bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical, Radiological and Pathological Features of a Large American Cohort of Spinocerebellar Ataxia (SCA27B)
Authors: Abou Chaar Widad , Eranki Anirudh N. , Stevens Hannah A. , Watson Sonya L. , Wong Darice Y. , Avila Veronica S. , Delfeld Megan , Gary Alexander J. , Tawde Sanjukta , Triebold Malia , Cherchi Marcello , Xie Tao , Lockhart Paul J. , Bahlo Melanie , Pellerin David , Dicaire Marie‐Josée , Danzi Matt , Zuchner Stephan , Brais Bernard C. , Perlman Susan , Burmeister Margit , Paulson Henry , Srinivasan Sharan , Schut Lawrence , Bower Matthew , Bushara Khalaf , Liao Chuanhong , Shakkottai Vikram G. , Collins John , Clark H. Brent , Das Soma , Fogel Brent L. , Gomez Christopher M. 
Published: September 2024

ObjectivesSpinocerebellar ataxia 27B due to GAA repeat expansions in the fibroblast growth factor 14 (FGF14) gene has recently been recognized as a common cause of late‐onset hereditary cerebellar ataxia. Here we present the first report of this disease in the US population, characterizing its clinical manifestations, disease progression, pathological abnormalities, and response to 4‐aminopyridine in a cohort of 102 patients bearing GAA repeat expansions.MethodsWe compiled a series of patients with SCA27B, recruited from 5 academic centers across the United States. Clinical manifestations and patient demographics were collected retrospectively from clinical records in an unblinded approach using a standardized form. Post‐mortem analysis was done on 4 brains of patients with genetically confirmed SCA27B.ResultsIn our cohort of 102 patients with SCA27B, we found that SCA27B was a late‐onset (57 ± 12.5 years) slowly progressive ataxia with an episodic component in 51% of patients. Balance and gait impairment were almost always present at disease onset. The principal finding on post‐mortem examination of 4 brain specimens was loss of Purkinje neurons that was most severe in the vermis most particularly in the anterior vermis. Similar to European populations, a high percent of patients 21/28 (75%) reported a positive treatment response with 4‐aminopyridine.InterpretationOur study further estimates prevalence and further expands the clinical, imaging and pathological features of SCA27B, while looking at treatment response, disease progression, and survival in patients with this disease. Testing for SCA27B should be considered in all undiagnosed ataxia patients, especially those with episodic onset. ANN NEUROL 2024;96:1092–1103
Peptide‐Induced Division of Polymersomes for Biomimetic Compartmentalization
Authors: Bremm Madalosso Heloísa , Cao Shoupeng , Ivanov Tsvetomir , de Souza Melchiors Marina , Koynov Kaloian , Guindani Camila , Hermes de Araújo Pedro Henrique , Sayer Claudia , Landfester Katharina , Caire da Silva Lucas 
Published: September 2024

AbstractPolymersomes are synthetic vesicles that mimic the architecture of cellular compartments such as the cell membrane and organelles. These biomimetic compartments facilitate the creation of cell‐like chemical systems, including microreactors and synthetic organelles. However, the construction of hierarchical multi‐compartment systems remains challenging and typically requires the encapsulation of pre‐formed vesicles within a host compartment. Here, we report the formation of multicompartment polymersomes with a vesicle‐in‐vesicle architecture achieved through self‐division induced by short peptides incorporated into the vesicle membrane. A phenylalanine‐phenylalanine‐methionine (FFM) tripeptide was designed and encapsulated into the polymersome via microfluidics. We demonstrate that vesicle self‐division occurs due to peptide incorporation into the membrane in response to pH changes. This self‐division creates internal vesicles capable of colocalizing enzymes. The hybrid polymer‐peptide system described here provides a straightforward method for developing subcompartmentalized systems, paving the way for engineering microreactors with life‐like properties.
Reversible Structural Phase Transitions in Zero‐Dimensional Cu(I)‐Based Metal Halides for Dynamically Tunable Emissions
Authors: An Ran , Wang Qishun , Liang Yuan , Du Pengye , Lei Pengpeng , Sun Haizhu , Wang Xinyu , Feng Jing , Song Shuyan , Zhang Hongjie 
Published: September 2024

