bluuebunny/crossref_metadata_2025_split huggingface dataset:
Homozygous splicing mutation in NUP133 causes Galloway–Mowat syndrome
Authors: Fujita Atsushi , Tsukaguchi Hiroyasu , Koshimizu Eriko , Nakazato Hitoshi , Itoh Kyoko , Kuraoka Shohei , Komohara Yoshihiro , Shiina Masaaki , Nakamura Shohei , Kitajima Mika , Tsurusaki Yoshinori , Miyatake Satoko , Ogata Kazuhiro , Iijima Kazumoto , Matsumoto Naomichi , Miyake Noriko 
Published: November 2018

ObjectiveGalloway–Mowat syndrome (GAMOS) is a neural and renal disorder, characterized by microcephaly, brain anomalies, and early onset nephrotic syndrome. Biallelic mutations in WDR73 and the 4 subunit genes of the KEOPS complex are reported to cause GAMOS. Furthermore, an identical homozygous NUP107 (nucleoporin 107kDa) mutation was identified in 4 GAMOS‐like families, although biallelic NUP107 mutations were originally identified in steroid‐resistant nephrotic syndrome. NUP107 and NUP133 (nucleoporin 133kDa) are interacting subunits of the nuclear pore complex in the nuclear envelope during interphase, and these proteins are also involved in centrosome positioning and spindle assembly during mitosis.MethodsLinkage analysis and whole exome sequencing were performed in a previously reported GAMOS family with brain atrophy and steroid‐resistant nephrotic syndrome.ResultsWe identified a homozygous NUP133 mutation, c.3335‐11T>A, which results in the insertion of 9bp of intronic sequence between exons 25 and 26 in the mutant transcript. NUP133 and NUP107 interaction was impaired by the NUP133 mutation based on an immunoprecipitation assay. Importantly, focal cortical dysplasia type IIa was recognized in the brain of an autopsied patient and focal segmental glomerulosclerosis was confirmed in the kidneys of the 3 examined patients. A nup133‐knockdown zebrafish model exhibited microcephaly, fewer neuronal cells, underdeveloped glomeruli, and fusion of the foot processes of the podocytes, which mimicked human GAMOS features. nup133 morphants could be rescued by human wild‐type NUP133 mRNA but not by mutant mRNA.InterpretationThese data indicate that the biallelic NUP133 loss‐of‐function mutation causes GAMOS. Ann Neurol 2018;84:814–828
Helical Nanostructures: Chirality Transfer and a Photodriven Transformation from Superhelix to Nanokebab
Authors: Jiang Hejin , Jiang Yuqian , Han Jianlei , Zhang Li , Liu Minghua 
Published: November 2018

AbstractPhotosensitive cinnamic acid conjugated glutamides were designed to demonstrate photocontrolled hierarchical chirality transfer and switching in self‐assembled systems. In methanol, the cinnamic acid derivatives self‐assembled into superhelices, which could be switched into nanokebabs upon UV irradiation. These two nanostructures showed opposite helicity. The chiral nanostructures could further convey their chirality to achiral fluorescent molecules and result in the emission of circularly polarized luminescence (CPL). Remarkably, the CPL followed the helicity of the chiral nanostructure rather than the inherent molecular chirality. Photodriven dimerization of the cinnamic moiety lead to a significant change in molecular packing and subsequent switching of the helicity of the formed nanostructures.
Cyaarside (CAs−) and 1,3‐Diarsaallendiide (AsCAs2−) Ligands Coordinated to Uranium and Generated via Activation of the Arsaethynolate Ligand (OCAs−)
Authors: Hoerger Christopher J. , Heinemann Frank W. , Louyriac Elisa , Rigo Massimo , Maron Laurent , Grützmacher Hansjörg , Driess Matthias , Meyer Karsten 
Published: November 2018

