bluuebunny/crossref_metadata_2025_split huggingface dataset:
Identification of Novel Variants in the NHS in Four Turkish Patients With Nance–Horan Syndrome
Authors: Alavanda Ceren , Arslan Ateş Esra , Demir Şenol , Polat Hamza , Hanoğlu Onur , Arman Ahmet , Geçkinli Bilgen Bilge 
Published: February 2025

ABSTRACTNance–Horan Syndrome (NHS) is an ultra‐rare syndrome characterized by facial dysmorphism, teeth, and eye abnormalities. Pathogenic variants in NHS are responsible for NHS. Herein, we report four Turkish patients from two unrelated families having dysmorphic facial features, congenital bilateral cataracts, and dental anomalies. Molecular studies revealed two novel hemizygous (c.136_137del; p.Leu46Glyfs*136 and c.2690del; p.Thr897Serfs*19) variants in the NHS inherited from their mothers. In family 1, the mother and her children also carried a heterozygous known c.645G>A (p.Trp215*) AIPL1 variant, which explains the cone‐rod dystrophy (CRD). This is the first report documenting the coexistence of NHS and AIPL1‐related CRD.
Optimization analysis of epoxidation of silkworm pupae oil with citric acid and eco‐friendly oil coating on mild steel for corrosion protection
Authors: Nandhini Dhanavel , Halada Nandakrishnan Meenakshi , Viswanatha Ramarao 
Published: February 2025

AbstractSilkworm pupae oil (SPO) with high amount of unsaturation due to rich in oleic acid and α‐linolenic acids has been chosen for this investigation. Epoxidized silkworm pupae oil (ESPO) was produced by in situ generated peroxycitric with citric acid (CA) and hydrogen peroxide (H2O2). Literature covering the topic of epoxidation, including the catalytic aspect, is vast. No review articles have been written on the synthesis of epoxides from SPO without the catalyst to the best of our knowledge. Here, CA itself behaves as an oxygen carrier without any catalyst and H2O2 as an oxygen donor, which favors the epoxidation reaction. The epoxidation process was optimized by factors like temperature, time, and CA ratio. Fourier transform infrared (FTIR) analysis confirmed the presence of epoxy groups and the absence of (CC) bonds in ESPO. Optimal conditions for epoxidation were 70 °C, 0.75 mol/L CA, and 7 h, resulting in 92.1% double bond conversion (DBC) and 84.7% relative oxirane conversion (RC). The polymerized ESPO coated on mild steel (MS) exhibited enhanced corrosion resistance, with inhibition efficiency rising from 83.86% to 99.42% due to effective pore prevention and ion modification during curing.
Spatial and temporal dependence in distribution‐based evaluation of CMIP6 daily maximum temperatures
Authors: Virdee Mala , Kazlauskaite Ieva , Boland Emma J. D. , Shuckburgh Emily , Ming Alison 
Published: February 2025

AbstractClimate models are increasingly used to derive localised, specific information to guide adaptation to climate change. Model projections of future scenarios are conferred credibility by evaluating model skill in reproducing large‐scale properties of the observed climate system. Model evaluation at fine spatial and temporal scales and for rare extreme events is critical for provision of reliable adaptation‐relevant information, but may be challenging given significant internal variability and limited observed data in this setting. Comparing distributions of physical variables from historical simulations of Coupled Model Intercomparison Project models against observed distributions provides a comprehensive, concise and physically‐justified skill measure. Calculating divergence between distributions requires aggregation of data spatially or temporally. The spatial and temporal scales at which a divergence measure converges to a consistent value can indicate the scales at which a well‐defined climate signal emerges from internal variability. Below this threshold, there may be insufficient data for robust evaluation, particularly for rare extremes. Here, the behaviour of several divergence measures in response to spatial and temporal aggregation is analysed empirically to give a novel evaluation of CMIP6 daily maximum temperature simulations against reanalysis. Some key insights presented here can inform methodological choices made when deriving adaptation‐relevant information. Convergence varies according to model, geographic region and divergence measure; selection of the level of precision at which models can provide reliable information therefore requires a context‐specific understanding. For this purpose, an interactive tool provided alongside this study demonstrates scale‐dependent evaluation across several geographic regions. Commonly applied measures are found to be only weakly sensitive to discrepancies in the tails of distributions.
The Childhood Autism Rating Scale Second Edition (CARS2) and Its Applicability in an Iranian Sample
Authors: Samadi Sayyed Ali , Mahmoodizadeh Ameneh , Foladgar Mehdi , Moradi Shahnaz Bakhshalizadeh , Lotfi Baran , McConkey Roy 
Published: February 2025

