bluuebunny/crossref_metadata_2025_split huggingface dataset:
Is autism driven by epilepsy in infants with Tuberous Sclerosis Complex?
Authors: Moavero Romina , Kotulska Katarzyna , Lagae Lieven , Benvenuto Arianna , Emberti Gialloreti Leonardo , Weschke Bernhard , Riney Kate , Feucht Martha , Krsek Pavel , Nabbout Rima , Jansen Anna C. , Wojdan Konrad , Borkowska Julita , Sadowski Krzysztof , Hertzberg Christoph , Van Schooneveld Monique M. , Samueli Sharon , Maulisovà Alice , Aronica Eleonora , Kwiatkowski David J. , Jansen Floor E. , Jozwiak Sergiusz , Curatolo Paolo ,   the EPISTOP Consortium
Published: July 2020

AbstractObjectiveTo evaluate the relationship between age at seizure onset and neurodevelopmental outcome at age 24 months in infants with TSC, as well as the effect on neurodevelopmental outcome of early versus conventional treatment of epileptic seizures with vigabatrin (80–150 mg/kg/day).MethodsInfants with TSC, aged ≤4 months and without previous seizures were enrolled in a prospective study and closely followed with monthly video EEG and serial standardized neurodevelopmental testing (Bayley Scales of Infant Development and Autism Diagnostic Observation Schedule).ResultsEighty infants were enrolled. At the age of 24 months testing identified risk of Autism Spectrum Disorder (ASD) in 24/80 children (30.0%), and developmental delay (DD) in 26/80 (32.5%). Children with epilepsy (51/80; 63.8%) had a higher risk of ASD (P = 0.02) and DD (P = 0.001). Overall, no child presented with moderate or severe DD at 24 months (developmental quotient < 55). In 20% of children abnormal developmental trajectories were detected before the onset of seizures. Furthermore, 21% of all children with risk of ASD at 24 months had not developed seizures at that timepoint. There was no significant difference between early and conventional treatment with respect to rate of risk of ASD (P = 0.8) or DD (P = 0.9) at 24 months.InterpretationThis study confirms a relationship between epilepsy and risk of ASD/DD. However, in this combined randomized/open label study, early treatment with vigabatrin did not alter the risk of ASD or DD at age 2 years.
Safe‐by‐Design Exfoliation of Niobium Diselenide Atomic Crystals as a Theory‐Oriented 2D Nanoagent from Anti‐Inflammation to Antitumor
Authors: Miao Zhaohua , Huang Doudou , Wang Yichuan , Li Wei‐Jian , Fan Linxin , Wang Jingguo , Ma Yan , Zhao Qingliang , Zha Zhengbao 
Published: July 2020

AbstractThe accompanying relationship between tumor and inflammation raises the concept of concurrent antitumor and anti‐inflammation treatment in the clinic. Despite the wide application of 2D atomic crystals for cancer theranostics, their anti‐inflammation function has been rarely explored. Herein, it is reported that niobium diselenide nanosheets (NbSe2 NSs), a “star” 2D superconducting atomic crystal, can serve as a theory‐oriented 2D nanoagent from anti‐inflammation to antitumor. A safe‐by‐design exfoliation strategy, integration of cryo‐pretreatment and DNA‐assisted exfoliation, is proposed for high‐efficiency exfoliation of atomically thin NbSe2 NSs. Especially, computational simulation reveals that NbSe2 NSs effectively eliminate reactive oxygen and nitrogen species (RONS) via hydrogen atom transfer and redox reaction. Upon the injection of NbSe2 NSs into BALB/c mice, not only U87 subcutaneous tumors are rapidly ablated after photoacoustic imaging‐guided precise localization of tumor contour, but also lipopolysaccharide‐induced rear thigh inflammation or photothermal therapy‐mediated inflammation is efficiently inhibited through RONS scavenging. In addition, NbSe2 NSs are highly biocompatible both in vitro and in vivo due to high‐security element constituent and DNA modification. The work extends the biomedical application of 2D atomic crystals for anti‐inflammatory treatment.
Tough and Multi‐Recyclable Cross‐Linked Supramolecular Polyureas via Incorporating Noncovalent Bonds into Main‐Chains
Authors: Qin Bo , Zhang Shuai , Sun Peng , Tang Bohan , Yin Zihe , Cao Xiao , Chen Quan , Xu Jiang‐Fei , Zhang Xi 
Published: July 2020

