibolites along the northern margin of the Yeongnam Massif were probably emplaced in an arc‐related setting. Combined with the 2.02–1.96 Ga magmatism, it is proposed that the subduction might have lasted during the 2.02–1.85 Ga interval and mantle‐derived magma rose up due to the roll‐back process between 1.88–1.85 Ga.
Promotion effect of Co addition on the activity and SO2 tolerance of CrCe catalysts for selective catalytic reduction of NOx with NH3 at middle–low temperatures
Authors: Zeng Gai , Wei Ninghan , Hu Xiaomei , Zhao Cheng , Yun Junge , Chen Zimo , Zheng Han , Xiao Liping , Chen Zhihang
Published: June 2023

Background

Nitrogen oxides (NOx) can result in a series of environmental issues (haze, photochemical smog and acid rain) that endanger human health. Currently, selective catalytic reduction (SCR) of NOx with ammonia (NH3‐SCR) has been considered to be one of the most efficient techniques to eliminate NOx.

Results

Novel Co‐modified chromium/cerium (CrCe) catalysts were synthesized by the citric acid method to improve their insufficient SCR activity and sulfur dioxide (SO2) resistance for NOx removal at middle–low temperatures. Cobalt (Co) addition could significantly increase the catalytic activity of CrCe catalysts, and the 5CoCrCe catalyst exhibited the highest catalytic activity with ≤95% NOx conversion and >80% N2 selectivity within 180–280 °C. In addition, the 5CoCrCe catalyst exhibited outstanding SO2 resistance as well as reversibility, with >90% NO conversion under 600 ppm SO2 for 510 min.

Conclusion

It was determined by characterizations that Co addition can increase SSA, Cr6+ concentration, electron transfer between Co and Cr by redox cycle (Cr6+ + 4Co2+ ↔ 4Co3+ + Cr2+) and the redox properties of the CrCe catalyst. The 5CoCrCe catalyst surface consists mainly of Lewis acid and its NH3‐SCR reactions follows the Eley–Rideal (E–R) mechanism. The 5CoCrCe catalyst exhibited outstanding SO2 resistance due to higher Cr3+, Ce3+ and Oα concentrations and strong redox cycles (3Ce4+ + Cr3+ ↔ 3Ce3+ + Cr6+ and Cr6+ + 3Co2+ ↔ 3Co3+ + Cr3+). Furthermore, in situ DRIFTS results revealed that SO2 could induce more adsorbed NOx and NH3 species instead of bulk sulfate, which also was responsible for good SO2 resistance. © 2023 Society of Chemical Industry.