bluuebunny/crossref_metadata_2025_split huggingface dataset:
Slowly progressive behavioral frontotemporal dementia syndrome in a family co‐segregating the C9orf72 expansion and a Synaptophysin mutation
Authors: Prota Joana , Rizzi Liara , Bonadia Luciana , de Souza Leonardo Cruz , Caramelli Paulo , Secolin Rodrigo , Lopes‐Cendes Iscia , Balthazar Marcio L. F. 
Published: July 2021

AbstractIntroductionSynaptophysin, already related to X‐linked intellectual disability, is expressed mainly in the central nervous system. Studies in humans indicate that the downregulation of synaptophysin could be involved in the development of dementia. Our study presents the first familial case of behavioral variant frontotemporal dementia associated with the co‐occurrence of the repeat expansion in C9orf72 and a pathogenic variant in the SYP gene.MethodsExome sequencing and repeat‐primed PCR for C9orf72 were performed for two siblings with clinical and imaging findings suggestive of slowly progressive behavioral frontotemporal dementia.ResultsWe found that both siblings have the hexanucleotide expansion in C9orf72 and a null variant in the SYP gene. The most affected sibling presents the putative variant in a hemizygous state. With milder symptoms, his sister has the same pathogenic variant in heterozygosis, compatible with X‐linked inheritance.DiscussionOur results strengthened previous suggestive evidence that the phenotypes associated with C9orf72 repeat expansion are variable and probably influenced by additional genetic modifiers. We hypothesized that the pathogenic variant in the SYP gene might have modified the typical phenotype associated with the C9orf72 mutation.
Spektroskopische Charakterisierung eines reaktiven [Cu2(μ‐OH)2]2+ Intermediates in Cu/TEMPO‐katalysierten aeroben Alkoholoxidationen
Authors: Warm Katrin , Tripodi Guilherme , Andris Erik , Mebs Stefan , Kuhlmann Uwe , Dau Holger , Hildebrandt Peter , Roithová Jana , Ray Kallol 
Published: July 2021

AbstractCuI/TEMPO‐Katalysatorsysteme (TEMPO=2,2,6,6‐Tetramethylpiperidinoxyl) sind vielseitige Katalysatoren für aerobe Alkoholoxidationsreaktionen zur selektiven Synthese von Aldehyden. Jedoch sind mehrere Aspekte des Reaktionsmechanismus noch nicht aufgeklärt, was hauptsächlich daran liegt, dass bisher keine reaktiven Intermediate identifiziert werden konnten. Wir zeigen hier die Synthese und Charakterisierung eines dinuklearen Komplexes [L12Cu2]2+ (1), der in Gegenwart von TEMPO die katalytische 4 H+/4 e− Reduktion von O2 zu Wasser an die Oxidation von benzylischen und aliphatischen Alkoholen koppeln kann. Die Mechanismen der katalytischen O2‐Reduktion und der Alkoholoxidation wurden sowohl durch spektroskopische Detektion der reaktiven Intermediate in der Gas‐ und der kondensierten Phase als auch durch kinetische Studien an jedem Reaktionsschritt in den Katalysezyklen aufgeklärt. Die intermediären Bis(μ‐oxido)dikupfer(III)‐ (2) und Bis(μ‐hydroxido)dikupfer(II)‐Spezies (3) wurden als wichtige Reaktanden in beiden Reaktionen identifiziert. Die vorliegende Studie ermöglicht tiefgehende mechanistische Einblicke in die aerobe Alkoholoxidation, die eine wertvolle Grundlage bieten um übergangsmetallkatalysierte Reaktionen mit redoxaktiven Cokatalysatoren besser zu verstehen.
Brightening up Circularly Polarized Luminescence of Monosubstituted Polyacetylene by Conformation Control: Mechanism, Switching, and Sensing
Authors: Wang Sheng , Hu Deping , Guan Xiaoyan , Cai Siliang , Shi Ge , Shuai Zhigang , Zhang Jie , Peng Qian , Wan Xinhua 
Published: July 2021