AbstractExploring structural phase transitions and luminescence mechanisms in zero‐dimensional (0D) metal halides poses significant challenges, that are crucial for unlocking the full potential of tunable optical properties and diversifying their functional capabilities. Herein, we have designed two inter‐transformable 0D Cu(I)‐based metal halides, namely (C19H18P)2CuI3 and (C19H18P)2Cu4I6, through distinct synthesis conditions utilizing identical reactants. Their optical properties and luminescence mechanisms were systematically elucidated by experiments combined with density functional theory calculations. The bright cyan‐fluorescent (C19H18P)2CuI3 with high photoluminescence quantum yield (PLQY) of 77 % is attributed to the self‐trapped exciton emission. Differently, the broad yellow‐orange fluorescence of (C19H18P)2Cu4I6 displays a remarkable PLQY of 83 %. Its luminescence mechanism is mainly attributed to the combined effects of metal/halide‐to‐ligand charge transfer and cluster‐centered charge transfer, which effects stem from the strong Cu−Cu bonding interactions in the (Cu4I6)2− clusters. Moreover, (C19H18P)2CuI3 and (C19H18P)2Cu4I6 exhibit reversible structural phase transitions. The elucidation of the phase transitions mechanism has paved the way for an unforgeable anti‐counterfeiting system. This system dynamically shifts between cyan and yellow‐orange fluorescence, triggered by the phase transitions, bolstering security and authenticity. This work enriches the luminescence theory of 0D metal halides, offering novel strategies for optical property modulation and fostering optical applications.
Strain‐Release‐Driven Electrochemical Skeletal Rearrangement of Non‐Biased Alkyl Cyclopropanes/Butanes
Authors: Qi Jing , Wang Chu , Wang Gan , O'Neill Patrick , Reddy Dubbaka Srinivas , Ting Ang Hwee , Chen Xuebo , Wu Jie 
Published: September 2024

AbstractCapitalizing the inherent strain energy within molecules, strain‐release‐driven reactions have been widely employed in organic synthesis. Small cycloalkanes like cyclopropanes and cyclobutanes, with their moderate ring strain, typically require dense functionalization to induce bias or distal activation of (hetero) aromatic rings via single‐electron oxidation for relieving the tension. In this study, we present a pioneering direct activation of alkyl cyclopropanes/butanes through electrochemical oxidation. This approach not only showcases the potential for ring‐opening of cyclopropane/butane under electrochemical conditions but also streamlines the synthesis of diverse oxazolines and oxazines. The applicability of our method is exemplified by its broad substrate scopes. Notably, the products derived from cyclobutanes undergo a formal ring contraction to cyclopropanes, introducing an intriguing aspect to our discoveries. These discoveries mark a significant advancement in strain‐release‐driven skeletal rearrangement reactions of moderately strained rings, offering sustainable and efficient synthetic pathways for future endeavours.
Complexity‐Building Exhaustive Dearomatization of Benzenoid Aromatics within an ESIPT‐Initiated Three‐Step Photochemical Cascade.
Authors: Beduru Srinivas , Huple Deepak B. , Kutateladze Andrei G. 
Published: September 2024

AbstractDearomative cycloadditions offer rapid access to complex 3D molecular architectures, commonly via a sp2‐to‐sp3 rehybridization of two atoms of an aromatic ring. Here we report that the 6e π‐system of a benzenoid aromatic pendant could be exhaustively depleted within a single photochemical cascade. An implementation of this approach involves the initial dearomative [4+2] cycloaddition of the Excited State Intramolecular Proton Transfer (ESIPT)‐generated azaxylylene, followed by two consecutive [2+2] cycloadditions of auxiliary π moieties strategically positioned in the photoprecursor. Such photochemical cascade fully dearomatizes the benzenoid aromatic ring, saturating all six sp2 atoms to yield a complex sp3‐rich scaffold with high control of its 3D molecular shape, rendering it a robust platform for rapid systematic mapping of underexplored chemical space. Significant growth of molecular complexity—starting with a modular synthesis of photoprecursors from readily available building blocks—is quantified by Böttcher score calculations.
Diffusion and sorption of water vapor by polyamide‐imides
Authors: Chalykh Anatoly E. , Petrova Tatiana F. , Matveev Vladimir V. , Nikulova Uliana V. 
Published: September 2024

AbstractThe study of sorption and diffusion characteristics of glassy polymers based on polyamide‐imides was carried out using the methods of static sorption, dilatometry, electron microscopy. Water sorption isotherms of polyamide‐imides based on trimellitimido‐N‐acetic acid (PAI‐A) and trimellitimido‐N‐p‐benzoic acid (PAI‐B) with different intramolecular ‘hinges’ in the diamine fragment were obtained. The isotherms are S‐shaped and occupy an intermediate position between polyheteroarylenes and aliphatic polyamides. The dual sorption model was used to interpret the results. It is shown that the Langmuir component of the isotherms is determined by the thermal prehistory of glassy polymers, while the sorption component associated with water dissolution according to Flory‐Huggins is determined by the chemical nature of the functional groups included in the monomeric unit. The diffusion coefficients of water and the temperature and concentration dependences of the diffusion coefficients of sorbed water molecules were determined.
Community‐Engaged Research and the Use of Open Access ToxVal/ToxRef In Vivo Databases and New Approach Methodologies (NAM) to Address Human Health Risks From Environmental Contaminants
Authors: Silva Marilyn , Capps Shosha , London Jonathan K. 
Published: September 2024