AbstractReaction of the trivalent uranium complex [((Ad,MeArO)3N)U(DME)] with one molar equiv [Na(OCAs)(dioxane)3], in the presence of 2.2.2‐crypt, yields [Na(2.2.2‐crypt)][{((Ad,MeArO)3N)UIV(THF)}(μ‐O){((Ad,MeArO)3N)UIV(CAs)}] (1), the first example of a coordinated η1‐cyaarside ligand (CAs−). Formation of the terminal CAs− is promoted by the highly reducing, oxophilic UIII precursor [((Ad,MeArO)3N)U(DME)] and proceeds through reductive C−O bond cleavage of the bound arsaethynolate anion, OCAs−. If two equiv of OCAs− react with the UIII precursor, the binuclear, μ‐oxo‐bridged U2IV/IV complex [Na(2.2.2‐crypt)]2[{((Ad,MeArO)3N)UIV}2(μ‐O)(μ‐AsCAs)] (2), comprising the hitherto unknown μ:η1,η1‐coordinated (AsCAs)2− ligand, is isolated. The mechanistic pathway to 2 involves the decarbonylation of a dimeric intermediate formed in the reaction of 1 with OCAs−. An alternative pathway to complex 2 is by conversion of 1 via addition of one further equiv of OCAs−.
Helikale Multi‐Koordinations‐Anionenbindungskatalysatoren ermöglichen hoch enantioselektive Dearomatisierung von Pyryliumderivaten
Authors: Fischer Theresa , Bamberger Julia , Gómez‐Martínez Melania , Piekarski Dariusz G. , García Mancheño Olga 
Published: November 2018

AbstractEine allgemeine und hoch enantioselektive Synthese von Sauerstoffheterocyclen aus in situ leicht zugänglichen Pyryliumderivaten wurde durch einen multi‐koordinierenden Triazol‐basierten Anionenbindungskatalyse‐Ansatz realisiert. Die hohe Aktivität der helikalen Tetrakistriazolkatalysatoren (TetraTri) ermöglicht durch diverse Anionenbindungstaschen bemerkenswert niedrige Katalysatorbeladungen (bis zu 0.05 Mol%) und bietet einen einfachen Zugang zu chiralen Chromanonen und Dihydropyronen mit hohen Enantioselektivitäten (bis zu 98:2 e.r.). Darüber hinaus liefern experimentelle und theoretische Studien neue Einblicke in die H‐Donor‐Fähigkeit und wichtige Bindungswechselwirkungen der TetraTri‐Katalysatoren und ihrer Wirt‐Gast‐Komplexe, die auf die Bildung einer 1:3‐Spezies schließen lassen.
A Soybean Oil‐Based Adhesive and Its Application for Birdseed Binding
Authors: Fei Tao , Slagle Melissa Anne , Jarboe Darren H. , Wang Tong 
Published: November 2018

AbstractCurrent birdseed binders are mainly based on animal protein and fat. However, exporting the seed cakes containing animal products to European countries can be difficult due to their import policies. A plant oil‐based adhesive may be capable of binding loose seeds together, enabling it to be used as an alternative to the animal protein‐based and fat‐based binders used currently and making the exportation possible. A soybean oil‐based adhesive was, therefore, synthesized and tested for birdseed binding in this study. The effects of the quantity of saturated fatty acylglycerols (i.e., fully hydrogenated soybean oil, FHSO) introduced into epoxidized soybean oil (ESO) and a phosphoric acid (H3PO4) cross‐linker on product characteristics were investigated. Increased quantity of FHSO improved the glass transition point (Tg), hardness, and adhesion while further increasing the quantity led to various degrees of phase separation of the product. Increased quantity of H3PO4also improved hardness and adhesion of the binder. A binder‐MDAG (a mixture of mono and diacylglycerols at about a 1:1 ratio as a hardening agent) mixture (BMD) having an average hardness and adhesion of 4024 and 1197 g, respectively, was selected for seed binding. Seed cakes bound with 15 wt.% BMD were about twice as hard as gelatin‐bound ones. Storage of the BMD itself in open air led to increased hardness, adhesion, and melting point, and storage of the seed cakes bound by BMD in open air led to increased hardness and high temperature tolerance. Seed cakes bound with BMD also presented much better water resistance.
Use of Aggregation‐Induced Emission for Selective Detection of Phase Transformation during Evaporative Crystallization of Hexaphenylsilole
Authors: Ito Fuyuki , Oka Narumi 
Published: November 2018

AbstractCrystallization of organic molecules is quite complicated because the crystallization process is governed by weak intermolecular interactions. By exploiting aggregation‐induced emission (AIE), we attempted to realize the selective detection of phase transformation during the evaporative crystallization of hexaphenylsilole (HPS), which shows different fluorescent colors in the amorphous and crystalline phases. No fluorescence emission was observed in the HPS solution immediately after dropping on the glass substrate due to the non‐radiative deactivation induced by intramolecular rotational or vibrational motion, suggesting that HPS exists as a monomer in solution. As time elapsed after dropping, green emission first appeared, which changed to blue after solvent evaporation, because of phase transformation from the amorphous state to the crystalline state. This phenomenon supports not only the two‐step nucleation model involving an intermediate such as a liquid‐like cluster prior to nucleation but also the real‐time detection of Ostwald's rule of stages during evaporative crystallization.
The maximum surplus in a finite‐time interval for a discrete‐time risk model with exchangeable, dependent claim occurrences
Authors: Gebizlioglu Omer L. , Eryilmaz Serkan 
Published: November 2018