AbstractThe first example of luminescent monosubstituted polyacetylenes (mono‐PAs) is presented, based on a contracted cis‐cisoid polyene backbone. It has an excellent circularly polarized luminescence (CPL) performance with a high dissymmetric factor (up to the order of 10−1). The luminescence stems from the helical cis‐cisoid PA backbone, which is tightly fixed by the strong intramolecular hydrogen bonds, thereby reversing the energy order of excited states and enabling an emissive energy dissipation. CPL switches are facilely achieved by the solvent and temperature through reversible conformational transition. By taking advantages of fast response and high sensitivity, the thin film of mono‐PAs could be used as a CPL‐based probe for quantitative detection of trifluoroacetic acid with a wider linear dynamic range than those of photoluminescence and circular dichroism. This work opens a new avenue to develop novel smart CPL materials through modulating conformational transition.
Arylamine‐Linked 2D Covalent Organic Frameworks for Efficient Pseudocapacitive Energy Storage
Authors: Yang Zongfan , Liu Jingjuan , Li Yusen , Zhang Guang , Xing Guolong , Chen Long 
Published: July 2021

AbstractThe development of new linkages is one of the most efficient strategies to enrich the diversity of covalent organic frameworks (COFs). Particularly, functional linkages can endow COFs with additional tailored properties besides the building units, which further diversify COFs with desirable functions. Herein, we have developed a new arylamine linkage for the construction of COFs. Two new arylamine‐linked COFs (AAm‐TPB and AAm‐Py) were prepared by condensing cost‐effective dimethyl succinyl succinate (DMSS) with corresponding multitopic amines (TPB‐NH2 and Py‐NH2). Due to the abundant electroactive diphenylamine moieties in the COF skeletons resembling that of polyaniline (PANI), a state‐of‐the‐art conductive polymer, the pseudocapacitive energy storage performance of AAm‐TPB was further investigated. Remarkably, the AAm‐TPB electrode exhibits a high capacitance of 271 F g−1 with a three‐electrode setup at a discharge rate of 1 A g−1, which represents one of the highest capacitances among the reported COF‐based electrode materials.
Wide temperature range damping polyurethane elastomer based on dynamic disulfide bonds
Authors: Ma Yuanhao , Qin Rui , Xu Min , Jiang Xiaolin , Sheng Yeming , Wang Minhui , Zhang Wencong , Lu Xun 
Published: July 2021

AbstractThe design and synthesis of high‐performance polyurethane with excellent damping performance and superior mechanical property is a challenge owing to conflicting attributes of damping and mechanical performance. Herein, we innovatively introduced dynamic aromatic disulfide bonds and suitable disulfide catalysts into the system and synthesized a polyurethane elastomer with both admirable damping performance (wide damping temperature range [tan δ ≥ 0.3] of 148°C) and excellent mechanical properties (16.8 MPa, 960%). In our design, (i) dynamic aromatic disulfide exchange reaction above room temperature is able to enhance energy dissipation and segmental mobility, thus promoting the damping performance of polyurethane; (ii) the introduced catalyst can further promote dynamic exchange of disulfide bonds above room temperature, making the chains easier to move and leading to excellent damping property. Moreover, by adjusting the chain extension coefficient, the molecular structure of the chain extender, it's easy to design the microphase separation and rigid structure of the elastomer and the system is endowed with improved mechanical strength. Owing to its outstanding properties, the elastomer has great potential to be used for practical application.
Stop moving! Pain, body and trauma in pictures and narratives of patients, suffering with a somatoform disorder
Authors: Ruettner Barbara , Zander Michael , Kappler Melanie , Niehaus Johanna , Thomas Nele , Kaiser Paul Maximilian , Goetzmann Lutz 
Published: July 2021

AbstractSomatoform pain disorders are strongly associated with adverse, mostly traumatic childhood experiences. Little is known, however, about the psychodynamic background, for example, with regard to defence, affect regulation, and traumatic representations. As part of a qualitative study in which 13 patients with somatoform disorder were investigated, we compared the interviews and drawings by the two patients which featured the most pictorial codes for deaffectualization as a defence of the autistoid type of early disorders with the two patients that displayed the fewest codes for deaffectualization. These patients suffered from a more mature, non‐autistoid disorder. The drawings were analyzed according to the Visual Grounded Theory, the interviews according to Content Analysis. We used the qualitative atlas.ti‐software. In the two patients with a high evidence of pictorial deaffectualization, a correspondence was found between the deaffectualization and the type of pain sensation: Physical movements led to an increase in pain. The affective as well as the physical rigidity may have been intended to avoid the body image's fragmentation. The drawings by the more mature patients showed strong, partly violent interactions. In the narratives, the patients tended more to report feelings of agitation; generally, more physical movements could be allowed. In all four patients, a considerable correspondence was identified between the drawings and the narratives about physical pain. The dimension of the imaginary was determined both physically (on the level of the body image) and pictorially (on the level of the mental image) through the regulation of traumatic affects.
Brain morphology, autistic traits, and polygenic risk for autism: A population‐basedneuroimaging study
Authors: Alemany Silvia , Blok Elisabet , Jansen Philip R. , Muetzel Ryan L. , White Tonya 
Published: July 2021

