bluuebunny/crossref_metadata_2025_split huggingface dataset:
NRAS associated RASopathy and embryonal rhabdomyosarcoma
Authors: Garren Benjamin , Stephan Mark , Hogue Jacob S. 
Published: November 2019

AbstractRASopathies are a group of phenotypically overlapping disorders that arise from dysregulation of the RAS/MAPK pathway. These disorders include Noonan syndrome, Costello syndrome, cardiofaciocutaneous syndrome, and neurofibromatosis‐Type 1. While somatic mutations in the three human Ras genes (KRAS, HRAS, and NRAS) are a common finding in a variety of cancers, germline mutations in each of the these genes cause developmental RASopathy phenotypes with mutations in specific genes typically correlating with specific phenotypes. We present the case of a germline heterozygous NRAS mutation producing a severe phenotype involving embryonal rhabdomyosarcoma, severe intellectual disability, and numerous melanocytic nevi in addition to more typical manifestations of Noonan syndrome. Additionally, the specific p.G12R NRAS mutation in this case is a common somatic mutation in cancer cells, and analysis of previously reported NRAS‐RASopathy cases suggests that mutations at traditionally oncogenic codons are associated with elevated cancer risk not present with mutations at other sites.
Recent advances in shear‐thinning and self‐healing hydrogels for biomedical applications
Authors: Uman Selen , Dhand Abhishek , Burdick Jason A. 
Published: November 2019

ABSTRACTShear‐thinning and self‐healing hydrogels are being investigated in various biomedical applications including drug delivery, tissue engineering, and 3D bioprinting. Such hydrogels are formed through dynamic and reversible interactions between polymers or polypeptides that allow these shear‐thinning and self‐healing properties, including physical associations (e.g., hydrogen bonds, guest–host interactions, biorecognition motifs, hydrophobicity, electrostatics, and metal–ligand coordination) and dynamic covalent chemistry (e.g., Schiff base, oxime chemistry, disulfide bonds, and reversible Diels–Alder). Their shear‐thinning properties allow for injectability, as the hydrogel exhibits viscous flow under shear, and their self‐healing nature allows for stabilization when shear is removed. Hydrogels can be formulated as uniform polymer and polypeptide assemblies, as hydrogel nanocomposites, or in granular hydrogel form. This review focuses on recent advances in shear‐thinning and self‐healing hydrogels that are promising for biomedical applications. © 2019 Wiley Periodicals, Inc. J. Appl. Polym. Sci.2020,137, 48668.
High performance cellulose fibers regenerated from 1‐butyl‐3‐methylimidazolium chloride solution: Effects of viscosity and molecular weight
Authors: Zhang Jiaping , Yamagishi Naoki , Gotoh Yasuo , Potthast Antje , Rosenau Thomas 
Published: November 2019

ABSTRACTIn the present study, we focused on several factors affecting the utility of 1‐butyl‐3‐methylimidazolium chloride (BMIMCl) for obtaining higher performance fibers. The dependence of the spinnability and tensile strength of the fibers on the zero‐shear viscosity of the spinning solutions was investigated based on differences in the molecular weight of the cellulose, pulp concentration, and the pH of BMIMCl. We demonstrated an appropriate viscosity range of 2000–4000 Pa s−1 (100 °C) for spinning dopes to obtain good spinnability and high tensile strength. The pH of the BMIMCl and the molecular weight of the cellulose clearly impacted tensile strength. The high molecular weight of cellulose contributed to high mechanical properties of the regenerated cellulose fibers. Optimizing the molecular weight and concentration of the cellulose based on the appropriate viscosity allowed us to prepare high performance cellulose fibers with a tensile strength of 1.15 GPa and a Young's modulus of 42.9 GPa. © 2019 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2019, 137, 48681.
In situ polymerization of flame retardant modification polyamide 6,6 with 2‐carboxy ethyl (phenyl) phosphinic acid
Authors: Chen Xiangyang , Xu Dongfeng , Zhang Hua , Feng Xinxing , Deng Jianping , Pan Kai 
Published: November 2019

