bluuebunny/crossref_metadata_2025_split huggingface dataset:
TEK gene‐related primary congenital glaucoma: Phenotypic features and mutational spectrum in a Mexican cohort of 10 unrelated families
Authors: Chacon‐Camacho Oscar Francisco , Ordaz‐Robles Thania , Cid‐García Marion Aline , Hofmann‐Blancas María Enriqueta , Ledesma‐Gil Jasbeth , García‐Huerta María Magdalena , Prado‐Larrea Carolina , Cortés‐González Vianney , Lozano‐Garza Rodrigo Isaac , García‐Vega Daphne , Kim JiHye , Khang Rin , Lee Eugene , Zenteno Juan Carlos 
Published: June 2024

AbstractPrimary congenital glaucoma (PCG) is one of the leading causes of visual damage and blindness, severely affecting the quality of life of affected children. It is characterized by cupping of the optic disc and loss of ganglion cells due to elevated intraocular pressure. While most PCG patients exhibit epiphora, photophobia, and buphthalmos with corneal opacity, variability in phenotypic manifestations is not uncommon. Prompt diagnosis and treatment of PCG affected individuals becomes relevant to preserve visual function throughout their lives. Most PCG cases are sporadic or autosomal recessive; however, an incompletely dominant autosomal dominant form arising from mutations in the TEK gene has recently been demonstrated. Here, we describe the clinical and mutational features of a cohort of Mexican patients with TEK‐related PCG. Our results support the involvement of the TEK gene as an important cause of the disease in our ethnic group and expand the mutational spectrum causing PCG by reporting 10 novel disease‐causing variants.
Noonan syndrome and type 1 Chiari malformation: Possible association
Authors: Samuels Megan , Northrup Hope 
Published: June 2024

AbstractNoonan syndrome (NS) is mostly an autosomal dominant genetic disorder that affects between 1 in 1000 and 1 in 2500 people. Type 1 Chiari malformations (CM1) have an estimated prevalence of <1 in 1000 people. Though NS typically spares the posterior fossa, there have been 11 past instances of patients with NS having a concurrent CM1 that have been published in the literature. Each of these 11 cases occurred sporadically, in an isolated individual with no published family history of CM1. This case report presents a three generational family with four members having both NS and concurrent CM1. All affected family members share a pathogenic variant in PTPN11. A literature review was performed to identify and compile data regarding all past published cases of NS and CM1 occurring concurrently. Since 1982, a dozen case reports have detailed NS with concurrent CM1. Where molecular genetic data was presented, seven had a variant in PTPN11, and only one had a variant in another gene. The clustering of NS with CM1 within a single family that shares the same genotype, along with the exclusion of both NS and CM1 in other family members, may indicate that CM1 is a part of the NS phenotype.
Structure‐Activity Relationships of the Structural Analogs Au8Cu1 and Au8Ag1 in the Electrocatalytic CO2 Reduction Reaction
Authors: Su Shangyu , Zhou Yanting , Xiong Lin , Jin Shan , Du Yuanxin , Zhu Manzhou 
Published: June 2024

AbstractOwing to the significant attention directed toward alloy metal nanoclusters, it is crucial to explore the relationship between their structures and their performance during the electrocatalytic CO2 reduction reaction (eCO2RR) and discover potential synergistic effects for the design of novel functional nanoclusters. However, a lack of suitable analogs makes this investigation challenging. In this study, we synthesized a well‐defined pair of structural analogs, [Au8Cu1(SAdm)4(Dppm)3Cl]2+ and [Au8Ag1(SAdm)4(Dppm)3Cl]2+ (Au8Cu1 and Au8Ag1, respectively), and characterized them. Single‐crystal X‐ray diffraction analysis revealed that Au8M1 (M=Cu/Ag) consists of a tetrahedral Au3M1 core capped by three (Dppm)Au staples, one Au2(SR)3 staple, one lone SR ligand, and a terminal Cl ligand. Ag and Cu were doped at the same site in the Au8M1 nanoclusters, which has rarely been reported. Au8Cu1 exhibited a significantly higher CO Faradaic efficiency (FECO; ~82.2 %) during eCO2RR than that of Au8Ag1 (FECO; ~33.1 %). Density functional theory calculations demonstrated that *COOH is the key intermediate in the reduction of CO2 to CO. The formation of *COOH on Au8Cu1 is more thermodynamically stable than on Au8Ag1, and Au8Cu1 shows a smaller *CO formation energy than that on Au8Ag1, which promotes the reduction of CO2. We believe that the structural analogs Au8Cu1 and Au8Ag1 offer a suitable template for the in‐depth investigation of structure‐property correlations at the atomic level.
Cobaltkatalysierte Photochemische Semipinakol‐Umlagerung Nichtaktivierter Allylalkohole
Authors: Lindner Henry , Carreira Erick M. 
Published: June 2024

