bluuebunny/crossref_metadata_2025_split huggingface dataset:
Extending the phenotypic spectrum of Bohring‐Opitz syndrome: Mild case confirmed by functional studies
Authors: Leon Eyby , Diaz Jullianne , Castilla‐Vallmanya Laura , Grinberg Daniel , Balcells Susanna , Urreizti Roser 
Published: November 2019

AbstractBohring‐Opitz syndrome (BOS) has been described as a clinically recognizable genetic syndrome since 1999. Clinical diagnostic criteria were established in 2011 and include microcephaly, trigonocephaly, distinctive craniofacial dysmorphic features, facial nevus flammeus, failure to thrive, and severe developmental delays. The same year, different de novo heterozygous nonsense mutations in the ASXL1 were found in affected individuals. Since then, several cases have been reported confirming the association between this chromatin remodeling gene and BOS. Most affected individuals die in early childhood because of unexplained bradycardia, obstructive apnea, or pulmonary infections. Those that survive usually cannot walk independently and are nonverbal. Some have had success using walkers and braces in late childhood. While few are able to speak, many have been able to express basic needs using communication devices as well as gestures with associated basic vocalizations. In this article, we present a mild case of BOS with a de novo pathogenic mutation c.1720‐2A>G (p.I574VfsX22) in ASXL1 detected on whole‐exome sequencing and confirmed by functional analysis of the messenger RNA splicing pattern on the patient's fibroblasts. She has typical dysmorphic features and is able to run and walk independently as well as to communicate with basic sign language.
HELLP syndrome: An ultrasound case of geographically altered liver parenchymal echogenicity with shear wave elastography, shear wave dispersion and attenuation imaging correlation
Authors: Mould Andrea , Abbott Steven , Welman Christopher J. 
Published: November 2019

AbstractHELLP syndrome is a rare, potentially life threatening obstetric condition that is diagnosed by the combination of Haemolysis, Elevated Liver enzyme levels, and Low Platelets (Pregnancy Hypertens, 1, 164, 2011). HELLP affects between 0.5% and 0.9% of all pregnancies (BMC Pregnancy Childbirth, 9, 8, 2009), however less than 0.5% of HELLP syndrome cases demonstrate any imaging abnormalities (Am J Roentgenol 185, 1205, 2005). This case report outlines the use of three new liver ultrasound technologies – shear wave elastography (SWE), shear wave dispersion (SWD) and attenuation imaging (ATI) in the evaluation of a patient with HELLP syndrome. The ultrasound applications were used to examine temporal changes of the disease with corresponding biochemical variations. The SWE and SWD values correlated with B mode ultrasound imaging and the clinical findings of HELLP.
Receptor activator of nuclear factor κB ligand is a biomarker for osteitis of chronic rhinosinusitis
Authors: Kong Il Gyu , Kim Dong‐Kyu , Eun Kyoung Mi , Yang Seung Koo , Kim Minju , Oh Heonjeong , Kim Dae Woo 
Published: November 2019

BackgroundEvidence of osteitis is frequently observed in patients with chronic rhinosinusitis (CRS), especially in recalcitrant cases. However, studies focusing on biological markers of osteitis are limited and it remains unclear whether osteitis is associated with different phenotypes of CRS. This study aimed to analyze the expression and assess the roles of receptor activator of nuclear factor κB ligand (RANKL) in patients with CRS and osteitis.MethodsCRS patients with nasal polyps (CRSwNP, n = 63), CRS patients without nasal polyps (CRSsNP, n = 8), and control subjects (n = 12) were enrolled. Histologic phenotypes, clinical information, and computed tomography (CT) scores were investigated. The Global Osteitis Scoring Scale (GOSS) and RANKL, a molecular marker of bone remodeling, were analyzed in each type of CRS. CRS mouse models were treated with anti‐RANKL.ResultsGOSS values were significantly higher in all CRS patients than in the control group. The GOSS value in non‐eosinophilic CRSwNP was higher than in eosinophilic CRSwNP. RANKL was upregulated whereas decoy receptor osteoprotegerin (OPG) was downregulated in CRS. RANKL messenger RNA (mRNA) and protein levels were positively correlated with GOSS. RANKL/OPG was increased in recurrent cases compared with primary cases. Multiple inflammatory mediators were positively correlated with the protein level of RANKL in CRS tissues. In the mouse CRSwNP model, anti‐RANKL treatment abrogated mucosal inflammation and bone remodeling.ConclusionRANKL expression is associated with clinical osteitis and disease severity in CRSwNP. These findings shed light on the importance of RANKL as a potential biomarker of CRS and a key player in CRS pathogenesis.
Homogeneous Oligomeric Ligands Prepared via Radical Polymerization that Recognize and Neutralize a Target Peptide
Authors: Hoshino Yu , Taniguchi Shohei , Takimoto Hinata , Akashi Sotaro , Katakami Sho , Yonamine Yusuke , Miura Yoshiko 
Published: November 2019