AbstractThis paper investigates a discrete‐time risk model that involves exchangeable dependent loss generating claim occurrences and compound binomially distributed aggregate loss amounts. First, a general framework is presented to derive the distribution of a surplus sequence using the model. This framework is then applied to obtain the distribution of any function of a surplus sequence in a finite‐time interval. Specifically, the distribution of the maximum surplus is obtained under nonruin conditions. Based on this distribution, the computation of the minimum surplus distribution is given. Asset and risk management–oriented implications are discussed for the obtained distributions based on numerical evaluations. In addition, comparisons are made involving the corresponding results of the classical discrete‐time compound binomial risk model, for which claim occurrences are independent and identically distributed.
Strategy and Effects of Polyproline Peptide Stapling by Copper(I)‐Catalyzed Alkyne–Azide Cycloaddition Reaction
Authors: Tseng Wen‐Hsiu , Li Meng‐Che , Horng Jia‐Cherng , Wang Sheng‐Kai 
Published: November 2018

AbstractPolyproline is a unique type of peptide that has a stable, robust, and well‐defined helical structure in an aqueous environment. These features have allowed polyproline to be used as a nanosized scaffold for applications in chemical biology and related fields. To understand its structural properties and to expand the applications, this secondary structure was tested systematically by stapling the peptide at different locations with staples of various lengths. Using the efficient copper(I)‐catalyzed alkyne–azide cycloaddition (CuAAC), we successfully prepared stapled polyproline and investigated the impact of this peptide macrocyclization through circular dichroism analysis. Whereas the stapling seems to have no significant effect on polyproline helix II (PPII) conformation in water, the location and the length of the staple affect the transformation of conformation in n‐propanol. These results provide valuable information for future research using peptide stapling to manipulate polyproline conformation for various applications.
Study of an Annular Photoreactor with Tangential Inlet and Outlet. II. The UV/H2O2 Reactive Flow
Authors: Peres José Carlos G. , Tambani Pamela C. , Teixeira Antonio Carlos S. C. , Guardani Roberto , Vianna Ardson dos S. 
Published: November 2018

AbstractThe concentration profiles of species involved in the degradation of phenol by an advanced oxidation process (AOP) are modeled by a computational fluid dynamics tool in an annular reactor whose fluid dynamics was the object of a previous study. The reactive flow was fully described together with the radiation field and the kinetic model, which encompasses large kinetic constants such as 1010 L mol−1s−1. Phenol degradation can be simulated by using relaxation factors of at least 1012 kg m−3s−1. The hydroxyl radical concentration profile depends on the radiation field, assessed by the discrete ordinate and the discrete transfer methods. Phenol can be completely degraded along the reactor. A centrifugal effect was observed, with higher concentrations of degradation products along the inner wall at the reactor outlet.
Mesoporous PtCu Alloy Nanoparticles with Tunable Compositions and Particles Sizes Using Diblock Copolymer Micelle Templates
Authors: Kang Yunqing , Jiang Bo , Alothman Zeid A. , Badjah Ahmad Yacine , Naushad Mu , Habila Mohamed , Wabaidur Saikh , Henzie Joel , Li Hexing , Yamauchi Yusuke 
Published: November 2018

AbstractA simple, scalable route for the generation of mesoporous Rh particles by chemical reduction on self‐assembled block‐copolymer micelle templates was reported recently (Nat. Commun. 2017, 8, 15581). Here, this concept is extended to generate mesoporous PtCu alloy nanoparticles through the same approach. The PtCu alloy particles possess high‐surface‐area nanoporous architectures and good chemical stability for applications in catalysis. Both the composition and diameter of the bimetallic PtCu nanoparticles can be controlled by adjusting the amount of precursor in the reaction, which affects the electrochemical properties of the material. The combination of the mesoporous structure with the synergistic bimetallic electronic effects of PtCu gives rise to enhanced activity for the catalytic oxidation of methanol compared with commercial Pt black.
Selective Array‐Based Sensing of Anabolic Steroids in Aqueous Solution by Host–Guest Reporter Complexes
Authors: Gill Adam D. , Perez Lizeth , Salinas Isaac N. Q. , Byers Samantha R. , Liu Yang , Hickey Briana L. , Zhong Wenwan , Hooley Richard J. 
Published: November 2018