AbstractEine photochemische Methode für die Semipinakol‐Umlagerung von nichtaktivierten Allylalkoholen wird beschrieben. Die Bedingungen erlauben die Umlagerung sowohl aliphatischer als auch aromatischer Reste und formen eine Vielzahl von α,α‐disubstituierten Ketonen. Die Reaktion erfolgt unter milden Bedingungen und toleriert unter anderem Ether, Ester, Halogene, Nitrile, Carbamate und substituierte Arene. Die operativ einfachen und vollständig katalytischen Bedingungen erfordern 1 mol % Benzothiazinoquinoxalin als Organophotokatalysator, 0,5 mol % Co‐Salen und 10 mol % Lutidiniumtriflat und ermöglichen eine Reaktivität, die komplementär zu der mit Brønsted‐Säure beobachteten ist. Wir demonstrieren den Nutzen des Protokolls für die Diversifizierung von Arzneistoffen.
Structure and composition of mesophase pitch prepared from aromatic‐rich fluid catalytic cracking slurry under different process conditions
Authors: Yu Tao , Ma Yu , Yu Xiaoyan , Riwan Muhammad , Wang Mingzhi , Zhou Xiaolong 
Published: June 2024

AbstractThe high aromaticity of fluidized catalytic cracking (FCC) slurry makes it a superior raw material for the production of high‐performance carbon materials. In this study, direct thermal polycondensation of aromatic‐rich FCC slurries is conducted to synthesize mesophase pitches with a significant anisotropic content. The effects of stirring speed and the pressurized‐atmospheric two‐stage reaction on the structure and composition of the products are investigated. Thermal stability analysis using thermogravimetric (TG) test, observation of mesophase content and optical structure through polarized light microscopy, characterization of material composition and molecular structure via Fourier transform infrared spectroscopy (FT‐IR) and nuclear magnetic resonance hydrogen spectrum (1H NMR), as well as comparison of crystal structures using X‐ray diffraction (XRD) are performed. The experimental results demonstrate that an increase in the stirring rate leads to a more homogeneous molecular distribution within the reaction system, thereby facilitating molecular contact polycondensation and promoting mesophase growth and development. Furthermore, the pressurized‐atmospheric two‐stage reaction process also contributes to mesophase development, resulting in products with more cycloalkane structure, improved thermal stability, and optimized optical structure transitioning from mosaic to flow or even domain.
Oil‐based epoxy and their composites: A sustainable alternative to traditional epoxy
Authors: Devansh  , Patil Pranit , Pinjari Dipak V. 
Published: June 2024

AbstractAs global industries move toward sustainability, the search for alternatives to current petroleum‐based polymer products is becoming necessary. Bio‐based epoxy resins have emerged as a sustainable alternative to petroleum‐based epoxy resins. Commonly, furan derivatives, carbohydrates, cardanol, and lignin are commercially utilized with bio‐based curing agents to form crosslinked structures. In recent years, epoxidized vegetable oils have gathered significant attention as sustainable bio‐based materials for epoxy resin. Vegetable oils have an abundance of unsaturation sites, leading to epoxy‐functionalized molecules. This review provides various materials, synthesis methods, properties, and applications of vegetable oil‐based epoxy resins.
Porous organic polymers from cashew nut shell liquid: Derived carbonized material for carbon dioxide capture
Authors: Krishnan Saumya , Raju Princy Deni , Sujatha Athira R. , Suneesh Chettiyam Veettil 
Published: June 2024

AbstractThe fast developing world is in search of measures to control the accumulation of unused by‐products and waste materials generated during the production of useful commodities. Accordingly, wastes from agro‐industrial sectors can form raw materials for the production of various resources that are extensively used in many areas such as gas sorption and energy production. In the present work, cashew nut shell liquid (CNSL), the vesicant liquid present in the outer shell of cashew seed, forms the source material for the synthesis of porous organic polymers for carbon dioxide capture. Herein, novel types of hypercrosslinked polymers (HCPs) are synthesized via a simple Friedel–Crafts reaction with CNSL as the starting material. Polymerization of natural CNSL with dimethoxymethane/bis‐(chloromethyl)‐biphenyl as external crosslinker provides products in quantitative yield. One of the synthesized HCP is tuned to attain the required high porosity by carbonization at elevated temperatures using an activator. Thus, activated carbon material prepared at 700°C is found to have a high surface area of 880 m2/g with a good carbon dioxide adsorption capacity of 14.72 wt%. These porous materials had moderate isosteric heat of adsorption (23–31 kJ/mol) with excellent recycling capability.
The incompressibility assumption and piezoresistivity in stretchable conductive composites
Authors: Ritchie Logan , Pahl Elke , Anderson Iain A. 
Published: June 2024

