bluuebunny/crossref_metadata_2025_split huggingface dataset:
The youngest pair of siblings with Mucopolysaccharidosis type IVA to receive enzyme replacement therapy to date: A case report
Authors: Frigeni Marta , Rodriguez‐Buritica David F. , Saavedra Heather , Gunther Kathryn A. , Hillman Paul R. , Balaguru Duraisamy , Northrup Hope 
Published: September 2021

AbstractMucopolysaccharidosis type IVA (OMIM 253000) is an autosomal recessive disorder caused by defective activity of the N‐acetylgalactosamine 6‐sulfatase (GALNS) enzyme. In 2014, enzyme replacement therapy (ERT) using recombinant human GALNS became available for affected patients. There is a limited number of studies to date that have explored the effect of ERT in infancy and there is also a lack of data assessing the effect of ERT in systems other than the skeletal. Here, we report on the effect of ERT in the youngest pair of siblings treated to date: Patient A, currently 4 years old, who started treatment at the age of 5 months; and Patient B, currently 3 years old, who started treatment at 58 days of life. Moreover, we investigate the effect of early ERT on the cardiovascular system. Our results show that, even when ERT is started before 2 months of age, it cannot fully prevent disease progression. As for the effect of ERT on the cardiovascular system, our preliminary results suggest that early treatment might play a role in preserving a normal left ventricular mass index in affected patients at least up to 1 year, but further observation over time will be required. Overall, this report shows that early diagnosis remains crucial and that prompt initiation of ERT has limited effect in slowing progression of the skeletal phenotype, thus confirming the need for new therapeutic approaches that target the skeletal system in affected patients.
Nucleophilic Phosphine Catalysis: The Untold Story
Authors: Khong San , Venkatesh Telugu , Kwon Ohyun 
Published: September 2021

AbstractThis Minireview provides insight into the early history of nucleophilic phosphinocatalysis. The concepts of 1,4‐addition of a tertiary phosphine to an α,β‐enone and of equilibrium between the resulting phosphonium zwitterion and phosphonium ylide established a fundamental basis for the development of several classical transformations, including the Rauhut‐Currier, Morita, McClure‐Baizer‐Anderson, and Oda reactions.
Evaluation of tensile strength retention and service life prediction of hydrothermal aged balanced orthotropic carbon/glass and Kevlar/glass fabric reinforced polymer hybrid composites
Authors: Muralidharan Muniraju , Sathishkumar Thottyeapalayam Palanisamy , Rajini Nagarajan , Navaneethakrishnan Palanisamy , Arun Kumar Shanmugam , Ismail Sikiru Oluwarotimi , Senthilkumar Krishnasamy , Siengchin Suchart 
Published: September 2021

AbstractQuest to reduce challenges of high structural weight and cost of metallic components is increasing. It has led to their replacement with carbon and Kevlar fibers reinforced polymer (FRP) composites. These aforementioned problems can be further solved through hybridization of carbon/glass (CG) and Kevlar/glass (KG) fibers to reduce the manufacturing cost and materials usage, not at detriment of their properties. Also, investigation into their tensile properties, diffusivity, and service life is germane. Therefore, the present study focuses on influence of hybridization of CG and KG fibers on seawater diffusivity, service life, and tensile strengths of their composite systems, through hydrothermal aging. The hybrid composites were aged in seawater for 50, 150, and 300 days at temperatures of 20, 40, and 60°C. From the results obtained, it was evident that the maximum moisture absorption of both FRP hybrid composites occurred at 60°C in 300 days of hydrothermal aging. The maximum tensile strengths were obtained in unaged composite counterparts. Also, the aged FRP hybrid composites exhibited the lowest tensile strengths at 150 days. The retention of maximum tensile strengths of CG and KG FRP hybrid composites showed 75% and 70% for 100 years at hydrothermal aging temperature of 40°C. Therefore, both FRP hybrid composite samples exhibited promising behaviors for various marine and outdoor applications.
Effect of interfacial properties of filled carbon black nanoparticles on the conductivity of nanocomposite
Authors: Shao Jiang , Liu Xue , Ji Mingbo 
Published: September 2021