ABSTRACTA novel halogen‐free flame retardant copolyamide 6,6 (FR‐PA66) was prepared successfully by in situ polymerizing with adipic acid hexamethylene salt and 2‐carboxy ethyl (phenyl) phosphinic acid (CEPPA). The elemental composition and chemical structure of FR‐PA66 were characterized by energy dispersive X‐ray spectroscopy, Fourier transform infrared spectrometer and 13C Nuclear magnetic resonance spectrometer. The flame retardancy, thermal stability, and morphology of char residues were also investigated by the limiting oxygen index (LOI), UL 94 test, thermogravimetric analysis, and scanning electron microscopy. The results showed that FR‐PA66 samples had much better flame retardancy and char formation ability than pure PA66 after the flame retardant modification. The LOI values were increased from 24.0 to 28.0% by adding 6 wt % of CEPPA and all FR‐PA66 samples were rated as V‐0 rating in UL‐94 test. Furthermore, the thermal stability analysis indicated that in situ polymerization with CEPPA effectively decreased the initial decomposition temperature and increased the amount of char residue. © 2019 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2019, 137, 48687.
GRK5 Inhibition Attenuates Cartilage Degradation via Decreased NF‐κB Signaling
Authors: Sueishi Takuya , Akasaki Yukio , Goto Norio , Kurakazu Ichiro , Toya Masakazu , Kuwahara Masanari , Uchida Taisuke , Hayashida Mitsumasa , Tsushima Hidetoshi , Bekki Hirofumi , Lotz Martin K. , Nakashima Yasuharu 
Published: November 2019

ObjectiveNF‐κB–dependent signaling is an important modulator in osteoarthritis (OA), and G protein–coupled receptor kinase 5 (GRK5) regulates the NF‐κB pathway. This study was undertaken to investigate the functional involvement of GRK5 in OA pathogenesis.MethodsGRK5 expression in normal and OA human knee joints was analyzed immunohistochemically. Gain‐ or loss‐of‐function experiments were performed using human and mouse chondrocytes. OA was induced in GRK5‐knockout mice by destabilization of the medial meniscus, and histologic examination was performed. OA was also induced in wild‐type mice, which were then treated with an intraarticular injection of amlexanox, a selective GRK5 inhibitor, every 5 days for 8 weeks.ResultsGRK5 protein expression was increased in human OA cartilage. In vitro, expression levels of OA‐related factors and NF‐κB transcriptional activation were down‐regulated by suppression of the GRK5 gene in human OA chondrocytes (3.49‐fold decrease in IL6 [P < 0.01], 2.43‐fold decrease in MMP13 [P < 0.01], and 2.66‐fold decrease in ADAMTS4 [P < 0.01]). Conversely, GRK5 overexpression significantly increased the expression of OA‐related catabolic mediators and NF‐κB transcriptional activation. On Western blot analysis, GRK5 deletion reduced IκBα phosphorylation (up to 4.4‐fold decrease [P < 0.05]) and decreased p65 nuclear translocation (up to 6.4‐fold decrease [P < 0.01]) in mouse chondrocytes. In vivo, both GRK5 deletion and intraarticular amlexanox protected mouse cartilage against OA.ConclusionOur results suggest that GRK5 regulates cartilage degradation through a catabolic response mediated by NF‐κB signaling, and is a potential target for OA treatment. Furthermore, amlexanox may be a major compound in relevant drugs.
Adaptive robust dynamic surface control for uncertain strict‐feedback nonlinear systems using fuzzy logic systems
Authors: Chen Sanyan , Wang Yuchao , Shi Wenrui , Wu Hansheng 
Published: November 2019

AbstractIn this paper, an adaptive robust stabilization problem is dealt with for a class of uncertain strict‐feedback nonlinear systems in the presence of unknown structure uncertainties, external disturbances, and unknown time‐varying virtual control coefficients. It is not required to know the upper bounds of external disturbances, as well as the upper and lower bounds of unknown time‐varying virtual control coefficients. The controller is designed by adopting backstepping. In addition, to avoid suffering from the problem of ‘explosion of terms’, a dynamic surface control approach is employed by introducing the first‐order low‐pass filter. Furthermore, at every step of the backstepping design procedure, fuzzy logic systems are used to approximate the unknown structure uncertainties. In particular, the norms of weight matrices and the upper bounds of approximation errors of fuzzy logic systems are supposed to be unknown. It is also shown that the proposed controller can guarantee the uniform boundedness of uncertain strict‐feedback nonlinear systems. Finally, the simulation for a single‐link manipulator actuated by a brush DC motor is carried out to illustrate the validity of the proposed controller.
Meta‐Analyses Support Previous and Novel Autism Candidate Genes: Outcomes of an Unexplored Brazilian Cohort
Authors: da Silva Montenegro Eduarda Morgana , Costa Claudia Samogy , Campos Gabriele , Scliar Marília , de Almeida Tatiana Ferreira , Zachi Elaine Cristina , Silva Isabela Maya Wahys , Chan Ada J. S. , Zarrei Mehdi , Lourenço Naila C. V. , Yamamoto Guilherme Lopes , Scherer Stephen , Passos‐Bueno Maria Rita 
Published: November 2019

