bluuebunny/crossref_metadata_2025 huggingface dataset:
Investment performance, regulation and incentives: the case of Chilean pension funds
Authors: López Fernando , Walker Eduardo
Published: January 2020

We examine the investment performance of Chilean pension funds during their multi-fund period (2003–17). Using tradable asset class benchmarks, we extend Sharpe's (1992) return-based style analysis by explicitly considering regulatory restrictions and currency hedging. We find that despite the significant differences between pension fund manager returns, they are statistically similar to our style benchmarks for all fund types. Furthermore, accounting for currency hedging improves the accuracy of the replicating portfolios and the selection return estimates. Our results have policy implications for investment regulation of pension systems with similar characteristics to the Chilean one.
Chiral emission and Purcell enhancement in a hybrid plasmonic-photonic microresonator
Authors: Cao Qi-Tao , Chen You-Ling , Xiao Yun-Feng
Published: January 2020

A high-Q hybrid plasmonic-photonic microresonator, which consists of a dielectric microdisk hybridized with a plasmonic nanoantenna dimer, enables an enlarged local density of states of the optical field and chiral propagation of photons inside the cavity.
Evidence for phylogenetically and catabolically diverse active diazotrophs in deep-sea sediment
Authors: Kapili Bennett J , Barnett Samuel E , Buckley Daniel H , Dekas Anne E
Published: January 2020

Diazotrophic microorganisms regulate marine productivity by alleviating nitrogen limitation. However, we know little about the identity and activity of diazotrophs in deep-sea sediments, a habitat covering nearly two-thirds of the planet. Here, we identify candidate diazotrophs from Pacific Ocean sediments collected at 2893 m water depth using 15N-DNA stable isotope probing and a novel pipeline for nifH sequence analysis. Together, these approaches detect an unexpectedly diverse assemblage of active diazotrophs, including members of the Acidobacteria, Firmicutes, Nitrospirae, Gammaproteobacteria, and Deltaproteobacteria. Deltaproteobacteria, predominately members of the Desulfobacterales and Desulfuromonadales, are the most abundant diazotrophs detected, and display the most microdiversity of associated nifH sequences. Some of the detected lineages, including those within the Acidobacteria, have not previously been shown to fix nitrogen. The diazotrophs appear catabolically diverse, with the potential for using oxygen, nitrogen, iron, sulfur, and carbon as terminal electron acceptors. Therefore, benthic diazotrophy may persist throughout a range of geochemical conditions and provide a stable source of fixed nitrogen over geologic timescales. Our results suggest that nitrogen-fixing communities in deep-sea sediments are phylogenetically and catabolically diverse, and open a new line of inquiry into the ecology and biogeochemical impacts of deep-sea microorganisms.
Identification of a transient state during the acquisition of temozolomide resistance in glioblastoma
Authors: Rabé Marion , Dumont Solenne , Álvarez-Arenas Arturo , Janati Hicham , Belmonte-Beitia Juan , Calvo Gabriel F. , Thibault-Carpentier Christelle , Séry Quentin , Chauvin Cynthia , Joalland Noémie , Briand Floriane , Blandin Stéphanie , Scotet Emmanuel , Pecqueur Claire , Clairambault Jean , Oliver Lisa , Perez-Garcia Victor , Nadaradjane Arulraj , Cartron Pierre-François , Gratas Catherine , Vallette François M.
Published: January 2020

Drug resistance limits the therapeutic efficacy in cancers and leads to tumor recurrence through ill-defined mechanisms. Glioblastoma (GBM) are the deadliest brain tumors in adults. GBM, at diagnosis or after treatment, are resistant to temozolomide (TMZ), the standard chemotherapy. To better understand the acquisition of this resistance, we performed a longitudinal study, using a combination of mathematical models, RNA sequencing, single cell analyses, functional and drug assays in a human glioma cell line (U251). After an initial response characterized by cell death induction, cells entered a transient state defined by slow growth, a distinct morphology and a shift of metabolism. Specific genes expression associated to this population revealed chromatin remodeling. Indeed, the histone deacetylase inhibitor trichostatin (TSA), specifically eliminated this population and thus prevented the appearance of fast growing TMZ-resistant cells. In conclusion, we have identified in glioblastoma a population with tolerant-like features, which could constitute a therapeutic target.
Normal early development in siblings with novel compound heterozygous variants in ASPM
Authors: Moriwaki Taro , Yamazaki Narutoshi , So Tetsumin , Kosuga Motomichi , Miyazaki Osamu , Narumi-Kishimoto Yoko , Kaname Tadashi , Nishimura Gen , Okuyama Torayuki , Fukuhara Yasuyuki
Published: January 2020