AbstractIn this study, an optimized model is proposed for predicting the effective conductivity of nanocomposites, which contains conductive spherical fillers above the percolation threshold. The influence of filler properties, interphase regions, filler concentrations, elemental cell sizes as well as contact area on the effective conductivity of nanocomposites was investigated and discussed in detail. The developed model is verified by using the experimental data reported in the literatures, and it is found that the prediction results are fitted well with the experimental results at different particle concentrations. In addition, high levels of filler conductivity, particle concentration, and elemental cell size led to a good conductivity. The maximum conductivity of nanocomposite with σeff = 1,500,000 S/m, Vf = 0.07, r = 10−4 nm, and D = 1250 nm is obtained at Ri = 40 nm and d = 10 nm. Furthermore, the conductivity of nanocomposite is not sensitive to interphase thickness, when d is larger than 25 nm.
Phenol functionalized high‐density polyethylene as compatibilizer of high‐density polyethylene/graphene nanocomposites toward enhanced mechanical and interfacial adhesion
Authors: Zapata‐Domínguez Alejandra , Martínez‐Colunga Juan Guillermo , Sánchez‐Valdes Saul , da Silva Luciano , Hernandez‐Gamez Jose Francisco , Ramos‐deValle Luis Francisco , Rodriguez‐Gonzalez José Alberto , Espinoza‐Martinez Adriana Berenice , Rodriguez‐Fernandez Oliverio Santiago , Ramírez‐Vargas Eduardo 
Published: September 2021

AbstractIn this work, the methodology to obtain high‐density polyethylene (HDPE) and graphene (G) nanocomposites using phenol‐modified graphene and phenol‐modified HDPE as compatibilizer obtained from the reaction of malleated polyethylene (HDPE‐g‐MA) with phenol, is described. Six different methods were used to obtain these composites, resulting from the combination of melt and solution mixing with three different procedures to functionalize either graphene or HDPE‐g‐MA with phenol. Nanocomposites were characterized by Fourier transform infrared (FTIR), scanning electron microscopy (SEM), wide angle X‐ray diffraction (WAXD), dynamic mechanic analysis (DMA) and tensile properties. The FTIR results evidenced the occurrence of an esterification reaction between HDPE‐g‐MA and phenol groups serving as compatibilizer. The SEM and WAXD results showed that the addition of this compatibilizer agent had a positive effect on graphene dispersion, which promoted greater stiffness in these composite materials, presenting higher storage modulus. In addition, a noticeable enhancement in dimensional stability at high temperatures was observed according to Tan Delta measurements. These results indicated a good interfacial adhesion between graphene and the polymer matrix by using this compatibilizer agent. It was observed that the composites obtained by solution mixing method showed better performance and stronger interfacial adhesions than the composites produced by melt mixing methods. The use of this phenol modified HDPE compatibilizer offers a new option to enhance the final performance of HDPE/graphene composites with better mechanical and thermal properties to be used in materials for various applications, such as automotive industry, electrical and electronics, construction, aerospace, and other engineering applications.
Synthesis of phosphorus and silicon co‐doped graphitic carbon nitride and its combination with ammonium polyphosphate to enhance the flame retardancy of epoxy resin
Authors: Zhang Wenyi , Chen Zhongwei , Yu Yuan , Chen Tingting , Zhang Qingwu , Li Changxin , Chen Zhiquan , Gao Wei , Jiang Juncheng 
Published: September 2021

AbstractPhosphorus and silicon co‐doped graphitic carbon nitride (PSiCN) was synthesized by inducing the polycondensation reaction of melamine, diammonium hydrogen phosphate, and silicic acid. The structure and thermal properties of PSiCN were characterized. With the incorporation of 2 wt% of PSiCN and 8 wt% of ammonium polyphosphate (APP), EP/2PSiCN‐8APP composite exhibited a limiting oxygen index value of 29.0% and passed a UL‐94V‐0 rating, which reached the refractory level. The thermogravimetric analysis confirmed that the addition of PSiCN and APP improved the thermal stability of the composites. The cone calorimetry test (CCT) revealed that the peak of heat release rate, total heat release, the peak of smoke production rate and total smoke production of EP/2PSiCN‐8APP composite was reduced by 71.1%, 53.5%, 65.4%, and 53.5%, respectively, compared with pure EP. Furthermore, the residues after CCT were evaluated by scanning electron microscopy, laser Raman spectroscopy and Fourier transform infrared spectroscopy, and the flame retardant mechanism of EP/2PSiCN‐8APP composite was proposed. Finally, the parallel arrangement of the aromatic ring and the surface of the PSiCN and the synergistic effect of the P/N/Si multi‐element resulted in EP/2PSiCN‐8APP with excellent mechanical properties.
Metal‐Free Chemoselective Oxidation of 4‐Methylquinolines into Quinoline‐4‐Carbaldehydes
Authors: Xu Jincheng , Li Yang , Ding Tianling , Guo Hao 
Published: September 2021