AbstractCovalent thermosets generally exhibit robust mechanical properties, while they are fragile and lack the ability to be reprocessed or recycled. Herein, a new strategy of incorporating noncovalent bonds into main‐chains is developed to construct tough and multi‐recyclable cross‐linked supramolecular polyureas (CSPU), which are prepared via the copolymerization of diisocyanate monomers, noncovalently bonded diamine monomers linked by quadruple hydrogen bonds, and covalent diamine/triamine monomers. The CSPU exhibit remarkable solvent resistance and outstanding mechanical properties owing to the covalent cross‐linking via triamine monomer. Through the incorporation of 9.7% and 14.6% quadruple hydrogen bonded diamine monomer, the transparent CSPU films are endowed with superior toughness of 74.17 and 124.17 MJ m−3, respectively. Impressively, even after five generations of recycling processes, the mechanical properties of reprocessed CSPU can recover more than 95% of their original properties, displaying excellent multiple recyclablity. As a result, the superior toughness, remarkable solvent resistance, high transparency, and excellent multiple recyclability are well‐combined in the CSPU. It is highly anticipated that this line of research will provide a facile and general method to construct various cross‐linked polymer materials with superior recyclability and mechanical properties.
Novel Bi‐Doped Amorphous SnOx Nanoshells for Efficient Electrochemical CO2 Reduction into Formate at Low Overpotentials
Authors: Yang Qi , Wu Qilong , Liu Yang , Luo Shuiping , Wu Xiaotong , Zhao Xixia , Zou Haiyuan , Long Baihua , Chen Wen , Liao Yujia , Li Lanxi , Shen Pei Kang , Duan Lele , Quan Zewei 
Published: July 2020

AbstractEngineering novel Sn‐based bimetallic materials could provide intriguing catalytic properties to boost the electrochemical CO2 reduction. Herein, the first synthesis of homogeneous Sn1−xBix alloy nanoparticles (x up to 0.20) with native Bi‐doped amorphous SnOx shells for efficient CO2 reduction is reported. The Bi‐SnOx nanoshells boost the production of formate with high Faradaic efficiencies (>90%) over a wide potential window (−0.67 to −0.92 V vs RHE) with low overpotentials, outperforming current tin oxide catalysts. The state‐of‐the‐art Bi‐SnOx nanoshells derived from Sn0.80Bi0.20 alloy nanoparticles exhibit a great partial current density of 74.6 mA cm−2 and high Faradaic efficiency of 95.8%. The detailed electrocatalytic analyses and corresponding density functional theory calculations simultaneously reveal that the incorporation of Bi atoms into Sn species facilitates formate production by suppressing the formation of H2 and CO.
Giant and Broadband Multiphoton Absorption Nonlinearities of a 2D Organometallic Perovskite Ferroelectric
Authors: Li Maofan , Xu Yanming , Han Shiguo , Xu Jinlong , Xie Zhenda , Liu Yi , Xu Zhiyun , Hong Maochun , Luo Junhua , Sun Zhihua 
Published: July 2020

AbstractMultiphoton absorption (MPA) has been utilized for important technological applications. High‐order multiphoton harvesting (e.g., five‐photon absorption, 5PA) exhibits unique properties that could benefit biophotonics. Within this field, perovskite oxide ferroelectrics (e.g., BaTiO3) enable low‐order optical nonlinearities of 2PA/3PA processes. However, it is challenging to obtain efficient, high‐order 5PA effects. Herein, for the first time, giant and broadband MPA properties are presented in the 2D hybrid perovskite ferroelectric (IA)2(MA)2Pb3Br10 (1; IA = isoamylammonium and MA = methylammonium), where multiphoton‐excited optical nonlinearities related to different MPA mechanisms over a broadband range of 550–2400 nm are observed. Strikingly, its 5PA absorption cross‐section (σ5) reaches up to 1.2 × 10−132 cm10 s4 photon−4 (at 2400 nm), almost 10 orders larger than some state‐of‐the‐art organic molecules and a record‐high value among all known ferroelectrics. This unprecedented 5PA effect results from the quantum‐confined motif of inorganic trilayer sheets (wells) and organic cations (barriers) in 1. Moreover, its large ferroelectric polarization of 5 µC cm−2 could promote modulation of MPA effects under external electric fields. As far as it is known, this is the first report on giant, broadband high‐order MPA properties in ferroelectrics, which provides potential, novel electric‐ordered materials for next‐generation biophotonic applications.
Highly UV Resistant Inch‐Scale Hybrid Perovskite Quantum Dot Papers
Authors: Li Ting‐You , Xu Xuezhu , Lin Chun‐Ho , Guan Xinwei , Hsu Wei‐Hao , Tsai Meng‐Lin , Fang Xiaosheng , Wu Tom , He Jr‐Hau 
Published: July 2020