AbstractAutism spectrum disorders (ASD) are associated with widespread brain alterations. Previous research in our group linked autistic traits with altered gyrification, but without pronounced differences in cortical thickness. Herein, we aim to replicate and extend these findings using a larger and older sample. Additionally, we examined whether (a) brain correlates of autistic traits were associated with polygenic risk scores (PRS) for ASD, and (b) autistic traits are related with brain morphological changes over time in a subset of children with longitudinal data available. The sample included 2400 children from the Generation R cohort. Autistic traits were measured using the Social Responsiveness Scale (SRS) at age 6 years. Gyrification, cortical thickness, surface area, and global morphological measures were obtained from high‐resolution structural MRI scans at ages 9‐to‐12 years. We performed multiple linear regression analyses on a vertex‐wise level. Corresponding regions of interest were tested for association with PRS. Results showed that autistic traits were related to (a) lower gyrification in the lateral occipital and the superior and inferior parietal lobes, (b) lower cortical thickness in the superior frontal region, and (c) lower surface area in inferior temporal and rostral middle frontal regions. PRS for ASD and longitudinal analyses showed significant associations that did not survive correction for multiple testing. Our findings support stability in the relationship between higher autistic symptoms and lower gyrification and smaller surface areas in school‐aged children. These relationships remained when excluding ASD cases, providing neurobiological evidence for the extension of autistic traits into the general population.Lay SummaryWe found that school‐aged children with higher levels of autistic traits had smaller total brain volume, cerebellum, cortical thickness, and surface area. Further, we also found differences in the folding patterns of the brain (gyrification). Overall, genetic susceptibility for autism spectrum disorders was not related to these brain regions suggesting that other factors could be involved in their origin. These results remained significant when excluding children with a diagnosis of ASD, providing support for the extension of the relationship between autistic traits and brain findings into the general population.
A large‐scale metagenomic study for enzyme profiles using the focused identification of the NGS‐based definitive enzyme research (FINDER) strategy
Authors: Sung Jae‐Yoon , Lee Yong‐Jik , Cho Yong‐Joon , Shin Myeong‐Na , Lee Sang‐Jae , Lee Han‐Seung , Koh Hong , Bae Jin‐Woo , Shin Jae‐Ho , Kim Hyun Jung , Lee Dong‐Woo 
Published: July 2021

AbstractExcavating the molecular details of many diverse enzymes from metagenomes remains challenging in agriculture, food, health, and environmental fields. We present a versatile method that accelerates metabolic enzyme discovery for highly selective gene capture in metagenomes using next‐generation sequencing. Culture‐independent enzyme mining of environmental DNA is based on a set of short identifying degenerate sequences specific for a wide range of enzyme superfamilies, followed by multiplexed DNA barcode sequencing. A strategy of ‘focused identification of next‐generation sequencing‐based definitive enzyme research’ enabled us to generate targeted enzyme datasets from metagenomes, resulting in minimal hands‐on obtention of high‐throughput biological diversity and potential function profiles, without being time‐consuming. This method also provided a targeted inventory of predicted proteins and molecular features of metabolic activities from several metagenomic samples. We suggest that the efficiency and sensitivity of this method will accelerate the decryption of microbial diversity and the signature of proteins and their metabolism from environmental samples.
A novel mechanism for the formation and propagation of neural tumors and lesions through neural highways
Authors: Spinner Robert J. 
Published: July 2021

AbstractBy recognizing anatomic and radiologic patterns of rare and often misdiagnosed peripheral nerve tumors/lesions, we have defined mechanisms for the propagation of neural diseases. The novel concept of the nervous system serving as a complex system of “highways” driving the neural and perineural spread of these lesions is described in three examples: Intraneural dissection of joint fluid in intraneural ganglion cysts, perineural spread of cancer cells, and dissemination of unknown concentrations of neurotrophic/inhibitory factors for growth in hamartomas/choristomas of nerve. Further mapping of these pathways to identify the natural history of diseases, the spectrum of disease evolution, the role of genetic mutations, and how these neural pathways interface with the lymphatic, vascular, and cerebrospinal systems may lead to advances in targeted treatments.
Migratory fishbones in the pharynx: A report of two cases
Authors: Xu Shuyi , Li Xiaomin , Peng Yue , Yang Jing , Liu Qianxu , Guo Jiefeng , Yu Zhijian 
Published: July 2021