ABSTRACTBackgroundThe paper analyzes opportunities for integrating Open access resources (Abstract Sifter, US EPA and NTP Toxicity Value and Toxicity Reference [ToxVal/ToxRefDB]) and New Approach Methodologies (NAM) integration into Community Engaged Research (CEnR).MethodsCompTox Chemicals Dashboard and Integrated Chemical Environment with in vivo ToxVal/ToxRef and NAMs (in vitro) databases are presented in three case studies to show how these resources could be used in Pilot Projects involving Community Engaged Research (CEnR) from the University of California, Davis, Environmental Health Sciences Center.ResultsCase #1 developed a novel assay methodology for testing pesticide toxicity. Case #2 involved detection of water contaminants from wildfire ash and Case #3 involved contaminants on Tribal Lands. Abstract Sifter/ToxVal/ToxRefDB regulatory data and NAMs could be used to screen/prioritize risks from exposure to metals, PAHs and PFAS from wildfire ash leached into water and to investigate activities of environmental toxins (e.g., pesticides) on Tribal lands. Open access NAMs and computational tools can apply to detection of sensitive biological activities in potential or known adverse outcome pathways to predict points of departure (POD) for comparison with regulatory values for hazard identification. Open access Systematic Empirical Evaluation of Models or biomonitoring exposures are available for human subpopulations and can be used to determine bioactivity (POD) to exposure ratio to facilitate mitigation.ConclusionsThese resources help prioritize chemical toxicity and facilitate regulatory decisions and health protective policies that can aid stakeholders in deciding on needed research. Insights into exposure risks can aid environmental justice and health equity advocates.
pH‐responsive persistent luminescence nanoprobes biosensor for autofluorescence‐free determination of glucose level in human samples and fingerprint encryption
Authors: Ma Xiaohu , Zhu Runzhi , Zhang Hui , Meng Zhe 
Published: September 2024

AbstractGlucose level is an important indicator of diabetes, and maintaining an appropriate physiological concentration of glucose is important for human health. However, traditional optical sensors are interfered by the interference of strong background autofluorescence and natural responsive luminescence, which severely limits their application in complex biological samples. Herein, as a novel glucose biosensing probe, green‐emitting Zn2GeO4:Mn2+, Eu3+ (ZGME) persistent luminescence nanoparticles (PLNPs) with pH stimulus‐responsive was prepared by a facile one‐pot hydrothermal method. We also investigated the pH stimulus‐responsive luminescence behaviour of ZGME over a range of pH values from 2.8 to 8.0. Taking advantage of the interesting property that ZGME photoluminescence intensity has a pH response, within an extraordinarily narrow pH range of 5.0–6.5 for highly selectivity and sensitive determination of glucose level in human samples by acid‐responsive quenching and persistent luminescent performance. The detection results show high accuracy of the measured values of glucose in serum with a wide detection range (2.5 μg L−1‐10 mg L−1) and low detection limit (0.5 μg L−1). Finally, the pH‐responsive persistent luminescence also makes ZGME promising for high‐level fingerprint information encryption. Hence, the established pH stimulation‐responsive PLNPs‐based biosensing probe offers excellent performance with high selective, accuracy and signal‐to‐noise ratio for detection of glucose level in human samples.
Social determinants of health on attitudes toward childbearing among women with multiple sclerosis: A cross‐sectional study
Authors: Khoshsirat Nahid Abbasi , Mokaram Romina , Mahmoodi Zohreh , Shahrestanaki Ehsan , Ghavidel Nooshin 
Published: September 2024