AbstractA convenient protocol for the synthesis of quinoline‐4‐carbaldehydes via chemoselective oxidation of 4‐methylquinolines using hypervalent iodine(III) reagents as oxidant is described. This method highlights metal‐free and mild reaction conditions, nice yield, good functional group tolerance, and high chemoselectivity.
Biopsychological correlates of repetitive and restricted behaviors in autism spectrum disorders
Authors: Comparan‐Meza Miguel , Vargas de la Cruz Ivette , Jauregui‐Huerta Fernando , Gonzalez‐Castañeda Rocio E. , Gonzalez‐Perez Oscar , Galvez‐Contreras Alma Y. 
Published: September 2021

AbstractBackgroundAutism Spectrum Disorder (ASD) is considered a neurodevelopmental condition that is characterized by alterations in social interaction and communication, as well as patterns of restrictive and repetitive behaviors (RRBs). RRBs are defined as broad behaviors that comprise stereotypies, insistence on sameness, and attachment to objects or routines. RRBs can be divided into lower‐level behaviors (motor, sensory, and object‐manipulation behaviors) and higher‐level behaviors (restrictive interests, insistence on sameness, and repetitive language). According to the DSM‐5, the grade of severity in ASD partially depends on the frequency of RRBs and their consequences for disrupting the life of patients, affecting their adaptive skills, and increasing the need for parental support.MethodsWe conducted a systematic review to examine the biopsychological correlates of the symptomatic domains of RRBs according to the type of RRBs (lower‐ or higher‐level). We searched for articles from the National Library of Medicine (PubMed) using the terms: autism spectrum disorders, ASD, and autism‐related to executive functions, inhibitory control, inflexibility, cognitive flexibility, hyper or hypo connectivity, and behavioral approaches. For describing the pathophysiological mechanism of ASD, we also included animal models and followed PRISMA guidelines.ResultsOne hundred and thirty‐one articles were analyzed to explain the etiology, continuance, and clinical evolution of these behaviors observed in ASD patients throughout life.ConclusionsBiopsychological correlates involved in the origin of RRBs include alterations in a) neurotransmission system, b) brain volume, c) inadequate levels of growth factors, d) hypo‐ or hyper‐neural connectivity, e) impairments in behavioral inhibition, cognitive flexibility, and monitoring and f) non‐stimulating environments. Understanding these lower‐ and higher‐level of RRBs can help professionals to improve or design novel therapeutic strategies.
Inhibition of lipid metabolism exerts antitumor effects on rhabdomyosarcoma
Authors: Miyagaki Satoshi , Kikuchi Ken , Mori Jun , Lopaschuk Gary D. , Iehara Tomoko , Hosoi Hajime 
Published: September 2021

AbstractRhabdomyosarcoma exhibits tumor‐specific energy metabolic changes that include the Warburg effect. Since targeting cancer metabolism is a promising therapeutic approach, we examined the antitumor effects of suppressing lipid metabolism in rhabdomyosarcoma. We suppressed lipid metabolism in rhabdomyosarcoma cells in vitro by administering an inhibitor of malonyl‐CoA decarboxylase, which increases malonyl‐CoA and decreases fatty acid oxidation. Suppression of lipid metabolism in rhabdomyosarcoma cells decreased cell proliferation by inducing cell cycle arrest. Metabolomic analysis showed an increase in glycolysis and inactivation of the pentose phosphate pathway. Immunoblotting analysis revealed upregulated expression of the autophagy marker LC3A/B‐II due to increased phosphorylation of AMP‐activated protein kinase, a nutrient sensor. p21 protein expression level also increased. Inhibition of both lipid metabolism and autophagy suppressed tumor proliferation and increased apoptosis. In vivo studies involved injection of human Rh30 cells into the gastrocnemius muscle of 6‐week‐old female nude mice, which were divided into normal chow and low‐fat diet groups. The mice fed a low‐fat diet for 21 days showed reduced tumor growth compared to normal chow diet‐fed mice. Suppression of lipid metabolism disrupted the equilibrium of the cancer‐specific metabolism in rhabdomyosarcoma, resulting in a tumor growth‐inhibition effect. Therefore, the development of treatments focusing on the lipid dependence of rhabdomyosarcoma is highly promising.
Student mental health profiles and barriers to help seeking: When and why students seek help for a mental health concern
Authors: Broglia Emma , Millings Abigail , Barkham Michael 
Published: September 2021

