bluuebunny/crossref_metadata_2025_split huggingface dataset:
Musculoskeletal Disease in MDA5‐Related Type I Interferonopathy: A Mendelian Mimic of Jaccoud's Arthropathy
Authors: de Carvalho Luciana Martins , Ngoumou Gonza , Park Ji Woo , Ehmke Nadja , Deigendesch Nikolaus , Kitabayashi Naoki , Melki Isabelle , Souza Flávio Falcäo L. , Tzschach Andreas , Nogueira‐Barbosa Marcello H. , Ferriani Virgínia , Louzada‐Junior Paulo , Marques Wilson , Lourenço Charles M. , Horn Denise , Kallinich Tilmann , Stenzel Werner , Hur Sun , Rice Gillian I. , Crow Yanick J. 
Published: June 2017

ObjectiveTo define the molecular basis of a multisystem phenotype with progressive musculoskeletal disease of the hands and feet, including camptodactyly, subluxation, and tendon rupture, reminiscent of Jaccoud's arthropathy.MethodsWe identified 2 families segregating an autosomal‐dominant phenotype encompassing musculoskeletal disease and variable additional features, including psoriasis, dental abnormalities, cardiac valve involvement, glaucoma, and basal ganglia calcification. We measured the expression of interferon (IFN)–stimulated genes in the peripheral blood and skin, and undertook targeted Sanger sequencing of the IFIH1 gene encoding the cytosolic double‐stranded RNA (dsRNA) sensor melanoma differentiation–associated protein 5 (MDA‐5). We also assessed the functional consequences of IFIH1 gene variants using an in vitro IFNβ reporter assay in HEK 293T cells.ResultsWe recorded an up‐regulation of type I IFN–induced gene transcripts in all 5 patients tested and identified a heterozygous gain‐of‐function mutation in IFIH1 in each family, resulting in different substitutions of the threonine residue at position 331 of MDA‐5. Both of these variants were associated with increased IFNβ expression in the absence of exogenous dsRNA ligand, consistent with constitutive activation of MDA‐5.ConclusionThese cases highlight the significant musculoskeletal involvement that can be associated with mutations in MDA‐5, and emphasize the value of testing for up‐regulation of IFN signaling as a marker of the underlying molecular lesion. Our data indicate that both Singleton‐Merten syndrome and neuroinflammation described in the context of MDA‐5 gain‐of‐function constitute part of the same type I interferonopathy disease spectrum, and provide possible novel insight into the pathology of Jaccoud's arthropathy.
Systematically Studying the Effect of Fluoride on the Properties of Cyclophanes Bearing Naphthalene Diimide and Dialkoxyaryl Groups
Authors: Young Nicholas A. , Drew Simon C. , Maniam Subashani , Langford Steven J. 
Published: June 2017

AbstractAnion–π interactions between the Lewis basic anion fluoride and π‐acidic naphthalene diimide was systematically studied in a series of cyclophanes in which the properties are modulated through the influence of a second, electron‐rich aromatic unit. The systems and subsequently generated radical anions, upon addition of fluoride, were studied by absorption spectroscopic and EPR techniques. The results infer a modulation as a result of the nature and strength of the π–π interaction in the macrocyclic structure.
Application of a developed distributed hydrological model based on the mixed runoff generation model and 2D kinematic wave flow routing model for better flood forecasting
Authors: Bao Hongjun , Wang Lili , Zhang Ke , Li Zhijia 
Published: June 2017