AbstractHalide perovskite quantum dots (PQDs) are promising materials for diverse applications including displays, light‐emitting diodes, and solar cells due to their intriguing properties such as tunable bandgap, high photoluminescence quantum yield, high absorbance, and narrow emission peaks. Despite the prosperous achievements over the past several years, PQDs face severe challenges in terms of stability under different circumstances. Currently, researchers have overcome part of the stability problem, making PQDs sustainable in water, oxygen, and polar solvents for long‐term use. However, halide PQDs are easily degraded under continuous irradiation, which significantly limits their potential for conventional applications. In this study, an oleic acid/oleylamine (traditional surface ligands)‐free method to fabricate perovskite quantum dot papers (PQDP) is developed by adding cellulose nanocrystals as long‐chain binding ligands that stabilize the PQD structure. As a result, the relative photoluminescence intensity of PQDP remains over ≈90% under continuous ultraviolet (UV, 16 W) irradiation for 2 months, showing negligible photodegradation. This proposed method paves the way for the fabrication of ultrastable PQDs and the future development of related applications.
Microfluidic‐Assisted Blade Coating of Compositional Libraries for Combinatorial Applications: The Case of Organic Photovoltaics
Authors: Rodríguez‐Martínez Xabier , Sevim Semih , Xu Xiaofeng , Franco Carlos , Pamies‐Puig Paula , Córcoles‐Guija Laura , Rodriguez‐Trujillo Romen , del Campo Francisco Javier , Rodriguez San Miguel David , deMello Andrew J. , Pané Salvador , Amabilino David B. , Inganäs Olle , Puigmartí‐Luis Josep , Campoy‐Quiles Mariano 
Published: July 2020

AbstractMicrofluidic technologies are highly adept at generating controllable compositional gradients in fluids, a feature that has accelerated the understanding of the importance of chemical gradients in biological processes. That said, the development of versatile methods to generate controllable compositional gradients in the solid‐state has been far more elusive. The ability to produce such gradients would provide access to extensive compositional libraries, thus enabling the high‐throughput exploration of the parametric landscape of functional solids and devices in a resource‐, time‐, and cost‐efficient manner. Herein, the synergic integration of microfluidic technologies is reported with blade coating to enable the controlled formation of compositional lateral gradients in solution. Subsequently, the transformation of liquid‐based compositional gradients into solid‐state thin films using this method is demonstrated. To demonstrate efficacy of the approach, microfluidic‐assisted blade coating is used to optimize blending ratios in organic solar cells. Importantly, this novel technology can be easily extended to other solution processable systems that require the formation of solid‐state compositional lateral gradients.
A PC‐SAFT model for heavy hydrocarbon thermodynamics in downstream separation processes
Authors: Marshall Bennett D. , Vella Joseph R. , Greene Jennifer , Kozuch Daniel J. 
Published: July 2020

AbstractThe Advanced Petroleum Parameterization Scheme (TAPPS) provides a very accurate method to determine pure component petroleum fraction model parameters for the polar PC‐SAFT equation of state. In this work we extend the TAPPS/PC‐SAFT petroleum thermodynamics methodology to vacuum residuum (VR) ranged molecules. The challenge for these heavy species is twofold: first, the normal boiling point ceases to be a sensible descriptor of molecular size; and second, the phase behavior of interest in downstream processing is liquid–liquid equilibria (LLE). We develop new parameter models for both unary and binary polar PC‐SAFT parameters for VR ranged molecules. In addition to PC‐SAFT parameters, we also develop a scheme to redefine a detailed model of composition to a number of pseudo‐components manageable in a process simulator (~102) using a lumping scheme based on the fraction of aromatic carbon and molecular weight. We demonstrate that this methodology allows for the accurate description of VR/paraffin LLE.
The recurrent TUBB3 Gly98Ser substitution is the first described to inconsistently result in CFEOM3
Authors: Smith Scott C. , Olney Ann Haskins , Beavers Angela , Spaulding Joanna , Nelson Marilu , Nielsen Shelly , Sanmann Jennifer N. 
Published: July 2020