AbstractBackgroundPregnancy and motherhood are very valuable but challenging for women with multiple sclerosis (MS). Given that there are limited studies in this field, this study aimed to determine the social determinants of health on attitudes toward childbearing among women with MS.MethodsWe conducted a cross‐sectional study on 206 women with MS in Alborz province, Iran, from February to June 2023 using convenience sampling. The data were collected using the questionnaire, and a linear regression analysis was applied.ResultsThe mean age of the participants was 36.80 ± 6.50 years. Participants’ Attitudes toward Fertility and Childbearing (PAFC) had a positive significant association with social support (B = .10, SE = .04, p = .023) and a significant negative association with the total score of depression, anxiety, and stress scale (B = −.13, SE = .06, p = .047) and depression (B = −.40, SE = .18, p = .023). However, the association between anxiety (B = −.25, SE = .20, p = .211), stress (B = −.36, SE = .18, p = .050), MS severity (B = .04, SE = .30, p = .890), and socioeconomic status scale (SES) (B = −.08, SE = .32, p = .806) was nonsignificant with PAFC.ConclusionOur results showed that factors, including social support and mental health especially depression, can affect PAFC in women with MS. Therefore, it is necessary to determine specific strategies for policymakers to help MS patients manage pregnancy and motherhood.
SGLT‐2 inhibitors and high‐dose acarbose as potential high‐risk combinations for ketosis and ketoacidosis in Asian patients with T2DM: A case series
Authors: Qiang Wei , Yang Fei , Liu Ling , Dong Ruiqing , Sun Yushi , Mondal Ahona , Guo Hui 
Published: September 2024

Key Clinical MessageHigh‐dose acarbose may increase the risk of diabetic ketosis/diabetic ketoacidosis in Asian patients on sodium‐glucose cotransporter‐2 inhibitors. Healthcare providers and patients should be cautious to avoid this combination.AbstractLow‐calorie diets should be avoided in patients receiving sodium‐glucose cotransporter‐2 (SGLT‐2) inhibitors to decrease the risk of diabetic ketoacidosis (DKA). High‐dose acarbose can decelerate carbohydrate absorption. We detail three cases of diabetic ketosis (DK) following concurrent SGLT‐2 inhibitor and high‐dose acarbose therapy (acarbose 300 mg/day and dapagliflozin 10 mg/day). Patients, aged 38–63 years with 3–10 years of type 2 diabetes mellitus (T2DM), developed DK, indicated by moderate urinary ketones and high glucose (urine ketone 2+ to 3+ and glucose 3+ to 4+) without acidosis, within 4 days to 1 month post‐therapy initiation. Serum glucose was 172.8–253.8 mg/dL; HbA1c was 9.97%–10.80%. The combination therapy was halted, and DK was managed with low‐dose intravenous insulin and fluids, followed by intensive insulin therapy. High‐dose acarbose with SGLT‐2 inhibitors may increase the risk of DK/DKA in Asian patients.
Metastatic hepatocellular carcinoma presenting as a lytic calvarial mass
Authors: Parraga Shirley , Pasli Melisa , Behm Hayley , Zeches Breann , Reddy Revanth 
Published: September 2024

Key Clinical MessageHepatocellular carcinoma (HCC) is one of the most common types of cancer. More focus is being placed on clinical presentations of distant metastasis from HCC. Head masses should be investigated even when no concerning symptoms are found in patients with prior history of alcohol abuse and liver comorbidities.
Effect of TiO2 Crystal Phase on the Catalytic Transfer Hydrogenation Using Formic Acid over the Pd/TiO2 Catalyst
Authors: Mu Lili , Hua Zelin , Zhao Xuejuan , Ruan Chenxuanzhi , Chen Shanshan , Li Licheng 
Published: September 2024

AbstractThe influence of crystal phase of TiO2 on the catalytic performance of transfer hydrogenation using formic acid (HCOOH) over Pd/TiO2 catalyst has not been clarified before. In this study, Pd/TiO2 catalysts with four typical TiO2 crystal phases, that is, rutile, anatase, brookite, and TiO2(B), were synthesized and analyzed. The catalytic results showed that the TiO2 crystal phase could vary the transfer hydrogenation performance of the Pd/TiO2 catalysts by several to tens of times, which was in the following tendency: Pd/rutile > Pd/anatase > Pd/brookite > Pd/TiO2(B). Detailed comparison manifests the Pd dispersion state is obviously different on various TiO2 surfaces, which the relatively small particle size and more metallic are observed on Pd/rutile and Pd/anatase catalysts, contributing to the high catalytic performance. The reaction mechanism study further indicates that HCOOH on Pd/rutile is more liable to be dehydrogenated than that on other Pd/TiO2 catalysts, especially for the transformation of bidentate to monodentate formate. Pd/brookite shows the relatively highest selectivity of HCOOH dehydration. However, it is difficult for the HCOOH dehydrogenation to occur on Pd/TiO2(B). This work elucidates the roles of TiO2 crystal phase in Pd/TiO2 catalysts for the transfer hydrogenation reaction, which is expected to facilitate the development of efficient catalysts.
MoSe2‐Sensitized Water Splitting Assisted by C60‐Dendrons on the Basal Surface
Authors: Tajima Tomoyuki , Matsuura Tomoki , Efendi Arif , Yukimoto Mariko , Takaguchi Yutaka 
Published: September 2024