AbstractDue to the specific characteristics of semi‐arid regions, this paper aims to establish a Grid‐and‐Mixed‐runoff‐generation‐and‐two‐dimensional‐Kinematic‐wave‐based distributed hydrological model (GMKHM‐2D model) coupling a mixed runoff generation model and 2D overland flow routing model based on kinematic wave theory for flood simulation and forecasting with digital drainage networks. Taking into consideration the complex runoff generation in semi‐arid regions, a mixed runoff generation model combining saturation‐excess mechanism and infiltration‐excess mechanism is developed for grid‐based runoff generation and 2D implicit finite difference kinematic wave flow model is introduced for routing to solve depressions water storing for grid‐based overland flow routing in the GMKHM‐2D model. The GMKHM‐2D model, the GMKHM‐1D model with coupling the mixed runoff generation model and one‐dimension kinematic wave flow model, the Shanbei model and the Xin'anjiang model were employed to the upper Kongjiapo basin in Qin River, a tributary of the Yellow River, with an area of 1454 km2 for flood simulation. Results show that two grid‐based distributed hydrological models and the Shanbei model perform better in flood simulation and can be used for flood forecasting in semi‐arid basins. Comparing with GMKHM‐1D, the flood peak simulation accuracy of the GMKHM‐2D model is higher.
Li/MgO Catalysts Doped with Alio‐valent Ions. Part II: Local Topology Unraveled by EPR/NMR and DFT Modeling
Authors: Simon Ulla , Alarcón Villaseca Sebastián , Shang Honghui , Levchenko Sergey V. , Arndt Sebastian , Epping Jan D. , Görke Oliver , Scheffler Matthias , Schomäcker Reinhard , van Tol Johan , Ozarowski Andrew , Dinse Klaus‐Peter 
Published: June 2017

AbstractThe role of Li in Li/MgO as a catalyst for oxidative coupling of methane (OCM) is to promote MgO surface morphology change rather than serve as a constituent of catalytically active sites. While Li/MgO is unstable at realistic conditions with respect to loss of Li, the resulting samples show enhanced selectivity towards C2 hydrocarbons versus CO2, although activity is low and close to pristine MgO. The way (co‐)doping with alio‐valent metal ions affects the catalytic performance of Li/MgO has now been explored. To analyze the structure and the stability of the samples, catalysts with well‐defined stoichiometry were prepared using a co‐precipitation method with freeze‐drying and subsequent annealing. Gd and Fe were used as dopants. Apart from their potential direct role in catalysis, these dopants are anticipated to stabilize Li in the catalyst under the reaction conditions, allowing further clarification of the role of Li. In the case of Gd/Li co‐doping, changes observed in EPR and 7Li‐NMR spectra indicate the formation of correlated, next‐neighbor Li−Mg⋅⋅⋅Gd+Mg pairs co‐existing with “isolated” Gd3+ ions at octahedral Mg lattice sites. For Li/Fe co‐doping, no significant change in the EPR pattern is observed in the presence of Li+ ions, indicating a larger distance between the Li+ and Fe3+ cations in the MgO lattice. Hybrid DFT calculations explain the difference between Fe and Gd co‐doping by a less efficient screening of the Coulomb repulsion between Gd3+ and neighboring cations in Gd doped samples, leading to the stabilization of LiMg near GdMg.
Platinum Supported on a Hybrid of Nickel and Tungsten Carbide on New‐Diamond Electrocatalyst for Methanol Oxidation and Oxygen Reduction Reactions
Authors: Wang Yanhui , Su Jing , Dong Liang , Zhao Pengjuan , Zhang Yan , Wang Weiping , Jia Shaopei , Zang Jianbing 
Published: June 2017