AbstractMissense variants in TUBB3 have historically been associated with either congenital fibrosis of the extraocular muscles type 3 (CFEOM3) or malformations of cortical development (MCD). Until a recent report identified two amino acid substitutions in four patients that had clinical features of both disorders, pathogenic variants of TUBB3 were thought distinct to either respective disorder. Three recurrent de novo Gly71Arg TUBB3 substitutions and a single patient with a de novo Gly98Ser substitution blurred the MCD and CFEOM3 phenotypic distinctions. Here we report a second patient with a missense c.292G>A (p.Gly98Ser) substitution, but without CFEOM3, the first reported evidence that even the same TUBB3 substitution can produce a spectrum of TUBB3 syndrome phenotypes. Our patient presented with amblyopia, exotropia, optic disc pallor, and developmental delay. Neuroimaging identified hypoplasia of the corpus callosum, interdigitation of the frontal lobe gyri, and dysplasia or hypoplasia of the optic nerves, basal ganglia, brainstem, and cerebellum. This report identifies the TUBB3 Gly98Ser substitution to be recurrent but inconsistently including CFEOM3, and identifies the absence of joint contractures and the presence of optic disc abnormalities that may be genotype‐specific to the TUBB3 Gly98Ser substitution.
The [(NHC)B(H)C6F5]+ Cations and Their [B](H)−CO Borane Carbonyls
Authors: Chen Chaohuang , Li Jun , Daniliuc Constantin G. , Mück‐Lichtenfeld Christian , Kehr Gerald , Erker Gerhard 
Published: July 2020

AbstractHydride abstraction from the heterocyclic carbene borane adducts (NHC)BH2C6F5 (NHC: IMes or IMe4) gave the B−H containing [(NHC)B(H)C6F5]+ borenium cations. They added carbon monoxide to give the respective [(NHC)B(H)(C6F5)CO]+ boron carbonyl cations. Carbon nucleophiles add to the boron carbonyl to give [B](H) acyls. Hydride reduced the [B]CO cation to hydroxymethylborane derivatives.
Nanochaperone‐Based Strategies to Control Protein Aggregation Linked to Conformational Diseases
Authors: Caballero Ana B. , Gamez Patrick 
Published: July 2020

AbstractThe generation of highly organized amyloid fibrils is associated with a wide range of conformational pathologies, including primarily neurodegenerative diseases. Such disorders are characterized by misfolded proteins that lose their normal physiological roles and acquire toxicity. Recent findings suggest that proteostasis network impairment may be one of the causes leading to the accumulation and spread of amyloids. These observations are certainly contributing to a new focus in anti‐amyloid drug design, whose efforts are so far being centered on single‐target approaches aimed at inhibiting amyloid aggregation. Chaperones, known to maintain proteostasis, hence represent interesting targets for the development of novel therapeutics owing to their potential protective role against protein misfolding diseases. In this minireview, research on nanoparticles that can either emulate or help molecular chaperones in recognizing and/or correcting protein misfolding is discussed. The nascent concept of “nanochaperone” may indeed set future directions towards the development of cost‐effective, disease‐modifying drugs to treat several currently fatal disorders.
Peanut FAD2 Genotype and Growing Location Interactions Significantly Affect the Level of Oleic Acid in Seeds
Authors: Tonnis Brandon , Wang Ming Li , Li Xianran , Wang Jianping , Puppala Naveen , Tallury Shyam , Yu Jianming 
Published: July 2020

AbstractThe level of oleic acid is an important parameter in determining seed nutritional quality and oil stability. The level of oleic acid in peanut is genetically controlled by a pair of fatty acid desaturase genes (FAD2A and FAD2B), but the environmental conditions of the production sites can also have a significant effect. To investigate the effect of gene and environment interaction, 45 accessions were grown at three locations for 2 years. Environmental data were collected; individual plants were genotyped with functional SNP markers from FAD2A and FAD2B; and seed level of oleic acid was determined by gas chromatography. Three FAD2A/FAD2B genotypes (448G/no insertion 442A, 448A/no insertion 442A, and 448A/insertion 442A) were identified and designated as G/W, A/W, and A/A, respectively. A/A genotype averaged the highest level of oleic acid (80.0%), followed by A/W (56.0%), and then G/W (40.7%). Analysis of gene and environment interaction revealed that oleic acid phenotype plasticity could be explained by the interaction of FAD2 genotype and photothermal time, which quantified environmental conditions. The A/W genotype was the most sensitive to photothermal time changes. The oleic acid plasticity revealed in this study would be useful for breeders, farmers, and product processors.
Distribution and habitat use by endangered Cook Inlet beluga whales: Patterns observed during a photo‐identification study, 2005–2017
Authors: McGuire Tamara L. , Himes Boor Gina K. , McClung John R. , Stephens Amber D. , Garner Christopher , Shelden Kim E.W. , Wright Bruce 
Published: July 2020