AbstractArrayed complexes of a water‐soluble deep cavitand and two fluorescent indicators show selective sensing of anabolic‐androgenic steroids in aqueous environments. By combining the host–guest complexes with small amounts of heavy metal ions, discrimination between steroids that vary in structure by only a single π bond is possible. The sensing occurs through a triggered aggregation mechanism, which can be mediated by both the presence of metal ions and the steroids. The use of both “turn‐on” and “turn‐off” fluorophores is essential for good discrimination. As low as 10 μm steroid can be detected, and the discrimination is selective in steroid samples spiked into human urine.
Dynamic Molecular Metamorphism Involving Palladium‐Assisted Dimerization of π‐Cation Radicals
Authors: Kahlfuss Christophe , Grüber Raymond , Dumont Elise , Royal Guy , Chevallier Floris , Cobo Saioa , Saint‐Aman Eric , Bucher Christophe 
Published: November 2018

AbstractA dynamic supramolecular approach is developed to promote the π‐dimerization of viologen radicals at room temperature and in standard concentration ranges. The approach involves cis‐ or trans‐protected palladium centers serving as inorganic hinges linking two functionalized viologens endowed with metal‐ion coordinating properties. Based on detailed spectroscopic, electrochemical and computational data, we show that the one‐electron electrochemical reduction of the viologen units in different dynamic metal/ligand mixtures leads to the formation of the same intramolecular π‐dimer, regardless of the initial environment around the metallic precursor and of the relative ratio between metal and ligand initially introduced in solution. The large‐scale electron‐triggered reorganization of the building blocks introduced in solution thus involves drastic changes in the stoichiometry and stereochemistry of the palladium/viologen complexes proceeding in some cases through a palladium centered trans→cis isomerization of the coordinated ligands.
Function‐Oriented: The Construction of Lanthanide MOF Luminescent Sensors Containing Dual‐Function Urea Hydrogen‐Bond Sites for Efficient Detection of Picric Acid
Authors: Liu Wei , Huang Xin , Chen Chunyang , Xu Cong , Ma Jingxin , Yang Lizi , Wang Wenjie , Dou Wei , Liu Weisheng 
Published: November 2018

AbstractTwo novel lanthanide metal–organic framework (Ln‐MOF) luminescent sensors for the detection of picric acid have been successfully assembled. Following a function‐oriented strategy, urea hydrogen‐bonding functional sites were introduced into two MOF frameworks. A structural analysis indicated that the two MOFs have the exact same structure, namely 2D layers with diamond‐shaped holes that are accumulated into a 3D framework through the hydrogen‐bonding interactions between urea and carboxylate groups. Interestingly, only half of the urea units are involved in supporting the MOF framework through N−H⋅⋅⋅O hydrogen‐bonding interactions, whereas the other half are located in the pore channel and act as empty recognition sites. Abundant N−H urea bonds are present in the inner walls of three types of interpenetrating 1D channels. Luminescence studies revealed that the two Ln‐MOFs exhibit high sensitivity, good selectivity, and a fast luminescence quenching response towards picric acid. In particular, the two Ln‐MOFs can be simply and quickly regenerated, and exhibit excellent recyclability. In summary, we have successfully used a function‐oriented strategy to achieve multiple functions in a ligand to construct lanthanide MOF luminescent sensors for the detection of picric acid, thereby providing a potential strategy for the future development of MOF luminescent sensors with a specific target.
Pharmacokinetics of Single and Repeat Doses of Fluticasone Furoate/Umeclidinium/Vilanterol in Healthy Chinese Adults
Authors: Li Yan , Li Huafang , Sheng Yucheng , Du Xin , Yao Yuhui , Luo Xian , Ma Peiming 
Published: November 2018