Autosomal recessive primary microcephaly 5 (MCPH5) is caused by pathogenic variants in ASPM. Using whole-exome sequencing, we diagnosed two siblings with MCPH5. A known pathogenic variant (NM_018136.4: c.9697C > T, p.(Arg3233*)) and a novel pathogenic variant (c.1402_1406del, p.(Asn468Serfs*2)) of ASPM were identified in affected siblings with normal intelligence. Their pathogenic variants were not located in the critical regions of ASPM, but the relationship between the genotypes and their normal intelligence was unclear.
Differential functional roles of fibroblasts and pericytes in the formation of tissue-engineered microvascular networks in vitro
Authors: Kosyakova Natalia , Kao Derek D. , Figetakis Maria , López-Giráldez Francesc , Spindler Susann , Graham Morven , James Kevin J. , Won Shin Jee , Liu Xinran , Tietjen Gregory T. , Pober Jordan S. , Chang William G.
Published: January 2020

Formation of a perfusable microvascular network (μVN) is critical for tissue engineering of solid organs. Stromal cells can support endothelial cell (EC) self-assembly into a μVN, but distinct stromal cell populations may play different roles in this process. Here we describe the differential effects that two widely used stromal cell populations, fibroblasts (FBs) and pericytes (PCs), have on μVN formation. We examined the effects of adding defined stromal cell populations on the self-assembly of ECs derived from human endothelial colony forming cells (ECFCs) into perfusable μVNs in fibrin gels cast within a microfluidic chamber. ECs alone failed to fully assemble a perfusable μVN. Human lung FBs stimulated the formation of EC-lined μVNs within microfluidic devices. RNA-seq analysis suggested that FBs produce high levels of hepatocyte growth factor (HGF). Addition of recombinant HGF improved while the c-MET inhibitor, Capmatinib (INCB28060), reduced μVN formation within devices. Human placental PCs could not substitute for FBs, but in the presence of FBs, PCs closely associated with ECs, formed a common basement membrane, extended microfilaments intercellularly, and reduced microvessel diameters. Different stromal cell types provide different functions in microvessel assembly by ECs. FBs support μVN formation by providing paracrine growth factors whereas PCs directly interact with ECs to modify microvascular morphology.
Optimized fluorodendrimer-incorporated hybrid lipid–polymer nanoparticles for efficient siRNA delivery
Authors: Xiong Qingqing , Li Yujing , Zhou Kun , Chen Ping , Guo Hua , Chen Lu , Ding Jianxun , Song Tianqiang , Shi Jinjun
Published: January 2020

The optimized fluorodendrimer G0-(F13)5 is developed to construct hybrid lipid–polymer nanoparticles for efficient siRNA delivery and gene silencing.
Real-time monitoring of surface acetone enolization and aldolization
Authors: Li Houqian , Sun Junming , Li Gengnan , Wu Di , Wang Yong
Published: January 2020

Real-time DRIFTS reveals the formation of acetone enolate and its subsequent aldolization via an Eley–Rideal type mechanism on Zn1Zr10Oz.
Construction of novel phosphonate-based MOF/P–TiO2 heterojunction photocatalysts: enhanced photocatalytic performance and mechanistic insight
Authors: Zeng Tianyu , Shi Dajun , Cheng Qingrong , Liao Guiying , Zhou Hong , Pan Zhiquan
Published: January 2020

A new phosphonate-based MOF/P–TiO2Z-scheme heterojunction photocatalyst had been successfully prepared for the environmental remediation.
Two-dimensional metal–organic frameworks and their derivatives for electrochemical energy storage and electrocatalysis
Authors: Zhao Kuangmin , Zhu Weiwei , Liu Suqin , Wei Xianli , Ye Guanying , Su Yuke , He Zhen
Published: January 2020

The recent progress on the fabrication of two-dimensional metal–organic frameworks and their derivatives as well as their applications in electrochemical energy storage and electrocatalysis are reviewed.
Visible-light-driven self-coupling and oxidative dehydrogenation of amines to imines via a Mn(ii)-based coordination polymer
Authors: Liu Hui , Guo Zhifen , Lv Hui , Liu Xin , Che Yan , Mei Yingchun , Bai Rong , Chi Yanhong , Xing Hongzhu
Published: January 2020