AbstractCook Inlet beluga whales (CIBWs;Delphinapterus leucas), are an isolated and declining endangered population in Alaska, USA. Their critical habitat has been broadly defined, with little empirical data on habitat use to direct management actions. This article examines data collected during surveys for a long‐term photo‐ID study to gain insight into the use of critical habitat by CIBW groups and individuals, with a focus on areas where belugas were observed feeding, calving, rearing young, and in transit. It also describes patterns of group distribution, size, and composition, according to sex and age class, in order to better understand how CIBWs are distributed throughout critical habitat during the ice‐free season. The results are presented according to survey month and survey zone, so that patterns of habitat use are accessible in a useful format to aid managers in evaluations of how timing and location of human activities occurring in CIBW critical habitat may overlap with CIBW occurrence and use of these areas.There were 575 beluga groups encountered during 477 photo‐ID surveys conducted in CIBW critical habitat 2005–2017. CIBW locations were mapped, and models were developed to test for temporal and spatial patterns. Approximately 400 individual whales were identified, and their sighting histories were compiled to examine residency and movement patterns.CIBWs were found seasonally in distinct areas, where they aggregate in large groups of both sexes and all age classes while rearing calves and feeding. These areas, and the general corridors connecting them, represent important beluga habitat that warrant focused management attention and protection efforts.Whales travelling among distinct areas of Cook Inlet increase their likelihood of exposure to multiple, localized threats.Habitat protection and the regulation of anthropogenic activities should consider cumulative effects of all activities in the range of CIBWs and their potential to affect the entire population.
Recent Advances in the Cycloaddition Reactions of 2‐Benzylidene‐1‐benzofuran‐3‐ones, and Their Sulfur, Nitrogen and Methylene Analogues
Authors: Deng Qingsong , Meng Xiangtai 
Published: July 2020

AbstractBenzothiophene, benzofuran, indole, and indene derivatives are privileged heterocyclic motifs. These are present in a wide range of bioactive natural products and pharmaceutical drugs and are the subject of materials science research. However, the construction of benzothiophene, benzofuran, indole, and indene frameworks have been long‐standing challenges to organic chemists. In this review, we classify the derivatives of four structures synthesized from 2‐benzylidene‐1‐benzofuran‐3‐one and their analogues in terms of their ring size (from three‐ to ten‐membered) and type (fused or spiro), as well as summarizing the developments of this field. Finally, we discuss the ring opening and 1,4‐addition reactions.
Rapid and sensitive detection of Mycobacterium tuberculosis based on strand displacement amplification and magnetic beads
Authors: Chu Zhao Jun , Xiao Sai Jin , Yuan Ming Yue , Wang Li Zhi , Wang Shan Ping , Zhang Guang Mei , Zhang Zhi Bin 
Published: July 2020

AbstractTuberculosis is one of the main infectious diseases threatening public health, and the development of simple, rapid, and cost‐saving methods for tuberculosis diagnosis is of profound importance for tuberculosis prevention and treatment. The bacterium Mycobacterium tuberculosis (MTB) is the pathogen that causes tuberculosis, and assaying for MTB is the only criterion for tuberculosis diagnosis. A new enzyme‐free method based on strand displacement amplification and magnetic beads was developed for simple, rapid, and cost‐saving MTB detection. Under optimum conditions, a good linear relationship could be observed between fluorescence and MTB specific DNA concentration ranging from 0.05 to 150 nM with a correlation coefficient of 0.993 (n = 8) and a detection limit of 47 pM (3σ/K). The present method also distinguished a one base mismatch from MTB specific DNA, showing great promise for MTB genome single base polymorphism analysis. MTB specific DNA content in polymerase chain reaction samples was successfully detected using the new method, and recoveries were 97.8–100.8%, indicating that the present method had high accuracy and shows good potential for the early diagnosis of tuberculosis.
Enzyme‐coupled fluorescence sensor for sensitive determination of uric acid and uricase inhibitor
Authors: Jiao Yuan , Xing Yunlong , Li Kai , Li Zainan , Zhao Guoqing 
Published: July 2020

AbstractIn this study, an enzyme‐coupled fluorescence sensor was developed successfully for the sensitive detection of uric acid (UA) and uricase inhibitor based on graphene quantum dots (GQDs). UA was first oxidized using uricase and produced hydrogen peroxide (H2O2), which could oxidize phenol to benzoquinone in the presence of horseradish peroxidase (HRP) as the catalysis. Benzoquinone is an efficient quencher and can cause fluorescence quenching of GQDs. The degree of quenching was proportional to UA concentration and was linear for the UA concentration in the ranges 0.2–14 μmol/L. The detection limit was 0.06 μmol/L for UA. In addition, the proposed sensor was further utilized for UA detection in human serum samples with satisfactory reproducibility and accuracy. The proposed strategy provided may be widely utilized to sense H2O2 or H2O2 generating processes in related biological environment.