AbstractThe pharmacokinetics (PK) and safety of single‐inhaler fluticasone furoate/umeclidinium/vilanterol (FF/UMEC/VI) after single and repeat dosing in healthy Chinese adults were assessed. In this open‐label study (NCT02837380), subjects received once‐daily FF/UMEC/VI 100/62.5/25 µg on day 1 and repeat doses on days 2–7. PK parameters (days 1 and 7) included maximum observed concentration (Cmax) and area under the plasma concentration‐time curve (AUC) from time zero (predose) to last time of quantifiable concentration (AUC0–t). Terminal phase half‐life (t½) on day 1 was estimated. The primary objective was to assess systemic exposure of FF 100 µg, UMEC 62.5 µg, and VI 25 µg following single‐inhaler triple therapy on days 1 and 7. On day 1, geometric mean t½ of UMEC and VI was 0.36 and 0.52 hours, respectively; t½ of FF was not representative because of nonquantifiable concentration data. On days 1 and 7, geometric mean Cmax of FF was 10.46 and 27.32 pg/mL, respectively; Cmax of UMEC was 144.14 and 241.35 pg/mL, respectively; and Cmax of VI was 120.42 and 196.78 pg/mL, respectively. AUC0–t of FF was 1.77 and 276.96 pg·h/mL, respectively; AUC0–t of UMEC was 28.44 and 117.19 pg·h/mL, respectively; and AUC0–t of VI, 42.46 and 101.12 pg·h/mL, respectively. The PK of FF/UMEC/VI was as expected for the individual‐component PK previously reported in healthy Chinese adults. No new safety signals were observed.
A certificateless signcryption scheme for smart home networks
Authors: Ji Sai , Huang Rui , Shen Jian , Jin Xin , Cho Youngju 
Published: November 2018

SummarySmart home networks (SHNets) are based on the Internet of Things (IoT) and provide users with a highly intelligent home life. In recent years, SHNets have become more and more popular. In SHNets, a large amount of data interactions are needed. These data related to the privacy of users may be subject to attacks such as eavesdropping attacks, replay attacks, and mobile phone stolen attacks. Privacy protection and communication security in smart home networks have been widely studied by many scholars. However, most of the existing user authentication in current researches focus on identity‐based authentication, where the encryption protocols are divided into two steps: the signing step and the encryption step. In this paper, a certificateless signcryption scheme for SHNets is proposed which can provide biometric‐based user identity authentication. The security and performance analyses show that our scheme is efficient and secure.
Urban Particulate Matter‐Induced Decomposition of S‐Nitrosoglutathione Relevant to Aberrant Nitric Oxide Biological Signaling
Authors: Oszajca Maria , Wądołek Anna , Hooper James , Brindell Małgorzata , van Eldik Rudi , Stochel Grażyna 
Published: November 2018

AbstractExposure to airborne particulate matter (PM) is associated with hazardous effects on human health. Soluble constituents of PM may be released in biological fluids and disturb the precisely tuned nitric oxide signaling processes. The influence of aqueous extracts from two types of airborne urban PM (SRM 1648a, a commercially available sample, and KR PM2.5, a sample collected “in‐house” in Krakow, Poland) on the stability of S‐nitrosoglutathione (GSNO) was investigated. The particle interfaces had no direct effect on the studied reaction, but extracts obtained from both samples facilitated NO release from GSNO. The effectiveness of NO release was significantly affected by glutathione (GSH) and ascorbic acid (AscA). Examination of the combined influence of Cu2+, Fe3+, and reductants on GSNO stability revealed copper to be the main GSNO decomposing species. Computational models of nitrosothiols interacting with metal oxide substrates and solvated metal ions support these claims. The study stresses the importance of the interplay between metal ions and biological reductants in S‐nitrosothiols decomposition.
Redox‐Mediator‐Enhanced Electrochemical Capacitors: Recent Advances and Future Perspectives
Authors: Hu Lintong , Zhai Tianyou , Li Huiqiao , Wang Yonggang 
Published: November 2018

AbstractSupercapacitors deliver exceptional power densities, high cycling stability, and inherent safety but suffer from low energy densities. Many methods to enhance the energy density are based on exploring electrode materials with well‐developed structures and designing asymmetric systems with wide voltage windows. The energy density is substantially enhanced at the compromise of power density by utilizing the sluggish kinetics of pseudocapacitive materials. Redox‐active electrolytes can contribute additional pseudocapacitance from the reactions of redox mediators at the interface, which have attracted increasing attention of researchers. Redox‐mediator‐enhanced supercapacitors deliver high energy densities while retaining high power densities. This Minireview highlights the recently prominent progresses of single‐, dual‐, and ambipolar‐redox‐mediator‐enhanced supercapacitors, the challenges they face, and approaches to suppress self‐discharge and develop high‐concentration redox‐active electrolytes for performance promotion.