AbstractThe possibility of fishbone migration into the surrounding tissues, especially in cases where it cannot be identified on routine inspection. Early diagnosis of migratory fishbone and therapeutic management are essential for optimal patient survival.
Modeling and Simulation for the Production Process of Soda Ash by the Ammonium Sulfate‐Soda Method
Authors: Pak Kyong Song , Son Sun Il , Li Bong Hak , Jang Myong Pok , Jang Paek San , Kim Kuk Chol , Pak Sung Nam 
Published: July 2021

AbstractThe ammonium sulfate‐soda method is a procedure that produces soda ash and the by‐product ammonium sulfate, using mirabilite as the raw material. Here, a process for producing soda ash by the ammonium sulfate‐soda method is proposed. Based on the reaction mechanisms occurring in the Na2SO4‐NH3‐CO2‐H2O system and the kinetic‐thermodynamic data of the CO2 absorption process using aqueous NH3, the soda ash production process is modeled and simulated using Aspen Plus. The effects of various process parameters (the concentration of the raw mirabilite solution, the amounts of NH3 and CO2 flowing into the process, and the recycle ratio of CO2 exhausted from the NaHCO3 calciner, etc.) on the process performance are investigated.
Design and Control for the Dimethyl Adipate Process with a Side‐Reactor Column Configuration
Authors: Zhang Guowen , Wang Le , Tang Jihai , Jin Hao , Zhang Zhuxiu , Fei Zhaoyang , Liu Qing , Chen Xian , Cui Mifen , Qiao Xu 
Published: July 2021

AbstractA side‐reactor column (SRC) configuration, which integrates vacuum distillation with atmospheric reaction, was developed for dimethyl adipate (DMA) production. The sequential optimization method was applied to optimize the design parameters including side stream drawn stage, reactor numbers, and catalyst loading with total annual cost (TAC) as objective function in order to realize the economical design. The best economic performance was achieved as the result of the specific SRC configuration featuring one column with 20 stages where DMA was drawn from the 5th stage and one side‐reactor was equipped in the bottom. Moreover, a proportion‐temperature cascade control structure was successfully developed which avoids the disturbance of inlet flow rate and composition.
Investigating the feasibility of using carbon fiber tapes as reinforcement for 3D concrete printing
Authors: Scheurer Martin , Quenzel Philipp , Nölke Peter , Reuter‐Schniete Jonas , Gries Thomas 
Published: July 2021

AbstractThe main advantages of textiles as concrete reinforcement are high corrosion resistance, allowing for thin concrete covers, high drapability, and high strength. Due to these advantages, textiles are particularly suitable as reinforcement for 3D printed concrete, where thin elements and freeform geometries are possible. In this article, a new type of textile reinforcement for the integration into 3D concrete printing is investigated: unidirectional carbon fiber tapes. Carbon fiber tapes are made from carbon fiber rovings by spreading. Three carbon fiber tapes are produced and used as reinforcement in concrete specimens subjected to four‐point bending tests. The results show that for a spreading factor of 2.2 (areal weight 136 g/m2), the flexural strength of the beams is increased by 125% compared to reference beams reinforced with unspread rovings. Further increase of the spreading factor does not increase flexural strength further as the failure mode changes from rupture to spalling.
Chalcone‐Supported Cardiac Mesoderm Induction in Human Pluripotent Stem Cells for Heart Muscle Engineering
Authors: Raad Farah S. , Khan Taukeer A. , Esser Tilman U. , Hudson James E. , Seth Bhakti Irene , Fujita Buntaro , Gandamala Ravi , Tietze Lutz F. , Zimmermann Wolfram-Hubertus 
Published: July 2021