AbstractStretchable electronic conductors are vital components in soft robotics and flexible electronics. One method for producing these is combining conductive filler with a nonconductive elastomer. These composites commonly exhibit significant piezoresistivity. This work examines various mechanisms that may underlie this effect. These composites are generally analyzed through percolation theory, which describes the nonlinear relationship between filler volume fraction and conductivity. However, it is unclear whether percolation theory can explain their piezoresistivity or whether mechanisms such as rearrangement of the conductive network under deformation must be considered. This work compares volumetric change in the context of percolation theory against network rearrangement to examine the relative significance of these factors in determining piezoresistivity. Digital image correlation is utilized to investigate volumetric changes in carbon‐black silicone composites and finds that the typical assumption of incompressibility is reasonable, suggesting that volumetric changes alone cannot account for the behavior. A computational model is also developed, which implies that network rearrangement is likely a more significant factor and that interparticle interactions are crucial in understanding this effect. It was found that the most realistic modeling results were achieved only when both rigid and attractive interparticle interactions were accounted for in the model.
One‐pot method for instant nanoformulation via self‐assembling of alginate with quaternary ammonium for controlled release of emamectin benzoate
Authors: Xie Landie , Hou Xiaojun , Yang Limei , Wen Xiaoqiu , Cai Fengjun , Zhang Min , Ma Lin 
Published: June 2024

AbstractTo prompt the nanopesticides from laboratory investigation to agricultural application and reduce the use of pesticides and the impacts on the environment, sodium alginate (SA) was self‐assembled with cetyl trimethyl ammonium bromide (CTAB) into stable and uniform nanoparticle under mild conditions. The structure of the nanoparticle was investigated by dynamic light scattering (DLS), ζ‐potential measurement, Fourier transform infrared spectroscopy (FTIR) and transmission electron microscopy (TEM) and related to the loading and release properties. It was found that the nanoparticle size increased and ζ‐potential decreased with increasing concentration of SA, leading to higher loading efficiency and sustained release efficacy towards emamectin benzoate (EB). The amount of CTAB had little effects on the size of the nanoparticle, however made a competition to EB and reduced the loading and sustaining efficacy of the nanocarrier. The release of EB from SA‐CTAB nanoparticle was pH‐sensitive and was slower in the medium of higher pH value, due to the increased interaction with the nanocarrier. The entrapment into the nanoparticle greatly increased the insecticidal toxicity of EB due to the interaction between the carriers and cell membrane. The median lethal concentration of typical nanoformulation against Mythimna separata larvae was 30% of that of technical EB, after feeding to the insect for 48 h.
Screening, characterization, and optimization of the fermentation conditions of a novel cellulase‐producing microorganism from soil of Qinghai–Tibet Plateau
Authors: Zhao Longmei , Zhang Lan , Qin Yaning , Li Wang , Li Yuanxiao , Cao Hui , Cao Pinghua , Ding Ke , He Wanling 
Published: June 2024

AbstractCellulases play an important role in the bioconversion of lignocellulose. Microorganisms found in extreme environments are a potentially rich source of cellulases with unique properties. Due to the uniqueness of the environment, the abundant microbial resources in the Qinghai–Tibet Plateau (QTP) are worth being explored. The aim of this study was to isolate and characterize an acidic, mesophilic cellulase‐producing microorganism from QTP. Moreover, the fermentation conditions for cellulase production were optimized for future application of cellulase in the development of lignocellulose biomass. A novel cellulase‐producing strain, Penicillium oxalicum XC10, was isolated from the soil of QTP. The cellulase produced by XC10 was a mesophilic cellulase that exhibited good acid resistance and some cold‐adaptation properties, with maximum activity at pH 4.0 and 40°C. Cellulase activity was significantly enhanced by Na+ (p < 0.05) and inhibited by Mg2+, Ca2+, Cu2+, and Fe3+ (p < 0.05). After optimization, maximum cellulase activity (8.56 U/mL) was increased nearly 10‐fold. Optimal fermentation conditions included an inoculum size of 3% (v/v) in a mixture of corn straw (40 g/L), peptone (5 g/L), and Mg2+ (4 g/L), at pH 4.0, 33°C, and shaking at 200 rpm. The specific properties of the P. oxalicum XC10 cellulase suggest the enzyme may serve as an excellent candidate for the bioconversion and utilization of lignocellulose biomass generated as agricultural and food‐processing wastes.
Dimensionality Control of Li Transport by MOFs Based Quasi‐Solid to Solid Electrolyte (Q‐SSEs) for Li−Metal Batteries
Authors: Salado M. , Fernández de Luis R. , Smith T. H. , Hasanpoor M. , Lanceros‐Mendez S. , Forsyth M. 
Published: June 2024