AbstractBackgroundThe student mental health landscape in higher education requires preventative strategies that maintain daily functioning as well as early intervention to respond to episodes of mental ill‐health. As a community, this requires capturing the needs of students in the general university population as well as those using counselling and psychological wellbeing services.AimsThis cross‐sectional study aimed to characterise the mental health profile of students in the general university population and identify barriers to help seeking.MethodsA total of 1,956 students from five UK universities completed an online questionnaire comprising the CCAPS‐34 and open‐ended questions about their mental health concerns and intentions to seek help. Mental health profiles were explored using mixed factorial ANOVAs and post hoc simple effect analyses. Help‐seeking behaviours and intentions were identified using conceptual content analysis.ResultsDistinctive mental health profiles emerged across faculties and help‐seeking behaviours. Content analysis identified students’ help‐seeking experiences, intentions, preferences and barriers.ConclusionsCharacterising the mental health profile of students outside of support services, as well as their barriers to seeking help, provides a rich understanding for shaping preventative policies and service provision. Doing so will respond to student mental health needs before they require clinical intervention.
Revisiting the Molecular Interactions between the Tumor Protein TCTP and the Drugs Sertraline/Thioridazine
Authors: Malard Florian , Jacquet Eric , Nhiri Naima , Sizun Christina , Chabrier Amélie , Messaoudi Samir , Dejeu Jérôme , Betzi Stéphane , Zhang Xu , Thureau Aurélien , Lescop Ewen 
Published: September 2021

AbstractTCTP protein is a pharmacological target in cancer and TCTP inhibitors such as sertraline have been evaluated in clinical trials. The direct interaction of TCTP with the drugs sertraline and thioridazine has been reported in vitro by SPR experiments to be in the ∼30‐50 μM Kd range (Amson et al. Nature Med 2012), supporting a TCTP‐dependent mode of action of the drugs on tumor cells. However, the molecular details of the interaction remain elusive although they are crucial to improve the efforts of on‐going medicinal chemistry. In addition, TCTP can be phosphorylated by the Plk‐1 kinase, which is indicative of poor prognosis in several cancers. The impact of phosphorylation on TCTP structure/dynamics and binding with therapeutical ligands remains unexplored. Here, we combined NMR, TSA, SPR, BLI and ITC techniques to probe the molecular interactions between TCTP with the drugs sertraline and thioridazine. We reveal that drug binding is much weaker than reported with an apparent ∼mM Kd and leads to protein destabilization that obscured the analysis of the published SPR data. We further demonstrate by NMR and SAXS that TCTP S46 phosphorylation does not promote tighter interaction between TCTP and sertraline. Accordingly, we question the supported model in which sertraline and thioridazine directly interact with isolated TCTP in tumor cells and discuss alternative modes of action for the drugs in light of current literature.
Identifiability of Biologicals: An Analysis Using EudraVigilance, the European Union's Database of Reports of Suspected Adverse Drug Reactions
Authors: Correia Pinheiro Luis , Giezen Thijs J. , Wolff‐Holz Elena , Weise Martina , Laslop Andrea , Hidalgo‐Simon Ana 
Published: September 2021