AbstractA hybrid of nickel and tungsten carbide on new‐diamond (Ni‐WC/nD) was synthesized by a microwave‐assisted method, in which nickel(tungsten) hydroxide was deposited on carbon black and then reduced carbothermally under N2 atmosphere. Pt nanoparticles (NPs) were deposited on the hybrid to form the Pt/Ni‐WC/nD electrocatalyst. XRD and TEM were used to investigate the structure and morphology of Pt/Ni‐WC/nD, respectively. The results revealed that Ni and WC particles formed on carbon black during the reduction of nickel(tungsten) hydroxide at 1300 °C under N2, and the amorphous carbon was translated to new‐diamond as a result of the catalysis of Ni. The Pt NPs deposited on Ni‐WC/nD exhibited a good affinity with the support. We demonstrated that Pt/Ni‐WC/nD was an active electrocatalyst for the methanol oxidation reaction (MOR) and the oxygen reduction reaction (ORR). Pt/Ni‐WC/nD exhibited a better electrocatalytic activity and durability than Pt/C electrocatalyst for the MOR and ORR. This can be attributed to the strong interaction between the Ni‐WC/nD support and Pt and the excellent stability of the new‐diamond.
Syndiotactic Poly(aminostyrene)‐Supported Palladium Catalyst for Ketone Methylation with Methanol
Authors: Jiang Lei , Guo Fang , Shi Zhenghai , Li Yang , Hou Zhaomin 
Published: June 2017

AbstractPalladium nanoparticles immobilized on an amino‐functionalized syndiotactic polystyrene (sPS‐N) served as a novel recyclable catalyst for the dimethylation and cross methyl alkylation of a wide range of ketones with methanol as the methylation agent. This heterogeneous catalyst (Pd@sPS‐N) was highly robust and showed excellent thermal stability and chemical resistance. It not only showed remarkably high activity, but it could also be easily recovered by filtration without loss of activity.
On the Feasibility of Sodium Metal as Pseudo‐Reference Electrode in Solid State Electrochemical Cells
Authors: Boschin Andrea , Abdelhamid Muhammad E. , Johansson Patrik 
Published: June 2017

AbstractA set‐up of a sodium metal anode vs. a solid polymer electrolyte (SPE) comprising poly(ethylene oxide) (PEO) and sodium bis(trifluoromethanesulfonyl)imide (NaTFSI) has been evaluated in detail for the feasibility to use sodium metal as a pseudo‐reference electrode (pseudo‐RE). To evaluate the stability and reproducibility, we monitored the half‐wave potential (E1/2) of added decamethylferrocene (Me10Fc) and the stability of the interface by electrochemical impedance spectroscopy (EIS). The sodium/SPE interface resistance (RNa/SPE) increases with time, up to 2.8 kΩ cm−2, and causes the E1/2 of the Me10Fc+/0 reference redox couple to drift up to 15 mV during 88 hours. Moreover, the sodium potential is very irreproducible, even initially after cell assembling the values can differ by 60 mV, likely due to extreme sensitivity of the metal surface even to an “inert and dry” glove box environment. Indeed, freshly cut sodium readily reacts with water, forming NaOH, and adsorbs impurities that can be present even in a glove box atmosphere. The oxidation layer and the amount of adsorbed impurities increase with the exposure to the glove box atmosphere, as revealed by ATR‐FTIR spectroscopy. Altogether, this calls for attention when evaluating any battery materials in half‐cell configurations using sodium metal as the pseudo‐RE.
Reactivity of NHC Alane Adducts towards N‐Heterocyclic Carbenes and Cyclic (Alkyl)(amino)carbenes: Ring Expansion, Ring Opening, and Al−H Bond Activation
Authors: Schneider Heidi , Hock Andreas , Bertermann Rüdiger , Radius Udo 
Published: June 2017