Large genomic databases of neurodevelopmental disorders (NDD) are helpful resources of genomic variations in complex and heterogeneous conditions, as Autism Spectrum Disorder (ASD). We evaluated the role of rare copy number variations (CNVs) and exonic de novo variants, in a molecularly unexplored Brazilian cohort of 30 ASD trios (n = 90), by performing a meta‐analysis of our findings in more than 20,000 patients from NDD cohorts. We identified three pathogenic CNVs: two duplications on 1q21 and 17p13, and one deletion on 4q35. CNVs meta‐analysis (n = 8,688 cases and n = 3,591 controls) confirmed 1q21 relevance by identifying duplications in other 16 ASD patients. Exome analysis led the identification of seven de novo variants in ASD genes (SFARI list): three loss‐of‐function pathogenic variants in CUL3, CACNA1H, and SHANK3; one missense pathogenic variant in KCNB1; and three deleterious missense variants in ATP10A, ANKS1B, and DOCK1. From the remaining 12 de novo variants in non‐previous ASD genes, we prioritized PRPF8 and RBM14. Meta‐analysis (n = 13,754 probands; n = 2,299 controls) identified six and two additional patients with validated de novo variants in PRPF8 and RBM14, respectively. By comparing the de novo variants with a previously established mutational rate model, PRPF8 showed nominal significance before multiple test correction (P = 0.039, P‐value adjusted = 0.079, binomial test), suggesting its relevance to ASD. Approximately 60% of our patients presented comorbidities, and the diagnostic yield was estimated in 23% (7/30: three pathogenic CNVs and four pathogenic de novo variants). Our uncharacterized Brazilian cohort with tetra‐hybrid ethnic composition was a valuable resource to validate and identify possible novel candidate loci. Autism Res 2020, 13: 199–206. © 2019 International Society for Autism Research, Wiley Periodicals, Inc.Lay SummaryWe believed that to study an unexplored autistic population, such as the Brazilian, could help to find novel genes for autism. In order to test this idea, with our limited budget, we compared candidate genes obtained from genomic analyses of 30 children and their parents, with those of more than 20,000 individuals from international studies. Happily, we identified a genetic cause in 23% of our patients and suggest a possible novel candidate gene for autism (PRPF8).
Hybrid transcatheter pulmonary valve replacement with a SAPIEN S3 valve after pulmonary artery banding via left lateral thoracotomy
Authors: Serfas John D. , Turek Joseph , Haney John , Krasuski Richard A. , Fleming Gregory A. 
Published: November 2019

AbstractFor many patients with repaired congenital heart disease, the need for reintervention on dysfunctional right ventricular outflow tracts is pervasive. Many such patients are poor candidates for both transcatheter pulmonary valve replacement and cardiopulmonary bypass, and hybrid surgical and transcatheter procedures have evolved to meet this need. We present two cases of hybrid pulmonary valve replacement involving pulmonary artery band placement via left anterior thoracotomy followed by transvenous placement of a SAPIEN S3 valve without prestenting. This approach avoids cardiopulmonary bypass as well as redo sternotomy and will likely see an increase in utilization in the future.
Considerations of psychotic symptomatology in anti‐NMDA encephalitis: Similarity to cycloid psychosis
Authors: Giné Servén Eloi , Boix Quintana Ester , Guanyabens Buscà Nicolau , Casado Ruiz Virginia , Torres Rivas Cristina , Niubo Gurgui Marta , Dalmau Josep , Palma Carol 
Published: November 2019