AbstractAbiotic ligands that bind to specific biomolecules have attracted attention as substitutes for biomolecular ligands, such as antibodies and aptamers. Radical polymerization enables the production of robust polymeric ligands from inexpensive functional monomers. However, little has been reported about the production of monodispersed polymeric ligands. Herein, we present homogeneous ligands prepared via radical polymerization that recognize epitope sequences on a target peptide and neutralize the toxicity of the peptide. Taking advantage of controlled radical polymerization and separation, a library of multifunctional oligomers with discrete numbers of functional groups was prepared. Affinity screening revealed that the sequence specificity of the oligomer ligands strongly depended on the number of functional groups. The process reported here will become a general step for the development of abiotic ligands that recognize specific peptide sequences.
Effective Utilization of NIR Wavelengths for Photo‐Controlled Polymerization: Penetration Through Thick Barriers and Parallel Solar Syntheses
Authors: Wu Zilong , Jung Kenward , Boyer Cyrille 
Published: November 2019

AbstractThis contribution details an efficient and controlled photopolymerization regulated by far‐red (λ=680 nm) and NIR (λ=780 and 850 nm) light in the presence of aluminium phthalocyanine and aluminium naphthalocyanine. Initiating radicals are generated by photosensitization of peroxides affording an effective strategy that provides controlled polymerization of a variety of monomers with excellent living characteristics. Critically, long wavelength irradiation provides penetration through thick barriers, affording unprecedented rates of controlled polymerization that can open new and exciting applications. Furthermore, a more optimized approach to performing solar syntheses is presented. By combining the narrow Q‐bands of these photocatalysts with others possessing complementary absorptions, layered, independent polymerizations and organic transformations may be performed in parallel under a single broadband emission source, such as sunlight.
Single Chromium Atoms Supported on Titanium Dioxide Nanoparticles for Synergic Catalytic Methane Conversion under Mild Conditions
Authors: Shen Qikai , Cao Changyan , Huang Runkun , Zhu Lei , Zhou Xin , Zhang Qinghua , Gu Lin , Song Weiguo 
Published: November 2019

AbstractDirect conversion of methane to value‐added chemicals with high selectivity under mild conditions remains a great challenge in catalysis. Now, single chromium atoms supported on titanium dioxide nanoparticles are reported as an efficient heterogeneous catalyst for direct methane oxidation to C1 oxygenated products with H2O2 as oxidant under mild conditions. The highest yield for C1 oxygenated products can be reached as 57.9 mol molCr−1 with selectivity of around 93 % at 50 °C for 20 h, which is significantly higher than those of most reported catalysts. The superior catalytic performance can be attributed to the synergistic effect between single Cr atoms and TiO2 support. Combining catalytic kinetics, electron paramagnetic resonance, and control experiment results, the methane conversion mechanism was proposed as a methyl radical pathway to form CH3OH and CH3OOH first, and then the generated CH3OH is further oxidized to HOCH2OOH and HCOOH.
Intermolecular Stereospecific Substitution of Underivatized Enantioenriched Secondary Alcohols by Organocatalysis
Authors: Akkarasamiyo Sunisa , Samec Joseph S. M. 
Published: November 2019

AbstractThe stereospecific substitution of non‐derivatized and non‐allylic enantioenriched alcohols with only water as a by‐product would enable the use of readily available alcohols as substrates for green and sustainable transformations. However, the poor leaving group ability of the OH group has hampered the development of such a process. Denton and co‐workers recently described the use of (2‐hydroxybenzyl)diphenylphosphine oxide as a catalyst of a redox‐neutral and zero‐waste‐generating Mitsunobu reaction. This innovative process constitutes the first intermolecular stereospecific substitution of non‐allylic alcohols, and might find industrial applications.
Gold(III) π‐Allyl Complexes
Authors: Rodriguez Jessica , Szalóki György , Sosa Carrizo E. Daiann , Saffon‐Merceron Nathalie , Miqueu Karinne , Bourissou Didier 
Published: November 2019