AbstractThe synthesis of mono‐NHC alane adducts of the type (NHC)⋅AlH3 (NHC=Me2Im (1), Me2ImMe (2), iPr2Im (3 and [D3]‐3), iPr2ImMe (4), Dipp2Im (10); Im=imidazolin‐2‐ylidene, Dipp=2,6‐diisopropylphenyl) and (NHC)⋅AliBu2H (NHC=iPr2Im (11), Dipp2Im (12)) as well as their reactivity towards different types of carbenes is presented. Although the mono‐NHC adducts remained stable at elevated temperatures, ring expansion occurred when (iPr2Im)⋅AlH3 (3) was treated with a second equivalent of the carbene iPr2Im to give (iPr2Im)⋅AlH(RER‐iPr2ImH2) (6). In 6, {(iPr2Im}AlH} is inserted into the NHC ring. In contrast, ring opening was observed with the sterically more demanding Dipp2Im with the formation of (iPr2Im)⋅AlH2(ROR‐Dipp2ImH2)H2Al⋅(iPr2Im) (9). In 9, two {(iPr2Im)⋅AlH2} moieties stabilize the ring‐opened Dipp2Im. If two hydridic sites are blocked, the adducts are stable with respect to further ring expansion or ring opening, as exemplified by the adducts (iPr2Im)⋅AliBu2H (11) and (Dipp2Im)⋅AliBu2H (12). The adducts (NHC)⋅AlH3 and (iPr2Im)⋅AliBu2H reacted with cAACMe by insertion of the carbene carbon atom into the Al−H bond to give (NHC)⋅AlH2/iBu2(cAACMeH) (13–18) instead of ligand substitution, ring‐expansion, or ring‐opened products.
Phenothiazine‐Sensitized Solar Cells: Effect of Number of Cyanocinnamic Acid Anchoring Groups on Dye‐Sensitized Solar Cell Performance
Authors: Yellappa Shivaraj , Webre Whitney A. , Gobeze Habtom B. , Middleton Anna , KC Chandra B. , D'Souza Francis 
Published: June 2017

AbstractDye‐sensitized solar cells (DSSCs) were built to probe the effect of the number of cyanocinnamic acid anchoring groups on the ring periphery of phenothiazine dyes. Two kinds of dyes, one with substitution on the N‐terminal (1) and the other at the C‐3 aromatic ring (2–5), have been synthesized for this purpose. Additionally, the number of cyanocinnamic acid groups have been increased to two in the case of compound 6. In the case of compounds 2–6, the N terminus was functionalized to carry methyl, 2‐hydroxyethane, tris(ethylene oxide), or hexyl groups. Evidence for charge injection from the excited singlet state of phenothiazine to TiO2 was secured from femtosecond transient absorption spectral studies, wherein an ultrafast charge injection process (kCI≈1010–1011 s−1) was witnessed. The DSSCs were built on nanocrystalline TiO2 by using the traditional two‐electrode method. Cell performance was better for carbon‐functionalized phenothiazine dyes compared with that of nitrogen‐functionalized dyes. The second cyanocinnamic group also increased the efficiency to 5.8 %.
Influence of Sulfuric Acid on the Performance of Ruthenium‐based Catalysts in the Liquid‐Phase Hydrogenation of Levulinic Acid to γ‐Valerolactone
Authors: Ftouni Jamal , Genuino Homer C. , Muñoz‐Murillo Ara , Bruijnincx Pieter C. A. , Weckhuysen Bert M. 
Published: June 2017

AbstractThe presence of biogenic or process‐derived impurities poses a major problem on the efficient catalytic hydrogenation of biomass‐derived levulinic acid to γ‐valerolactone; hence, studies on their influence on catalyst stability are now required. Herein, the influence of sulfuric acid as feed impurity on the performance of Ru‐based heterogeneous catalysts, including Ru/ZrO2 and mono‐ and bimetallic Ru‐on‐carbon catalysts in dioxane as solvent, was investigated. The carbon‐supported Ru catalysts proved to be very sensitive to minor amounts of sulfuric acid. In stark contrast, Ru/ZrO2 showed a remarkable stability in the presence of the same impurity, which is attributed to the sulfate‐ion adsorption capacity of the support. Preferential sulfate adsorption onto the surface of ZrO2 effectively protects the Ru active phase from deactivation by sulfur poisoning. A simple catalyst regeneration strategy was effective in removing adsorbed impurities, allowing efficient catalyst recycling.
Stable Organic Radicals in Lignin: A Review
Authors: Patil Shradha V. , Argyropoulos Dimitris S. 
Published: June 2017