The direct synthesis of various imines through visible-light-driven photocatalytic self-coupling and dehydrogenation of amines was achieved using a novel coordination polymer.
Transition metal-free electrocatalytic halodeborylation of arylboronic acids with metal halides MX (X = I, Br) to synthesize aryl halides
Authors: Fu Zhengjiang , Hao Guangguo , Fu Yaping , He Dongdong , Tuo Xun , Guo Shengmei , Cai Hu
Published: January 2020

An efficient and regioselective ipso-halogenation of diverse arylboronic acids with metal halide salts MX (X = I, Br) has been well established under electrochemical conditions.
Copper-catalyzed [4 + 2] annulation reaction of β-enaminones and aryl diazonium salts without external oxidant: synthesis of highly functionalized 3H-1,2,4-triazines via homogeneous or heterogeneous strategy
Authors: Ding Qifeng , Li Mingrui , Sun Yiming , Yu Yang , Baell Jonathan B. , Huang Fei
Published: January 2020

Herein, both homogeneous and heterogeneous strategies were developed to access highly functionalized 3H-1,2,4-triazines using Cu-catalyzed [4 + 2] annulation.
Yellow pigment formation, pigment composition, and quality of fresh-cut yam (Dioscorea opposita) slices
Authors: Zhao Lei , Wang Dan , Ma Yue , Zhang Yan , Zhao Xiaoyan
Published: January 2020

Fresh-cut yam (Dioscorea opposita) slices brown easily and can turn yellow under certain storage conditions.
Cooperative effects of surface and interface treatments in a hematite (α-Fe2O3) photo-anode on its photo-electrochemical performance
Authors: Inaba Mika , Katayama Kenji , Sohn Woon Yong
Published: January 2020

To enhance the efficiency of the oxygen evolution reaction of hematite (α-Fe2O3), we engineered both the surface of the hematite nanorods and the FTO/hematite interface simultaneously with an optimal annealing condition.
Shape-stabilized phase-change materials supported by eggplant-derived porous carbon for efficient solar-to-thermal energy conversion and storage
Authors: Li Yaqiong , Huang Xiubing , Li Yang , Xi Zuoshuai , Hai Guangtong , Tao Zhang , Wang Ge
Published: January 2020

3D spongy-like porous carbon materials derived from eggplants were used as scaffolds for encapsulating polyethylene glycol (PEG) to fabricate shape-stabilized composite phase-change materials with excellent solar-to-thermal conversion efficiency.
Amorphous WO3 induced lattice distortion for a low-cost and high-efficient electrocatalyst for overall water splitting in acid
Authors: Fan Ke , He Min , Dharanipragada N. V. R. Aditya , Kuang Panyong , Jia Yufei , Fan Lizhou , Inge A. Ken , Zhang Biaobiao , Sun Licheng , Yu Jiaguo
Published: January 2020

An amorphous WO3 induced lattice distortion strategy leads to only 2 wt% Ir for efficient overall water splitting in acid.
A self-assembled peptide hydrogel for cytokine sequestration
Authors: Kim Ka-Kyung , Siddiqui Zain , Patel Manali , Sarkar Biplab , Kumar Vivek A.
Published: January 2020

Supramolecular hydrogels that can chelate cytokine mediators of inflammation.
Non-toxic green food additive enables efficient polymer solar cells through adjusting the phase composition distribution and boosting charge transport
Authors: Li Jianfeng , Wang Yufei , Liang Zezhou , Qin Jicheng , Ren Meiling , Tong Junfeng , Yang Chunyan , Yang Chunming , Bao Xichang , Xia Yangjun
Published: January 2020

Solvent additives play an important role in optimizing the morphology of the photoactive layer and improving the photovoltaic performance of polymer solar cells (PSCs).
Ultrafast, self-powered and charge-transport-layer-free photodetectors based on high-quality evaporated CsPbBr3 perovskites for applications in optical communication
Authors: Liu Xingyue , Liu Zhiyong , Li Junjie , Tan Xianhua , Sun Bo , Fang Han , Xi Shuang , Shi Tielin , Tang Zirong , Liao Guanglan
Published: January 2020