The relevance of biological therapies for an increasing number of conditions is on the rise. Following the expiry of the initial period of market exclusivity, many of these successful therapies have seen the arrival of biosimilars on the market. The clear identification of the precise medicine responsible for an adverse drug reaction (ADR) report is an important element for pharmacovigilance, allowing timely detection of potential product‐specific safety signals. We looked at the identifiability of biologicals up to the level of commercial product name in ADR reports received from European clinical practice between 2011 and December 2019. A good level of identification (91.5%) was observed overall, but at the same time a downward trend was observed in the last 5 years. This reduction in the level of identifiability of biological products (originators and biosimilars) at the commercial name level in general was driven by five widely used substances, whereas the identification of all other biologics stayed consistent over time (at over 90%). We observed that those five substances were used mostly within oncology. The introduction of the first biosimilar in the market did not appear to affect their identifiability. These results show that although the general level of identification at the commercial product name level in ADRs in Europe is robust and generally stable over time, decreasing trends can be down to a few commonly used substances, which need to be monitored to reverse the trend.
Effects of traditional harvest and burning on common camas (Camassia quamash) abundance in Northern Idaho: The potential for traditional resource management in a protected area wetland
Authors: Stucki Devin S. , Rodhouse Thomas J. , Reuter Ron J. 
Published: September 2021

AbstractThe bulbs of common camas (Camassia quamash) were a staple food of Indigenous Peoples of western North America for millennia. Camas harvesting site productivity was encouraged through intense management. Common camas is considered a facultative wetland species, and populations have declined due to contemporary wetland drainage and land conversion. Conservation of existing habitat, as well as restoration of degraded systems, is necessary. Traditional ecological knowledge (TEK) and resource management (TRM) are often promoted as viable modes of contemporary resource management but are rarely tested or implemented. We designed a controlled experiment, informed by a born‐in‐the‐tradition specialist, to evaluate the response of common camas populations to traditional bulb harvest, burning, and a combination of harvest and burning. We recorded camas plant counts of three life stage classes of camas plants (single‐leaf seedling, multiple‐leaf adult, and flowering adult) over the course of 6 years in arrays of plots and subjected to treatments or left undisturbed as control. Harvesting removed plants (>800 bulbs) and reduced aboveground counts of camas densities ( ~ 50% of control, p < .05). Burning contributed to a reduction in single‐leaf plants but had an overall positive effect ( ~ 150% of control, p < .05) on adult camas and flowering plant abundance, and ameliorated the digging impacts. Treatment impacts tapered over the course of the study, and results indicate that a sustainable harvesting return interval of approximately 5 years may be possible when combined with fire to reduce litter and competition from pasture grasses and to accelerate the recovery of camas. Our findings support the hypotheses proposed by traditional knowledge specialists and ethnobotanists that digging and burning reduce intra‐ and interspecific competition and stimulate the growth of unharvested adult plants. More generally, our study supports the integration of traditional ecological knowledge into the evidence base available for protected area wetland prairie management.
Functional groups in piscivorous fishes
Authors: Mihalitsis Michalis , Bellwood David R. 
Published: September 2021

AbstractPiscivory is a key ecological function in aquatic ecosystems, mediating energy flow within trophic networks. However, our understanding of the nature of piscivory is limited; we currently lack an empirical assessment of the dynamics of prey capture and how this differs between piscivores. We therefore conducted aquarium‐based performance experiments, to test the feeding abilities of 19 piscivorous fish species. We quantified their feeding morphology, striking, capturing, and processing behavior. We identify two major functional groups: grabbers and engulfers. Grabbers are characterized by horizontal, long‐distance strikes, capturing their prey tailfirst and subsequently processing their prey using their oral jaw teeth. Engulfers strike from short distances, from high angles above or below their prey, engulfing their prey and swallowing their prey whole. Based on a meta‐analysis of 2,209 published in situ predator–prey relationships in marine and freshwater aquatic environments, we show resource partitioning between grabbers and engulfers. Our results provide a functional classification for piscivorous fishes delineating patterns, which transcend habitats, that may help explain size structures in fish communities.
Enantioselective Organocatalytic Synthesis of 1,2,3‐Trisubstituted Cyclopentanes
Authors: Šotolová Martina , Kamlar Martin , Remeš Marek , Géant Pierre‐Yves , Císařová Ivana , Štícha Martin , Veselý Jan 
Published: September 2021

AbstractAn organocatalytic asymmetric domino Michael/α‐alkylation reaction between enals and non‐stabilized alkyl halides has been developed. Chiral secondary amine catalyzed cyclization reaction of 1‐bromo‐3‐nitropropane with α,β‐unsaturated aldehydes provides 1,2,3‐trisubstituted cyclopentane carbaldehydes with high diastereo‐ (dr up to 8 : 1) and enantioselectivities (ee up to 96 %).