AbstractLignin and the quest for the origin of stable organic radicals in it have seen numerous developments. Although there have been various speculations over the years on the formation of these stable radicals, researchers have not been able to arrive at a solid, unequivocal hypothesis that applies to all treatments and types of lignin. The extreme complexity of lignin and its highly aromatic, cross‐linked, branched, and rigid structure has made such efforts rather cumbersome. Since the early 1950s, researchers in this field have dedicated their efforts to the establishment of methods for the detection and determination of spin content, theoretical simulations, and reactions on model compounds and spin‐trapping studies. Although a significant amount of published research is available on lignin or its model compounds and the reactive intermediates involved during various chemical treatments (pulping, bleaching, extractions, chemical modifications, etc.), the literature provides a limited view on the origin, nature, and stability of such radicals. Consequently, this review is focused on examining the origin of such species in lignin, factors affecting their presence, reactions involved in their formation, and methods for their detection.
Regulation of lipid accumulation in Schizochytrium sp. ATCC 20888 in response to different nitrogen sources
Authors: Jiang Xin , Zhang Jia , Zhao Jing , Gao Ziqing , Zhang Chunzhi , Chen Ming 
Published: June 2017

Regulation of lipid accumulation in Schizochytrium sp. ATCC 20888 in response to different nitrogen sources was investigated in this study. Nitrogen sources had important effects on the cell growth, lipid accumulation, and docosahexaenoic acid (DHA) percentage in total fatty acids (TFAs). Compared with sole organic nitrogen source, the combination of yeast extract and monosodium glutamate (MSG) greatly enhanced the biomass, total lipids, and DHA percentage in TFAs, but inorganic nitrogen source addition decreased the biomass and total lipids. The results showed that lipids began to accumulate rapidly with the depletion of dissolved ammonia in broth, and the time required for nitrogen exhaustion was extended with increasing nitrogen source concentration. Furthermore, the analysis of isocitrate dehydrogenase (ICDH) and malic enzyme (ME) activities during fermentation process provided insight for the revelation of regulation mechanism of lipid accumulation in Schizochytrium sp. ATCC 20888 in response to nitrogen exhaustion in broth.Practical applications: DHA, an essential nutrient for human health, is widely used in infant formula and functional foods. DHA production by Schizochytrium sp. has recently received extensive attention as the commercial source of DHA to substitute marine fish oil. Nitrogen sources in the cultivation of Schizochytrium sp. have important effects on the cell growth, lipid accumulation, and DHA percentage in TFAs. This study provides valuable guidance for nitrogen source selection for efficient DHA‐rich lipids production by Schizochytrium sp.The combination of yeast extract and MSG enhances the biomass, lipid yield, and DHA percentage in TFAs, but inorganic nitrogen source addition decreases the biomass and lipid yield. The results in this study have important significance in understanding lipid accumulation regulation in Schizochytrium sp. in response to nitrogen sources and providing guidance for DHA‐rich lipid production enhancement.
The impact of natural disasters on energy consumption: An analysis of renewable and nonrenewable energy demand in the residential and industrial sectors
Authors: Doytch Nadia , Klein Yehuda L. 
Published: June 2017

This study examines the impact of infrastructure‐damaging natural disasters (meteorological and geophysical disasters) on energy consumption differentiating by type of energy: residential versus industrial and nonrenewable versus renewable. We used a novel comprehensive unbalanced data set spanning 50 years (1961–2011) for up to 80 countries, which we group by level of development to reduce heterogeneity within the group. We applied an estimation method that takes into account the dynamics of the economic processes in the panel: the Blundell and Bond GMM estimator. For high‐income economies, which are also technologically the most advanced, we are able to demonstrate a positive impact on renewable energy use 5 years after the occurrence of a geophysical disaster. For low‐income economies, we observe positive effects on industrial energy consumption; for middle‐income countries, on residential energy consumption. © 2017 American Institute of Chemical Engineers Environ Prog, 37: 37–45, 2018
Mitochondrial Point Mutation m.3243A&gt;G Associates With Lower Bone Mineral Density, Thinner Cortices, and Reduced Bone Strength: A Case-Control Study
Authors: Langdahl Jakob Høgild , Frederiksen Anja Lisbeth , Hansen Stinus Jørn , Andersen Per Heden , Yderstræde Knud Bonnet , Dunø Morten , Vissing John , Frost Morten 
Published: June 2017