AbstractGold(III) π‐complexes have been authenticated recently with alkenes, alkynes, and arenes. The key importance of PdII π‐allyl complexes in organometallic chemistry (Tsuji–Trost reaction) prompted us to explore gold(III) π‐allyl complexes, which have remained elusive so far. The (P,C)AuIII(allyl) and (methallyl) complexes 3 and 3′ were readily prepared and isolated as thermally and air‐stable solids. Spectroscopic and crystallographic analyses combined with detailed DFT calculations support tight quasi‐symmetric η3‐coordination of the allyl moiety. The π‐allyl gold(III) complexes are activated towards nucleophilic additions, as substantiated with β‐diketo enolates.
A Supported Nickel Catalyst Stabilized by a Surface Digging Effect for Efficient Methane Oxidation
Authors: Zhou Huang , Liu Tianyang , Zhao Xuyan , Zhao Yafei , Lv Hongwei , Fang Shi , Wang Xiaoqian , Zhou Fangyao , Xu Qian , Xu Jie , Xiong Can , Xue Zhenggang , Wang Kai , Cheong Weng‐Chon , Xi Wei , Gu Lin , Yao Tao , Wei Shiqiang , Hong Xun , Luo Jun , Li Yafei , Wu Yuen 
Published: November 2019

AbstractA surface digging effect of supported Ni NPs on an amorphous N‐doped carbon is described, during which the surface‐loaded Ni NPs would etch and sink into the underneath carbon support to prevent sintering. This process is driven by the strong coordination interaction between the surface Ni atoms and N‐rich defects. In the aim of activation of C−H bonds for methane oxidation, those sinking Ni NPs could be further transformed into thermodynamically stable and active metal‐defect sites within the as‐generated surface holes by simply elevating the temperature. In situ transmission electron microscopy images reveal the sunk Ni NPs dig themselves adaptive surface holes, which would largely prevent the migration of Ni NPs without weakening their accessibility. The reported two‐step strategy opens up a new route to manufacture sintering‐resistant supported metal catalysts without degrading their catalytic efficiency.
Total Synthesis of (+)‐6‐epi‐Ophiobolin A
Authors: Thach Danny Q. , Brill Zachary G. , Grover Huck K. , Esguerra Kenneth V. , Thompson Jordan K. , Maimone Thomas J. 
Published: November 2019

AbstractThe ophiobolin sesterterpenes are notable plant pathogens which have recently elicited significant chemical and biological attention because of their intriguing carbogenic frameworks, reactive functionalities, and emerging anticancer profiles. Reported herein is a total synthesis of (+)‐6‐epi‐ophiobolin A in 14 steps, a task which addresses construction of the synthetically challenging spirocyclic tetrahydrofuran motif as well as several other key stereochemical problems. This work demonstrates a streamlined synthetic platform to complex ophiobolins leveraging disparate termination modes of a radical polycyclization cascade for divergent elaboration and functionalization.
Glycidols Esters, 2‐Chloropropane‐1,3‐Diols, and 3‐Chloropropane‐1,2‐Diols Contents in Real Olive Oil Samples and their Relation with Diacylglycerols
Authors: Cichelli Angelo , Riciputi Ylenia , Cerretani Lorenzo , Caboni Maria F. , d'Alessandro Nicola 
Published: November 2019