AbstractMost patients with anti‐NMDA receptor (NMDAR) encephalitis present with acute psychosis which is difficult to differentiate from psychotic episodes related to a primarily psychiatric disease. A precise description of the psychiatric phenotype of this disease would greatly facilitate the early diagnosis of these patients. We provide here a detailed description of three of these patients and the similarity of the clinical features with cycloid psychosis. All three patients met Perris and Brockington's criteria for cycloid psychosis in the initial phase of the autoimmune process, including among other an acute and polysymptomatic onset, polymorphous psychotic symptomatology, mood swings, and changes in psychomotricity. In addition, none of the patients had experienced an extended psychiatric prodromal phase. External stress factors preceded symptom onset in the three patients, who also showed common base personality traits and intolerance to a range of antipsychotic treatments. Complementary studies disclosed that the three patients had ovarian teratoma as well as abnormal EEG, and CSF antibodies against NMDAR. Patients with anti‐NMDAR encephalitis may present with clinical features that resemble cycloid psychosis. In addition, our patients did not have prodromal history of psychiatric symptoms and showed intolerance to antipsychotic medication, which all should raise concern for anti‐NMDAR encephalitis, prompting CSF antibody testing.
Eruption disturbance caused by an odontoma that was partially attached to the crown of the adjacent maxillary first molar, and step lesion excavation for the tooth preservation: A case report
Authors: Miura Kenichi , Miyashin Michiyo , Kanao Takako , Oishi Atsushi 
Published: November 2019

AbstractDespite the partial attachment of a complex odontoma to an impacted tooth, it is possible to guide the tooth to erupt normally and preserve it by step lesion excavation and the prevention of infection.
Continuous Photo‐Electro‐Catalytic Synthesis of Bio‐Based Adipic Acid with Reaction Kinetics Modeling
Authors: Suhadolnik Luka , Bajec David , Žigon Dušan , Čeh Miran , Likozar Blaž 
Published: November 2019

AbstractPhoto‐electro‐catalytic oxidation reactions of 1,6‐hexanediol were carried out in a coil‐type continuous‐flow microreactor with an immobilized TiO2 catalyst material. Bio‐based adipic acid was formed as the only intermediate product before the total mineralization process. The production was stable, enabling green, selective and continuous resource conversion, but with low, non‐optimized yields. In addition, the catalyzed chemical kinetics was modeled, based on mechanisms as well as approximated rate‐determining steps. The Butler‐Volmer relation was used for activation. The electro‐catalytic oxidative pathway was demonstrated to dominate over photo‐catalysis, also on account of the band gap characteristics of the titania nanotube electrode.
Hydroxylation of Aromatics by H2O2 Catalyzed by Mononuclear Non‐heme Iron Complexes: Role of Triazole Hemilability in Substrate‐Induced Bifurcation of the H2O2 Activation Mechanism
Authors: Rebilly Jean‐Noël , Zhang Wenli , Herrero Christian , Dridi Hachem , Sénéchal‐David Katell , Guillot Régis , Banse Frédéric 
Published: November 2019

AbstractRieske dioxygenases are metalloenzymes capable of achieving cis‐dihydroxylation of aromatics under mild conditions using O2 and a source of electrons. The intermediate responsible for this reactivity is proposed to be a cis‐FeV(O)(OH) moiety. Molecular models allow the generation of a FeIII(OOH) species with H2O2, to yield a FeV(O)(OH) species with tetradentate ligands, or {FeIV(O); OH.} pairs with pentadentate ones. We have designed a new pentadentate ligand, mtL42, bearing a labile triazole, to generate an “in‐between” situation. Two iron complexes, [(mtL42)FeCl](PF6) and [(mtL42)Fe(OTf)2]), were obtained and their reactivity towards aromatic substrates was studied in the presence of H2O2. Spectroscopic and kinetic studies reflect that triazole is bound at the FeII state, but decoordinates in the FeIII(OOH). The resulting [(mtL42)FeIII(OOH)(MeCN)]2+ then lies on a bifurcated decay pathway (end‐on homolytic vs. side‐on heterolytic) depending on the addition of aromatic substrate: in the absence of substrate, it is proposed to follow a side‐on pathway leading to a putative (N4)FeV(O)(OH), while in the presence of aromatics it switches to an end‐on homolytic pathway yielding a {(N5)FeIV(O); OH.} reactive species, through recoordination of triazole. This switch significantly impacts the reaction regioselectivity.
Models for the Numerical Simulation of Bubble Columns: A Review
Authors: Mühlbauer Adam , Hlawitschka Mark W. , Bart Hans‐Jörg 
Published: November 2019