ABSTRACT Mitochondrial dysfunction is associated with several clinical manifestations including diabetes mellitus (DM), neurological disorders, renal and hepatic diseases, and myopathy. Although mitochondrial dysfunction is associated with increased bone resorption and decreased bone formation in mouse models, effects of alterations in mitochondrial function on bone remodeling and mass have not been investigated in humans. We recruited 45 carriers (29 females, 16 males) with the m.3243A&gt;G mutation and healthy controls matched for gender, age, height, and menopausal status. DXA and HRpQCT scans were performed, and bone turnover markers (BTMs) P1NP and CTX were measured. Cases and controls were well matched except for body weight, which was lower in cases (63.6 ± 18.1 kg versus 74.6 ± 14.8 kg, p &lt; 0.01), and manifest DM was present in 25 of 45 cases (none in controls). Bone scans showed lower BMD at the lumbar spine, total hip, and femoral neck in cases. Mean lumbar spine, total hip, and femoral neck T-scores were –1.5, –1.3, and –1.6 in cases, respectively, and –0.8, –0.3, and –0.7 in controls (all p &lt; 0.05). The m.3243A&gt;G mutation was associated with lower BMD, cortical but not trabecular density, cortical thickness, and estimated bone strength. Furthermore, BTMs were lower in the m.3243A&gt;G group before but not after adjustment for DM. The mitochondrial point mutation m.3243A&gt;G was associated with decreased bone mass and strength. Although the coexistence of DM may have influenced bone turnover, the bone phenotype observed in m.3243A&gt;G cases appeared to mirror age-related deterioration in bone, suggesting that mitochondrial dysfunction may cause a premature aging of bone. © 2017 The Authors. Journal of Bone and Mineral Research Published by Wiley Periodicals Inc.
Clinical implications of pretreatment inflammatory biomarkers as independent prognostic indicators in prostate cancer
Authors: Sun Zhaohui , Ju Ying , Han Fuyan , Sun Xiya , Wang Fang 
Published: June 2017

ObjectivesResearch on the relationship between inflammatory biomarkers and malignant tumors has become a hotspot. Many studies have demonstrated that neutrophil‐lymphocyte ratio (NLR), platelet‐lymphocyte ratio (PLR), and red blood cell distribution width (RDW) could act as independent prognostic indicators for several solid tumors. This study aimed to evaluate the clinical implications of pretreatment inflammatory biomarkers, including NLR, PLR, and RDW as independent prognostic indicators in prostate cancer (PCa).MethodsA total of 226 PCa patients who were diagnosed at our institution from 2011 to 2016 were analyzed retrospectively. We compared the clinicopathological features, survival curves, and prognosis of the PCa patients between the high and low groups according to the cutoffs of NLR, PLR, and RDW.ResultsThe pretreatment NLR, PLR, and RDW values were significantly higher in the patients with PCa than those in the controls (P<.05). Increased NLR and PLR values were significantly associated with high risk of progression, including higher Gleason scores, cell proliferation antigen 67 (Ki‐67) indexes, and prostate‐specific antigen (PSA) levels (P<.05), whereas an elevated RDW was only associated with an older age. An increased NLR was correlated with both overall survival (OS) (P=.025) and disease‐free survival (DFS) (P=.017). In addition, a higher PLR only showed a significantly worse DFS (P=.040). Pretreatment NLR was an independent prognostic indicator of DFS.ConclusionsThe pretreatment NLR and PLR might be beneficial to predict the progression and prognosis of PCa. Furthermore, NLR was more effective than PLR acting as an independent prognostic indicator for PCa.