ABSTRACTThe present study aimed to evaluate the reliability and validity of the Childhood Autism Rating Scale: Second Edition (CARS2) in diagnosing individuals with autism in Iran. A mixed‐method approach was used and 313 participants were recruited, with an age range of 2–32 years, for CARS2‐Standard Form (ST) and 218 individuals aged 6–25 years for CARS2‐High Functioning (HF). The participants were recruited from daycare centers, schools, and clinics with different developmental trajectories: autism, intellectual disabilities, and neurotypical development. All participants with autism and intellectual disabilities had been clinically diagnosed previously. In addition, the CARS2‐Questionnaire of Parent Concerns (QPC) was used to gather qualitative data on 30 randomly selected parents and the perspectives of the 20 test administrators were also collected. The CARS2 had high internal consistency, inter‐rater reliability, and test–retest reliability. Factor analyses revealed a one‐factor structure for CARS2‐ST and a three‐factor structure for CARS2‐HF. When adjustments were made to the cut‐off points, the discriminant analyses indicated that CARS2 effectively differentiated between those with autism and typical development but less so with persons who had intellectual disabilities. The qualitative data analysis and the extracted themes suggest that the CARS2‐QPC is a valid tool for collecting autism‐related information from parents. Our findings suggest that the CARS2 is broadly a reliable and valid instrument for diagnosing autism spectrum in Iran in the absence of more extensive assessments.
Prognostic and Diagnostic Value of Platelet Distribution Width in COPD Patients with Pulmonary Hypertension: A Retrospective Study
Authors: Xie Xiang , Wang Yan , Tian Suochen , Cao Dongming 
Published: February 2025

ABSTRACTThe objective of the study was to investigate the value of platelet distribution width (PDW) as a prognostic biomarker by comparing PDW between COPD patients with pulmonary hypertension (PH) (PASP 50 mmHg) and those without PH (PASP < 50 mmHg) and to explore its diagnostic and predictive value in this population. A retrospective study of 270 COPD patients in Liaocheng People's Hospital (July 2018 to July 2019) was done by dividing them into two groups according to pulmonary artery systolic pressure (PASP): COPD‐only (PASP <50 mmHg) and COPD with PH (PASP ≥50 mmHg). Routine blood tests, C‐reactive protein, pulmonary function tests, echocardiography, Chronic Obstructive Pulmonary Disease Assessment Questionnaire (CAT), Clinical COPD Questionnaire were performed. PDW was higher in COPD with PH group than COPD‐only group. After adjusting for confounders including age, smoking history, CAT scores, white blood cell count, PDW, and NT‐proBNP in COPD with PH, PDW was positively correlated with various parameters. PDW can diagnose COPD with PH, also prognostic value and cardiovascular distinction in these patients. The study concluded that PDW is a prognostic marker, reflecting pulmonary and cardiovascular physiology in COPD patients with PH. It can be used for early diagnosis, risk stratification, and individualized management for this high‐risk population.
Readiness for Mandatory Climate‐Related Disclosures: A Tri‐Jurisdictional Analysis of Governance Attributes in Australia, New Zealand and the United Kingdom
Authors: Moses Olayinka , Bui Binh , Houqe Muhammad Nurul , Borghei Zahra 
Published: February 2025

ABSTRACTWe evaluate the preparedness of companies in Australia, New Zealand and the United Kingdom to comply with emerging mandatory climate‐related disclosures (CRDs) aligned with TCFD recommendations, using their Carbon Disclosure Project (CDP) information. Our analysis also examines the corporate governance attributes influencing their readiness to disclose such information. The findings reveal a strong integration of the Governance aspect of TCFD‐recommended disclosure, with an 86% alignment between CDP and TCFD disclosures in the Governance theme. However, lower alignment is observed for Strategy (50%) and Metrics and Targets (49%), highlighting the need for immediate improvements in these areas. Firms with more gender‐diverse boards and the presence of a sustainability committee demonstrate greater readiness to comply with CRDs consistent with TCFD recommendations. These insights shed light on firms' readiness for emerging mandatory CRD across jurisdictions, especially considering the new IFRS sustainability standards. The results underscore the urgent need to enhance competencies in Strategy and Metrics and Targets, where alignment is weakest. Practically, by documenting these insights, we provide managers with early guidance on the implications of their current CRD practices. This is especially relevant for firms subject to, or soon to be impacted by, mandatory sustainability regulations in their jurisdictions. The findings hold paramount significance for managers, policymakers and regulators.
Antecedents and Consequences of Viewers' Engagement Toward Vlogging
Authors: Banik Shanta , Giri Arunangshu , Chatterjee Satakshi 
Published: February 2025