AbstractThis work reviews the state‐of‐the‐art models for the simulation of bubble columns and focuses on methods coupled with computational fluid dynamics (CFD) where the potential and deficits of the models are evaluated. Particular attention is paid to different approaches in multiphase fluid dynamics including the population balance to determine bubble size distributions and the modeling of turbulence where the authors refer to numerous published examples. Additional models for reactive systems are presented as well as a special chapter regarding the extension of the models for the simulation of bubble columns with a present solid particle phase, i.e., slurry bubble columns.
Experimental investigation of high‐temperature corrosion properties in simulated reducing‐sulphidizing atmospheres of the waterwall fireside in the boiler
Authors: Xu Ligang , Huang Yaji , Wang Jian , Liu Changqi , Liu Lingqin , Zou Lei , Yue Junfeng , Chen Kuixu 
Published: November 2019

AbstractThe presence of reducing atmospheres of the waterwall fireside in the boiler due to extensive use of low‐NOx combustion mode caused severe high‐temperature corrosion problems. In this study, high‐temperature sulphur corrosion properties of two types of low alloyed waterwall steel (15CrMoG and 12Cr1MoVG) in two kinds of reducing‐sulphidizing atmospheres were investigated by lab‐scale experiments. The experimental atmospheres and temperature were simulated according to field measurements in the boiler of a thermal power plant. The experimental results showed that the reducing‐sulphidizing atmosphere with CO was more corrosive, and the CO accelerated the corrosion of H2S and worsened the corrosion. The CO inhibited the formation of oxide scales, provided some corrosion resistance, and produced the intensely corrosive COS. The corrosivity of the alkali metal chlorides was limited in the reducing‐sulphidizing atmosphere without CO, while the corrosion was strongly hindered in the reducing‐sulphidizing atmosphere with CO.
Oxidative Cyclization‐Induced Activation of a Phosphoinositide 3‐Kinase Inhibitor for Enhanced Selectivity of Cancer Chemotherapeutics
Authors: Zhu Haizhou , Mishra Rosalin , Yuan Long , Abdul Salam Safnas F. , Liu Jing , Gray George , Sterling Alyssa D. , Wunderlich Mark , Landero‐Figueroa Julio , Garrett Joan T. , Merino Edward J. 
Published: November 2019

AbstractIn this work, we designed a prodrug that reacts with cellular oxidative equivalents leading to ether cleavage and cyclization to release an active phosphatidylinositol 3‐kinase (PI3K) inhibitor. We show that the compound reduces affinity for PI3KA relative to the PI3K inhibitor, is slow to intercellularly oxidize, and is resistant to liver microsomes. We observed modest activity in untreated acute myeloid leukemia cells and 14‐fold selectivity relative to non‐cancerous cells. The cellular activity of the compound can be modulated by the addition of antioxidants or oxidants, indicating the compound activity is sensitive to cellular reactive oxygen species (ROS) state. Co‐treatment with cytosine arabinoside or doxorubicin was used to activate the compound inside cells. We observed strong synergistic activity specifically in acute myeloid leukemia (AML) cancer cells with an increase in selective anticancer activity of up to 90‐fold. Thus, these new self‐cyclizing compounds can be used to increase the selectivity of anticancer agents.
An IEEE 802.21 MIS‐based mobility management for D2D communications over heterogeneous networks (HetNets)
Authors: Xu Yi‐Han , Liu Meng‐Lian , Xie Jing‐Wei , Zhou Jun 
Published: November 2019

SummaryThe rapid growth and innovation of the various mobile communication technologies have caused a change in the paradigm of Internet access. In next‐generation mobile system, which is also known as fifth‐generation (5G) network, all user connections are expected to be heterogeneous in nature. Hence, an intelligent mobility management mechanism is essential to satisfy the requirements of lower latency, less power consumptions, and last but not least, possible uninterrupted ongoing session when a user equipment (UE) moves across network boundaries. On the other hand, device‐to‐device (D2D) communication has been proposed as a promising technology to boost the network performance in 5G. The issue related to mobility management for D2D communication in 5G heterogeneous network environments has attracted attention from academic society. In this paper, we leverage on IEEE 802.21 Media Independent Service (MIS) and propose a new framework, enhanced version of IEEE 802.21 MIS that supports D2D communication (E‐MIS‐D2D) to enhance D2D mobility experience over heterogeneous networks. Moreover, a load‐aware mode selection algorithm is proposed for selecting the best target mode. Through extensive simulation studies, we validate that the proposed method outperforms the existing methods in terms of packet loss ratio, average throughput, latency, bandwidth usage, and load rate of Evolved Node B (eNB).