AbstractA series of authentic virgin, refined, and mixtures of olive oils was analyzed for their content of 2‐and 3‐chloropropanediol (MCPD) esters expressed as 2−/3‐MCPD, glycidol (and related glycidyl esters) (GE), and diglycerides (DAG). High concentrations of MCPD and GE were found, above all, in pomace oils, which come from the poorer starting raw materials, while virgin olive oils, as expected, do not contain any process contaminant. On the other hand, DAGs are present in all samples, demonstrating that their involvement in the formation of such contaminants only occurs when temperatures are higher than that used during the refining steps. The lack of correlation between the amounts of MCPD and GE can be ascribed to their completely different chemical stability as the epoxy ring of the GE opens easily, leading to both short‐chain derivatives and/or MCPD itself. This finding can also explain the data about the absence of 2‐MCPD in all the analyzed oil samples: other than the statistical probability and the steric effect of the SN2 formation mechanism, both in favor of the 3‐ derivative, we have also to consider the MCPD formation pathway involving glycidol that, under opportune conditions of refining, can increase the whole amount of 3‐MCPD (under thermodynamic control, 3‐MCPD is more stable).
Preparation of a NiAl‐Oxide@Polypyrrole Composite for High‐Performance Supercapacitors
Authors: Zou Kai‐Min , Zeng Hong‐Yan , Cao Xiao‐Ju , Yuan Jian , Claude Alain Gohi Bi Foua , Xu Sheng , Li Hao‐Bo , Long Yi‐Wen 
Published: November 2019

AbstractA core‐shell NiAlO@polypyrrole composite (NiAlO@PPy) with a 3D “sand rose”‐like morphology was prepared via a facile in situ oxidative polymerization of pyrrole monomer, where the role of PPy coating thickness was investigated for high‐performance supercapacitors. Microstructure analyses indicated that the PPy was successfully coated onto the NiAlO surface to form a core‐shell structure. The NiAlO@PPy exhibited a better electrochemical performance than pure NiAlO, and the moderate thickness of the PPy shell layer was beneficial for expediting the electron transfer in the redox reaction. It was found that the NiAlO@PPy5 prepared at 5.0 mL L−1 addition amount of pyrrole monomer demonstrated the best electrochemical performance with a high specific capacitance of 883.2 F g−1 at a current density of 1 A g−1 and excellent capacitance retention of 91.82 % of its initial capacitance after 1000 cycles at 3 A g−1. The outstanding electrochemical performance of NiAlO@PPy5 were due to the synergistic effect of NiAlO and PPy, where the uniform network‐like PPy shell with the optimal thickness made electrolyte ions more easily accessible for faradic reactions. This work provided a simple approach for designing organic–inorganic core‐shell materials as high‐performance electrode materials for electrochemical supercapacitors.
Ginkgolide B protects human pulmonary alveolar epithelial A549 cells from lipopolysaccharide‐induced inflammatory responses by reducing TRIM37‐mediated NF‐κB activation
Authors: Xiang Yijin , Zhang Shaoyan , Lu Jia , Zhang Wen , Cai Min , Xiang Jun , Cai Dingfang 
Published: November 2019

AbstractThe treatment options for acute stroke combined with pulmonary infection are limited. Clinically, there are several therapies to promote blood circulation and dissipate blood stasis; these treatment options include ginkgolide B (GB), which has PAF (platelet activating factor)‐inhibiting effects. PAF‐receptor (PAF‐R) antagonists are used to treat a variety of inflammatory diseases; however, the potential of PAF‐R antagonists as a treatment for lung infections remains unclear. The aim of the present study is to investigate the protective effect of GB on lipopolysaccharide‐induced inflammatory responses in A549 human pulmonary alveolar epithelial cells (HPAEpiC) in vitro. Cell viability and apoptosis were measured by CCK‐8 and flow cytometry. TRIM37, Caspase‐3, and NF‐κBp65 expression levels were measured by real‐time PCR and Western blotting. The release of tumor necrosis factor‐α and interleukin‐1β was measured by ELISA. The data indicates that GB may reduce TRIM37 expression by antagonizing the PAF‐R pathway, thereby inhibiting the activation of nuclear factor‐κB and alleviating the inflammatory response of alveolar epithelial cells. This study is the first to provide insight into the therapeutic potential of GB and suggests that clinical application of GB in acute stroke combined with pulmonary inflammation may be efficacious.
Adult and infant pharmacokinetic profiling of dihydrocodeine using physiologically based pharmacokinetic modeling
Authors: Ota Miki , Shimizu Makiko , Kamiya Yusuke , Emoto Chie , Fukuda Tsuyoshi , Yamazaki Hiroshi 
Published: November 2019