AbstractNowadays, lithium‐ion batteries (LIBs) are widely used in all walks of life and play a very important role. As complex systems composed of multiple materials with diverse chemical compositions, where different electrochemical reactions take place, battery interfaces are essential for determining the operation, performance, durability and safety of the battery. This work, set out to study the incorporation of lithium bis(fluorosulfonyl)amide (LiFSI) doped 1‐ethyl‐3‐methylimidazolium bis(trifluoromethylsulfonyl)imide ([EMIm][TFSI]) ionic liquid into an archetype Ti‐based Metal Organic Framework (MOF) ((Ti) MIL125−NH2) to create a solid to quasi‐solid (depending on the amount of IL in the system), and how it affects not only ionic transport but also the structural properties of the IL/MOF electrolyte. Remarkably high ionic conductivity values (2.13×10−3 S ⋅ cm−1 at room temperature) as well as a lithium transference number (tLi=0.58) were achieved, supported by pulsed field gradient (PFG) NMR experiments. Electrochemical characterization revealed reversible plating‐stripping of lithium and lower overpotential after 750 h at 50 °C. Additionally, a proof‐of‐concept solid state battery was fabricated resulting in a discharge capacity of 160 mAh ⋅ g−1 at 50 °C and 0.1 C rate after 50 cycles. This work presents a suitable strategy to dendrite suppression capability, allowing its implementation as interface modifiers in next‐generation solid‐state batteries.
Framing the Default Option Right
Authors: Meunier Luc , Bashirzadeh Yashar , Ohadi Sima 
Published: June 2024

ABSTRACTDefaults are powerful nudges to shape individuals' behavior: In three experiments, including a large experiment using representative samples from five European countries (n = 4207), we show that they can significantly affect risk‐taking by medium to large effect sizes. We also show that implementing a default nudge leads to a lower rating of the advice delivered by the wealth manager compared to no default, an effect that has a medium to large effect size. In addition, defaults the targeted individuals refuse result in lower advice ratings. These side effects call for caution before applying nudges and individualized defaults rather than a one‐size‐fits‐all approach. Additionally, we find evidence pointing to two small effects of framing in default presentation. First, asking how much individuals want to invest in the risky asset emphasizes risk. It reduces investment in the risky asset compared to asking them how much they want to leave on the safe account, particularly for more risk‐averse individuals. Second, asking individuals if they want to change a default allocation of 100% in the risky asset leads to more investment in the risky asset than asking them whether they accept such an allocation. Perceived wealth manager honesty appears to mediate the relationship between the default nudge and the rating of the advice.
Verbundorientiertes Faserdesign in UHFB
Authors: Lanwer Jan‐Paul , Empelmann Martin 
Published: June 2024

AbstractIm Beitrag wird ein Ansatz für ein verbundorientiertes Faserdesign in ultrahochfestem Beton (UHFB) vorgestellt. Im Gegensatz zur bisherigen, weitgehend auf Erfahrungswerten und Eignungsprüfungen basierenden Methodik zur Wahl von Faserart, Fasergeometrie und Fasermenge werden in dem vorgestellten Ansatz das reale Faserauszugsverhalten in dem gewählten UHFB und mechanisch hinterlegte Effekte aus der Faserorientierung erfasst und berücksichtigt. Die Grundlage bilden Auszugversuche von hochfesten Mikrostahlfasern unter monoton steigender Belastung mit variierender Einbindetiefe und Orientierung. Der hieraus mit Bezug auf die Zugfestigkeit der Faser bestimmte Ausnutzungsgrad bildet den Grundwert für das Faserdesign. Bei schief orientierten Fasern muss darüber hinaus eine zusätzliche Biegebeanspruchung infolge der Umlenkwirkung an der Faseraustrittstelle berücksichtigt werden, die zu einer Erhöhung des Ausnutzungsgrads führt. Die konsistente und mechanisch begründete Berücksichtigung dieser Effekte stellt einen neuartigen Ansatz im Rahmen eines verbundorientierten Faserdesigns unter ökonomischen und ökologischen Gesichtspunkten dar.
Elastizitätsmodul von Recyclingbeton
Authors: Jacobs Frank , Hoffmann Cathleen 
Published: June 2024