ABSTRACTVlogging is increasingly popular for sharing personalized experiences, yet research on audience engagement and behavior is limited. This study, guided by the elaboration likelihood model (ELM), examines the impact of information quality and source credibility on viewer engagement and their behaviors in food (Study 1) and travel vlogs (Study 2). Data are collected from 424 food and 442 travel vlog viewers in India using structured surveys and snowball sampling. Results indicate that information quality and source credibility significantly influence viewer engagement, which affects their social networking and patronage behaviors, with no notable role of gender in these relationships. This research contributes valuable insights to vlogging, highlighting how audience engagement drives behavior and creates value.
In Silico Study, Synthesis, and In Vitro Evaluation of Acetylcholinesterase and Butyrylcholinesterase Inhibitory Activity of Novel N‐Thiazole Substituted Acetamide Coumarin Derivatives
Authors: Patowary Pooja , Shakya Anshul , Ghosh Surajit Kumar , Jamir Lipoksangla , Sahariah Bhargab Jyoti , Gogoi Neelutpal , Singh Udaya Pratap , Bhat Hans Raj 
Published: February 2025

ABSTRACTIn this study, a structurally directed pharmacophore hybridization technique is used to combine the two essential structural scaffolds coumarin and thiazoles in search of a new class of acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE) inhibitor for Alzheimer's disease (AD). A library of 120 compounds was designed in two series 5a(1‐15), 5b(16‐30), 5c(31‐45), 5d(46‐60), and 6a(61‐75), 6b(76‐90), 6c(91‐105), 6d(106‐120) using various substituted phenol, β‐ketoesters, and thiazole derivatives. Eleven compounds were identified as potential hybrids using molecular property filter analysis and molecular docking studies, and they comprise N‐substituted thiazole coumarin derivatives. The docking results indicated that compounds 5b16 and 5c35 exhibited strong binding interactions with GLY116, GLY117, TYR332, and HIS438 (ranging from −27.42 to −24.18 kcal/mol) and GLY119, ASP72, and PHE288 (ranging from −32.21 to −25.92 kcal/mol) when tested against AChE (1EVE) and BuChE (1P0I) inhibitors. These compounds were synthesized via conventional methods and characterized by different spectroscopic methods. In vitro anti‐cholinesterase activity results indicated that two compounds, for example, 5b16 and 5c35 showed potent to moderate activity against AChE and BuChE with IC50 (2.00 ± 0.09–29.63 ± 0.48) µM and (34.93 ± 0.62–17.92 ± 0.42) µM, respectively. Our study demonstrated the development of a novel class of hybrid coumarin thiazole derivatives as AChE and BuChE inhibitors, and these compounds could be utilized against ADs.
Percutaneous Transluminal Angioplasty Balloon‐Assisted Adjacent Vascular Connection Without Stent Protection: Case Report
Authors: Sun Xiaoyi , Shao Xiang , Kong Weixin , Liu Hejing , Li Xiang 
Published: February 2025

ABSTRACTThe aneurysmal dilation and excessive blood flow of the cephalic vein caused by the relative stenosis of the cephalic venous arch are important challenges in the maintenance of arteriovenous fistulas, and the use of adjacent vascular connection without stent protection has shown great advantages over traditional open surgery.
Accurate Determination of Molecular Sizes of a Solute in Water From its Translational Self‐Diffusion Coefficient
Authors: Zaccaria Francesco , Macchioni Alceo , Zuccaccia Cristiano 
Published: February 2025