AbstractTo facilitate water splitting using MoSe2 as a light absorber, we fabricated water‐dispersible MoSe2/C60‐dendron nanohybrids via physical modification of the basal plane of MoSe2. Upon photoirradiation, the mixed‐dimension MoSe2/C60 (2D/0D) heterojunction generates a charge‐separated state (MoSe2⋅+/C60⋅−) through electron extraction from the exciton in MoSe2 to C60. This process is followed by the hydrogen evolution reaction (HER) from water in the presence of a sacrificial donor (1‐benzyl‐1,4‐dihydronicotinamide) and co‐catalyst (Pt‐PVP). The apparent quantum yields of the HER were estimated to be 0.06 % and 0.27 % upon photoexcitation at the A‐ and B‐exciton absorption peaks (λmax=800 and 700 nm), respectively.
3D printers – a great tool for chemistry lessons
Authors: Sieve Bernhard F. 
Published: September 2024

AbstractMithilfe eines 3D‐Druckers lassen sich heutzutage auch ohne Kenntnisse über die Handhabung von CAD‐Programmen einfach und kostengünstig Labormaterialien, Sachmodelle und Übungsmaterialien selbst erstellen. Im Beitrag werden Beispiele vorgestellt und nötige Grundlagen vermittelt.
Obstacle avoidance planning for industrial robots based on singular manifold splitting configuration space
Authors: Liu Yibo , Zhang Xuyan , Wu Chaoqun , Yang Minghui 
Published: September 2024

SummaryObstacle avoidance planning is the primary element in ensuring safe robot applications such as welding, assembly, and drilling. The states in the configuration space (C‐space) provide the pose information of any part of the manipulator and are preferentially considered in motion planning. However, it is difficult to express the environmental information directly in the high dimensional C‐space, limiting the application of C‐space obstacle avoidance planning. This paper proposes a singular manifold splitting C‐space method and designs a compatible obstacle avoidance strategy. The specific method is as follows: first, according to the specific structure of industrial robots, arm‐wrist separation obstacle avoidance planning is proposed to fix the robot as a 3R manipulator to reduce the dimension of C‐space. Next, the C‐space is segmented according to the singular manifolds, and the unique domain is delineated to complete the streamlining of the volume of the C‐space. Then, with the help of the point cloud, the obstacles are enveloped and mapped to the unique domain to construct the pseudo‐obstacle map. Industrial robots' obstacle avoidance planning is completed based on the pseudo‐obstacle map combined with an improved Rapidly‐Exploring Random Trees (RRT) algorithm. This method dramatically improves the efficiency of obstacle avoidance planning in the C‐space and avoids the effect of singularities on industrial robots. Finally, the effectiveness of the method is verified by physical experiments.
A Simple and Efficient Procedure for Developing Mouse Germline Stem Cell Lines with Gene Knock‐in via CRISPR/Cas9 Technology
Authors: Wang Yang , Hu Shuaitao , Han Chunsheng 
Published: September 2024

AbstractCultured mammalian spermatogonial stem cells (SSCs), also known as germline stem cells (GSCs), hold great promise for applications such as fertility preservation, gene therapy, and animal breeding, particularly in conjunction with accurate gene editing. Although the in vitro development of mouse GSC (mGSC) lines, and gene‐targeting procedures for such lines, were initially established about two decades ago, it remains challenging for beginners to efficiently accomplish these tasks, partly because mGSCs proliferate more slowly and are more resistant to lipid‐mediated gene transfection than pluripotent stem cells (PSCs). Meanwhile, methods for mGSC culture and gene editing have been evolving constantly to become simpler and more efficient. Here, we describe how to develop mGSC lines from small mouse testis samples and how to carry out gene knock‐in in these cells using CRISPR/Cas9 technology, detailing three basic protocols that constitute a streamlined procedure. Using these simple and efficient procedures, site‐specific knock‐in mGSC lines can be obtained in 3 months. We hope that these protocols will help researchers use genetically modified GSCs to explore scientific questions of interest and to accumulate experience for application to GSC research in other mammalian species. © 2024 Wiley Periodicals LLC.Basic Protocol 1: Establishment of mouse GSCs lines from small testicular samplesBasic Protocol 2: Preparation of plasmids for gene knock‐in using the CRISPR/Cas9 systemBasic Protocol 3: Establishment of gene knock‐in mGSC lines by electroporation gene delivery