Ultrafast and self-powered photodetectors based on high-quality evaporated CsPbBr3 perovskites for applications in optical communication are demonstrated. The photodetectors achieve an ultrafast response time of 3.8 μs and on/off ratio of 3.5 × 104.
Interplay of mRNA capping and transcription machineries
Authors: Kachaev Zaur M. , Lebedeva Lyubov A. , Kozlov Eugene N. , Shidlovskii Yulii V.
Published: January 2020

Early stages of transcription from eukaryotic promoters include two principal events: the capping of newly synthesized mRNA and the transition of RNA polymerase II from the preinitiation complex to the productive elongation state. The capping checkpoint model implies that these events are tightly coupled, which is necessary for ensuring the proper capping of newly synthesized mRNA. Recent findings also show that the capping machinery has a wider effect on transcription and the entire gene expression process. The molecular basis of these phenomena is discussed.
Les futures générations d’anticorps modulateurs des points de contrôle de la réponse immunitaire
Authors: Bonnefoy Nathalie , Olive Daniel , Vanhove Bernard
Published: January 2020

Les points de contrôle du système immunitaire sont des systèmes moléculaires qui complètent les processus déclenchés par la reconnaissance antigénique en contrôlant l’inhibition ou l’activation des lymphocytes et des cellules myéloïdes, notamment celle des lymphocytes T régulateurs (Treg), permettant ainsi de combiner réponses immunes et maintien de la tolérance au soi. En cancérologie, l’inhibition de points de contrôle inhibiteurs vise à amplifier les réponses immunitaires existantes dirigées contre les tumeurs. Parmi ces points de contrôle inhibiteurs, dont des antagonistes sont en utilisation clinique, se trouvent CTLA-4 (cytolytic T-lymphocyte-associated antigen 4 ou CD152), PD-1 (programmed cell death 1, ou CD279), PD-L1 (programmed cell death-ligand 1, ou CD274), LAG-3 (Lymphocyte-activation gene 3, ou CD223), TIM3 (T-cell immunoglobulin and mucin-domain containing-3), TIGIT (T cell immunoreceptor with Ig and ITIM domains), VISTA (V-domain Ig suppressor of T cell activation), ou B7/H3 (ou CD276). La stimulation de points de contrôle activateurs tels que les molécules de co-activation CD28, CD137 (aussi appelé 4-1BB), OX40 [aussi appelé tumor necrosis factor receptor superfamily, member 4 (TNFRSF4)], GITR (Glucocorticoid-induced tumor necrosis factor receptor family-related protein) ou CD40, est également testée en cancérologie, le plus souvent en combinaison avec un antagoniste de point de contrôle inhibiteur. Dans les maladies auto-immunes et inflammatoires, des antagonistes de points de contrôle activateurs (CD28, CD40) et des agonistes de points de contrôle inhibiteurs (LAG-3) sont également à l’essai. Dans cette revue, nous mettons l’accent sur certains modulateurs de points de contrôle pour lesquels le mécanisme d’action a été particulièrement étudié. Cette description ne pouvant être exhaustive, nous avons regroupé dans le Tableau I l’ensemble des anticorps monoclonaux (AcM) ou protéines recombinantes en usage clinique à notre connaissance, modulant l’action d’un point de contrôle du système immunitaire.
Ingénierie de lymphocytes B humains produisant des anticorps neutralisant le virus VIH-1 par édition génique CRISPR-Cas9
Authors: Beretta Maxime , Mouquet Hugo
Published: January 2020

Les anticorps (ou immunoglobulines, Ig) produits par les lymphocytes B sont essentiels aux réponses immunitaires induites par les infections et les vaccins. Les anticorps sont des glycoprotéines hétérodimériques résultant de l’association de deux chaînes lourdes (IgH), et de deux chaînes légères (IgL) d’immunoglobuline. Les chaînes IgH et IgL possèdent des régions « hypervariables », également appelées en anglais complementarity determining regions (CDR), situées dans leurs domaines variables, VH et VL, qui, en se combinant, forment le site de liaison à l’antigène ou paratope.
Anticorps monoclonaux en infectiologie
Authors: Desoubeaux Guillaume , Pelegrin Mireia
Published: January 2020