AbstractDetermining accurate molecular dimensions in water, from measured translational self‐diffusion coefficients (Dt), is extremely important in biochemistry, supramolecular chemistry, organometallic chemistry and beyond, but it still represents a big challenge especially for small and medium‐sized molecules. Indeed, current semiempirical adaptations of the Stokes‐Einstein equation, which allow accurate determination of molecular size of solutes in organic solvents, proved inadequate for aqueous systems. To overcome such a major limitation, herein, we introduce a novel approach that unlocks the quantitative interpretation of Dt in water. By analyzing ~70 diverse molecules with volumes ranging from 101 Å3 to 103 Å3, and selecting the partial molar radius (rM) as a reliable proxy for the hydrodynamic radius (rH), we derived a semiempirical equation that enables accurate determination of hydrodynamic volume (VH) of solutes in aqueous solutions, effectively accounting for the distinctive hydrogen‐bonding properties of water. This approach fills a crucial gap, enhancing precise molecular characterization of polar and non‐polar solutes in water.
Bioavailability, Metabolism, and Excretion of [14C]‐Tazemetostat in Patients With B‐Cell Lymphomas or Advanced Solid Tumors
Authors: Chen Yingxue , Teng Renli , Ogier Julien 
Published: February 2025

AbstractThis open‐label, multicenter study (NCT03010982) evaluated the absolute bioavailability, characterized the disposition and metabolism, and investigated the metabolic profile of tazemetostat, a US Food and Drug Administration–approved inhibitor of enhancer of zeste homolog 2, following intravenous and oral [14C]‐labeled and unlabeled tazemetostat in patients with B‐cell lymphomas or advanced solid tumors. Patients received oral tazemetostat 800 mg twice daily for 14 days. On Day 15, patients received tazemetostat 800‐mg tablets in a fasted state followed by an intravenous microdose of 12 µg [14C]‐tazemetostat. On Day 16, patients received a [14C]‐tazemetostat 800‐mg solution with a meal, then continued tazemetostat 800 mg twice daily. Blood, plasma, urine, and fecal samples were collected for pharmacokinetic analyses, and recovery and excretion of the radioactivity of [14C]‐labeled/unlabeled tazemetostat and its metabolites. The median absolute bioavailability was 31.8% (range, 20.2%‐49.8%). Notable plasma components were EPZ‐6930, unchanged tazemetostat, EPZ006931, and EPZ034163, accounting for 31.8%, 22.4%, 11.0%, and 3.5% of total drug‐related exposure, respectively. Recovery of radiolabeled material ranged from 93.2% to 94.7%, with most excreted doses recovered within 48 hours in urine and by 96 hours in feces. Fecal elimination represented the principal route of elimination with a mean of 78.9% of the administered radioactive dose and renal excretion accounted for 15.4%.
Redox‐Responsive Side Chain Structural Changes in a Seven‐Membered Cyclic α,α‐Disubstituted α‐Amino Acid with a Disulfide Bond Enable Reversible Conformational Changes in Peptides
Authors: Oba Makoto , Nonaka Hikaru , Umeno Tomohiro , Kato Takuma , Doi Mitsunobu , Ueda Atsushi , Tanaka Masakazu 
Published: February 2025

AbstractWe report the development of a redox‐responsive system that induces reversible conformational changes in peptides through the design of a seven‐membered cyclic α,α‐disubstituted α‐amino acid with a disulfide bond, 5‐amino‐1,2‐dithiepane‐5‐carboxylic acid (Dtp). Upon reduction, the disulfide bond in Dtp was cleaved to form thiols, converting Dtp into (2‐mercaptoethyl)homocysteine (Mhc), and this process was reversed by oxidation. Dtp‐containing peptides predominantly adopted 310‐helical conformation in solution, whereas Mhc‐containing peptides exhibited a mixture of helical and other conformations. This redox‐responsive mechanism allows for precise control over peptide secondary structures, making it a promising approach for designing functional helical peptides capable of acting molecular switches in response to intracellular reductive environments.
Deacidification of Coconut Oil Using Different Resins: A Comparative Study
Authors: K. P. Bhumika , V. P. Mayookha , K Arun Sharma , G. Suresh Kumar 
Published: February 2025