AbstractWe previously analysed the serum concentrations of dihydrocodeine in a 1‐month‐old infant with respiratory depression after being prescribed dihydrocodeine phosphate 2.0 mg/day divided t.i.d. for 2 days. The purpose was to develop a full physiologically based pharmacokinetic (PBPK) model that could account for these and other drug monitoring results. Based on experiments in Caco‐2 cell monolayers, the effective permeability of dihydrocodeine in human jejunum was established as 1.28 × 10−4 cm/s. The in vitro Vmax/Km values for dihydrocodeine demethylation mediated by recombinant cytochrome P450 2D6 and 3A4 were 0.19 and 0.066 μl/min/pmol, respectively, and for dihydrocodeine 6‐O‐glucuronidation mediated by recombinant UGT2B4 and 2B7, the Vmax/Km values were 0.14 and 0.22 μl/min/mg protein, respectively. Renal clearance was calculated as 5.37 L/h on the total clearance value multiplied by the fraction recovered in urine. The reported plasma concentration–time profiles of dihydrocodeine after intravenous administration in healthy volunteers were used to adjust the tissue partitioning ratios. The developed model simulated the pharmacokinetic profiles of dihydrocodeine after single and multiple oral administrations reasonably well in the same population. Subsequently, the validated model was used to simulate pharmacokinetic profiles for five pediatric cases, including the 1‐month‐old Japanese boy and a 14‐year‐old Japanese girl who took an overdose of dihydrocodeine phosphate (37 mg). The simulated pharmacokinetic profiles for five virtual pediatric subjects matching the age, gender, and P450 2D6 phenotype of each case approximately reflected the observed values. These results suggested that our dihydrocodeine PBPK model reproduced the results of clinical cases reasonably well for subjects.
Can Designer Indels Be Tailored by Gene Editing?
Authors: Trimidal Sara G. , Benjamin Ronald , Bae Ji Eun , Han Mira V. , Kong Elizabeth , Singer Aaron , Williams Tyler S. , Yang Bing , Schiller Martin R. 
Published: November 2019

AbstractGenome editing with engineered nucleases (GEENs) introduce site‐specific DNA double‐strand breaks (DSBs) and repairs DSBs via nonhomologous end‐joining (NHEJ) pathways that eventually create indels (insertions/deletions) in a genome. Whether the features of indels resulting from gene editing could be customized is asked. A review of the literature reveals how gene editing technologies via NHEJ pathways impact gene editing. The survey consolidates a body of literature that suggests that the type (insertion, deletion, and complex) and the approximate length of indel edits can be somewhat customized with different GEENs and by manipulating the expression of key NHEJ genes. Structural data suggest that binding of GEENs to DNA may interfere with binding of key components of DNA repair complexes, favoring either classical‐ or alternative‐NHEJ. The hypotheses have some limitations, but if validated, will enable scientists to better control indel makeup, holding promise for basic science and clinical applications of gene editing. Also see the video abstract here https://youtu.be/vTkJtUsLi3w
Three‐dimensional biomimetic hyaluronic acid hydrogels to investigate glioblastoma stem cell behaviors
Authors: Nakod Pinaki S. , Kim Yonghyun , Rao Shreyas S. 
Published: November 2019

ABSTRACTGlioblastoma multiforme (GBM) is the deadliest form of primary brain tumor. GBM tumors are highly heterogeneous, being composed of tumor cells as well as glioblastoma stem cells (GSCs) that contribute to drug resistance and tumor recurrence following treatment. To develop therapeutic strategies, an improved understanding of GSC behavior in their microenvironment is critical. Herein, we have employed three‐dimensional (3D) hyaluronic acid (HA) hydrogels that allow the incorporation of brain microenvironmental cues to investigate GSC behavior. U87 cell line and patient‐derived D456 cells were cultured as suspension cultures (serum‐free) and adherently (in the presence of serum) and were then encapsulated in HA hydrogels. We observed that all the seeded single cells expanded and formed spheres, and the size of the spheres increased with time. Increasing the initial cell seeding density of cells influenced the sphere size distribution. Interestingly, clonal expansion of serum‐free grown tumor cells in HA hydrogels was observed. Also, stemness marker expression of serum and/or serum‐free grown cells was altered when cultured in HA hydrogels. Finally, we demonstrated that HA hydrogels can support long‐term GSC culture (up to 60 days) with retention of stemness markers. Overall, such biomimetic culture systems could further our understanding of the microenvironmental regulation of GSC phenotypes.