AbstractRecyclingbeton kann sich in Festbetoneigenschaften von Beton, der nur mit natürlicher Gesteinskörnung hergestellt wurde, unterscheiden. Deshalb wird in der Schweiz bei der Bestellung von Recyclingbeton die Angabe einer E‐Modulklasse gefordert. Der Elastizitätsmodul ist nach SN EN 12390‐13 zu bestimmen, d. h. an Prüfkörpern, die unter Wasser gelagert werden. Deshalb stellte sich die Frage, ob der an wassergesättigten Prüfkörpern bestimmte Elastizitätsmodul aussagekräftig für Bauteile mit trockenen Expositionsbedingungen ist. Es wurden Untersuchungen an Recyclingbeton mit Beton‐ und Mischgranulat vorgenommen. Hierzu wurden die Prüfkörper bis zur Prüfung unter Wasser gelagert sowie vor der Prüfung getrocknet. Es zeigte sich, dass durch Wasserlagerung der Elastizitätsmodul höher als nach trockener Lagerung ist, die Unterschiede aber nicht sehr groß sind.
Assessing the reinforcement effect by response surface methodology of holocellulose from spent coffee grounds on biopolymeric films as food packaging materials
Authors: Hernández‐Varela Josué David , Chanona‐Pérez José Jorge , Foruzanmehr Reza , Medina Dora Iliana 
Published: June 2024

AbstractThe pollution caused by petroleum‐derived plastic materials has become a major environmental problem that has encouraged the development of new compostable and environmentally friendly materials for food packaging based on biomodified polymers with household residues. This study aims to design, synthesize, and characterize a biobased polymeric microstructure film from polyvinyl alcohol and chitosan reinforced with holocellulose from spent coffee grounds for food‐sustainable packaging. Chemical isolation with a chlorite‐based solution was performed to obtain the reinforced holocellulose from the spent coffee ground, and the solvent casting method was used to obtain the films to study. Physicochemical and microscopic characterizations were conducted to identify and select the best formulations using a simplex‐centroid design analysis. The response surface methodology results indicate that the new packaging material obtained with equal amounts of polymers and reinforced material (1:1:1) possesses the appropriate barrier properties and microstructural character to prevent water attack and hydrophobic behavior and thus could be used as an alternative for food packaging materials.
Gas chromatography mass spectrometer determination of dimethylamine impurity in N,N‐dimethylformamide solvent by derivatization of N,N‐dimethylbenzamide with benzoyl chloride agent
Authors: Kousrali Sayyad , Kowtharapu Leela Prasad , Mondal Tanmoy 
Published: June 2024

AbstractThis study describes the development of a reliable and linear analytical method for precisely determining dimethylamine impurity in N,N‐dimethylformamide solvent utilizing a benzoyl chloride derivatization reagent and a gas chromatography mass spectrometer. Benzoyl chloride was used to derivatize dimethylamine. At normal temperature, benzoyl chloride combined with dimethylamine, producing N,N‐dimethylbenzamide. This method separated N,N‐dimethylbenzamide using Rtx‐5 amine (30 m × 0.32 mm × 1.50 μm) as the stationary phase, helium as the carrier gas, argon as the collision gas, and methanol as the diluent. The column flow rate was 2 mL/min. The retention time of N,N‐dimethylbenzamide was determined to be 8.5 min. Precision, linearity, and accuracy were tested using ICH Q2 (R2) and USP<1225> guidelines. The percentage coefficient of variation (CV) for N,N‐dimethylbenzamide in the system suitability parameter was 1.1%. The correlation coefficient of N,N‐dimethylbenzamide was found to be >0.99. In the method precision parameter, the % CV for N,N‐dimethylbenzamide was found to be 1.9%, whereas the % CV for N,N‐dimethylbenzamide was 1.2% in intermediate precision. The percentage recovery of N,N‐dimethylbenzamide was determined to be between 80% and 98%.