AbstractHuman pluripotent stem cells (hPSCs) hold great promise for applications in cell therapy and drug screening in the cardiovascular field. Bone morphogenetic protein 4 (BMP4) is key for early cardiac mesoderm induction in hPSC and subsequent cardiomyocyte derivation. Small‐molecular BMP4 mimetics may help to standardize cardiomyocyte derivation from hPSCs. Based on observations that chalcones can stimulate BMP4 signaling pathways, we hypothesized their utility in cardiac mesoderm induction. To test this, we set up a two‐tiered screening strategy, (1) for directed differentiation of hPSCs with commercially available chalcones (4’‐hydroxychalcone [4’HC] and Isoliquiritigen) and 24 newly synthesized chalcone derivatives, and (2) a functional screen to assess the propensity of the obtained cardiomyocytes to self‐organize into contractile engineered human myocardium (EHM). We identified 4’HC, 4‐fluoro‐4’‐methoxychalcone, and 4‐fluoro‐4’‐hydroxychalcone as similarly effective in cardiac mesoderm induction, but only 4’HC as an effective replacement for BMP4 in the derivation of contractile EHM‐forming cardiomyocytes.
The impact of atezolizumab and bevacizumab in hepatocellular carcinoma with activated ß‐catenin signaling
Authors: Krug Sebastian , Mattheis Laura , Haemmerle Monika , Rosendahl Jonas , Kleeff Joerg , Michl Patrick 
Published: July 2021

AbstractBackgroundTo date, no biomarkers exist to predict response or resistance to immunotherapy in hepatocellular carcinoma (HCC). Recent approaches to classify HCC into different immunological states revealed a negative correlation between Wnt/ß‐catenin activation and immunogenicity and T‐cell infiltration. If these “cold” tumors with primary resistance to checkpoint inhibition (CPI) may benefit from dual treatment of CPI and anti‐angiogenic therapy has not been proved.CaseHere, we describe the case of a male patient with metastatic HCC. After failure of standard of care treatment with lenvatinib, sorafenib and ramucirumab fourth‐line systemic therapy with atezolizumab and bevacizumab were applied leading to a phenomenal response. Immunohistochemical evaluations were compatible with Wnt/ß‐catenin pathway activation and accompanying low T‐cell infiltration as well as low PD‐L1 score.ConclusionPatients with Wnt/ß‐catenin activation may benefit from combination therapy with atezolizumab and bevacizumab regardless of potential predictive markers for immune checkpoint inhibition.
A method of core concept extraction based on semantic‐weight ranking
Authors: Zhong Han , Ning Zhenhu , Li Guijun , Li Zheng 
Published: July 2021

SummaryIn order to improve the efficiency of domain ontology concept extraction, this paper proposes a method of semantic‐weight ranking to extract core concepts. Firstly, we make segmentation processing for corpus texts and obtain the candidate concept set, and then analyze the correlation between the candidate concepts through the method of semantic similarity to form a mesh graph structure. We secondly calculate the semantic‐weight of the concept according to the generated mesh graph. Ultimately, we realize the core concept extraction via ranking different semantic‐weight. At the same time, we give the fuzzy relationship representation of the core concepts through the statistical characteristics of the core concepts' membership. On the foundation of the extraction method, we carried out the experiments with the financial text and had achieved an accuracy rate of 81.8%, which could effectively extract the core concepts and play a positive role in the content construction, knowledge sharing, and knowledge reuse.
Metaheuristic task scheduling algorithms for cloud computing environments
Authors: Aktan Merve Nur , Bulut Hasan 
Published: July 2021

SummaryCloud computing has the advantage of providing flexibility, high‐performance, pay‐as‐you‐use, and on‐demand service. One of the important research issues in cloud computing is task scheduling. The purpose of scheduling is to assign tasks to available resources while providing optimization on some objectives. Tasks have diversified characteristics, and resources are heterogeneous. These properties make task scheduling an NP‐complete problem. In this study, metaheuristic and hybrid metaheuristic algorithms are developed for task scheduling problems in cloud computing environments. We have developed genetic algorithm (GA), differential evolution (DE), and simulated annealing (SA) based metaheuristic algorithms, which are also combined with a greedy approach (GR). In addition to this, we have developed hybrid metaheuristics algorithms, called DE‐SA and GA‐SA, which are also combined with a greedy approach. The proposed approaches are evaluated in terms of completion time and load balancing of virtual machines. In terms of average completion time, as the number of tasks increases, it has been observed that the DESA algorithm outperforms the solely used DE and SA algorithms. In addition, experiments show that hybrid algorithms improve both the average completion time and the average standard deviation of virtual machine loads for some task groups.