ABSTRACTIn the food sector, deacidification of vegetable oil is essential for enhancing edible oil's stability, quality, and shelf life. It is still difficult to come up with an efficient deacidification method that makes use of high‐acid‐value oil to produce edible goods that are both high‐quality and value‐added. The present work provides a newly developed, adaptable, and long‐lasting ion exchange deacidification method. Three different ion exchange resins, that is, Amberlite IRA 400 (AMT), Amberlyst A26 (A26), and Dowex WGR‐2 (DOX) at different concentrations were assessed to remove free fatty acids (FFAs) by adsorption using a column, where AMT (85.92%) and A26 (98.02%) showed a good result compared to DOX (75.4%). To further confirm the reduction in the acid value of the treated oil, multiple methods were used, including FFA measurement by titration, gas chromatography (GC), high‐performance liquid chromatography (HPLC), and attenuated total reflectance Fourier‐transform infrared spectroscopy (ATR‐FTIR). All these methods consistently demonstrated a reduction in FFA levels in the oil collected after treatment with resin. Additionally, to assess the effect of the resin on the oil, various physicochemical parameters were analyzed. It was found that most of the tocopherols and tocotrienols were preserved, and there was little to no impact on other key quality parameters.Practical Applications: Our study offers valuable insights into the development of a deacidification technology for removing FFAs from oil using resins. This environment‐friendly method effectively tackles the challenge of high acidity in oils by efficiently adsorbing FFAs. The use of resins provides an efficient solution while retaining the nutritional properties of the oil, making it an appealing alternative to traditional methods. This technology holds significant potential for industrial applications, such as improving the quality and shelf life of edible oils by removing FFA, as well as reducing waste during alkali treatment. Implementing this technique can help promote better practices in the oil processing industry.
A Novel Strategy for Authentication and Quantification of Porcine and Water Buffalo Hide Gelatin in Processed Products Using Multiple Reaction Monitoring Mass Spectrometry Approaches
Authors: Mishra Bidyut Prava , Maheswarappa Naveena B. , Rao B. Eswara , Banerjee Rituparna , Govindaiah Prasad M. , Manohar Balaji B. , Rath Prasana Kumar 
Published: February 2025

ABSTRACTPig skin and water buffalo hides were partially hydrolyzed to produce a heterogeneous mixture of polypeptides, known as gelatin, a commonly used food additive. Specific peptide markers were explored in the context of authentication and relative quantification of water buffalo hide gelatin (BHG) and porcine skin gelatin (PSG) via multiple reaction monitoring‐mass spectrometry (MRM‐MS). Tryptic gelatin peptides were separated and analyzed with ultra‐performance liquid chromatography‐tandem mass spectrometry (UPLC‐MS/MS), and five unique peptide biomarkers were selected for BHG and PSG. Gelatin extraction from BHG and PSG spiked chicken meat patties (CMP) followed by LC‐MS/MS MRM‐MS revealed two unique bovine gelatin peptides of m/z 781.336 and 852.718 and three unique porcine gelatin peptides of m/z 774.570, 971.776, and 727.436. The optimized MRM‐MS protocol was efficient in detecting BHG/PSG from spiked CMP up to 0.5% (w/w). Twenty‐five commercial food samples were screened, among which four samples showed the presence of BHG and six samples showed the presence of PSG. The proposed LC‐MS/MS MRM strategy provides an efficient and sensitive authentication and traceability of gelatin‐containing highly processed food, bakery, and confectionery products.
Multidimensional Spatiotemporal Clustering – An Application to Environmental Sustainability Scores in Europe
Authors: Morelli Caterina , Boccaletti Simone , Maranzano Paolo , Otto Philipp 
Published: February 2025

ABSTRACTThe assessment of corporate sustainability performance is extremely relevant in facilitating the transition to a green and low‐carbon intensity economy. However, companies located in different areas may be subject to different sustainability and environmental risks and policies. Henceforth, the main objective of this paper is to investigate the spatial and temporal pattern of the sustainability evaluations of European firms. We leverage a large dataset containing information about companies' sustainability performances, measured by MSCI ESG ratings, and geographical coordinates of firms in Western Europe between 2013 and 2023. By means of a modified version of the Chavent et al. (2018) hierarchical algorithm, we conduct a spatial clustering analysis, combining sustainability and spatial information, and a spatiotemporal clustering analysis, which combines the time dynamics of multiple sustainability features and spatial dissimilarities, to detect groups of firms with homogeneous sustainability performance. We are able to build cross‐national and cross‐industry clusters with remarkable differences in terms of sustainability scores. Among other results, in the spatio‐temporal analysis, we observe a high degree of geographical overlap among clusters, indicating that the temporal dynamics in sustainability assessment are relevant within a multidimensional approach. Our findings help to capture the diversity of ESG ratings across Western Europe and may assist practitioners and policymakers in evaluating companies facing different sustainability‐linked risks in different areas.