Le développement des anticorps thérapeutiques en infectiologie est beaucoup plus récent qu’en cancérologie, à l’exception d’un anticorps anti-virus respiratoire syncytial (VRS), mais il est désormais un domaine en pleine expansion. À l’échelle mondiale, sept de ces anticorps ont déjà été approuvés par des autorités de santé, dont seulement cinq en France. À ce jour, les indications sont restreintes à la prévention de la bronchiolite liée au VRS, au traitement de la maladie VIH/Sida en échec thérapeutique, à l’exposition au virus de la rage et à la maladie du charbon, à la colite post-antibiotique à Clostridium difficile, et au syndrome hémolytique et urémique atypique à Escherichia coli entéro-hémorragique. Dans un futur proche, l’essor des nouvelles technologies devrait permettre d’accélérer le développement d’anticorps monoclonaux anti-infectieux afin d’étoffer l’arsenal antibiotique et antibactérien déjà à disposition.
Les anticorps, outils de choix pour la chirurgie guidée par fluorescence
Authors: Pèlegrin André , Gutowski Marian , Cailler Françoise
Published: January 2020

La chirurgie guidée par fluorescence se développe en clinique depuis plusieurs années. Si l’utilisation de colorants non ciblés peut être utile dans certaines pathologies, des agents de contraste spécifiques sont indispensables en oncologie. Comme le montrent les dernières études cliniques, les anticorps monoclonaux ont toutes les caractéristiques pour jouer un rôle majeur dans ce domaine d’imagerie médicale, à condition que la cible antigénique soit pertinente.
Imiter la réponse immunitaire humorale polyclonale
Authors: Larbouret Christel , Poul Marie-Alix , Chardès Thierry
Published: January 2020

Les anticorps monoclonaux ont révolutionné le traitement de nombreuses maladies mais leur efficacité clinique reste limitée dans certains cas. Des associations d’anticorps se liant à une même cible (homo-combinaisons) ou à plusieurs cibles différentes (hétéro-combinaisons), mimant ainsi une réponse immunitaire humorale polyclonale, ont conduit à une amélioration thérapeutique dans des essais précliniques et cliniques, essentiellement en cancérologie et en infectiologie. Ces combinaisons augmentent l’efficacité des réponses biologiques et court-circuitent les mécanismes de résistances observés lors d’une monothérapie par anticorps. Le procédé de formulation et d’administration des combinaisons d’anticorps le plus fréquent est une formulation séparée, avec injection séquentielle de chaque anticorps « principe actif ». Alternativement, se développent des formulations combinées, où les anticorps produits séparément sont mélangés avant administration, ou produits simultanément par une lignée cellulaire unique ou un mélange de lignées cellulaires correspondant à unemaster-bankcellulaire polyclonale. La réglementation, la toxicité et la séquence d’injection des mélanges oligoclonaux restent des points à éclaircir et à optimiser pour un meilleur effet thérapeutique.
Les formats alternatifs aux anticorps
Authors: Kitten Olivier , Martineau Pierre
Published: January 2020

Les anticorps sont désormais devenus d’une utilisation courante dans un large champ thérapeutique qui n’est plus restreint à la cancérologie et à l’inflammation. Cette explosion du domaine conduit à des besoins nouveaux qui peuvent être mieux remplis par des molécules inspirées mais différentes des anticorps classiques. En particulier, la molécule anticorps a de multiples fonctions qui ne sont pas toujours nécessaires, comme sa capacité à recruter les cellules du système immunitaire, à se lier de façon bivalente à sa cible ou à présenter une demi-vie plasmatique élevée. En revanche, dans la grande majorité des applications, sa remarquable capacité à reconnaître spécifiquement sa cible moléculaire et surtout sa diversité de reconnaissance doivent être conservées. De plus, les anticorps sont des molécules de très haut poids moléculaire, coûteuses à produire et qui présentent des propriétés physicochimiques limitées ne permettant pas leur utilisation dans des milieux agressifs. Finalement, dans certaines applications thérapeutiques, la grande taille de la molécule (environ 150 kDa) peut également limiter sa diffusion dans les tissus et empêcher la reconnaissance de certaines structures moléculaires peu accessibles. Pour répondre à ces limitations, de nombreux formats alternatifs aux anticorps entiers ont été développés au cours de ces vingt dernières années. Les applications couvrent les domaines de la biotechnologie, du diagnosticin vitroetin vivoet de la thérapie. Deux grandes familles de molécules permettent de couvrir ce champ et seront présentées dans cette mini-revue. Une première famille s’appuie sur la diversité naturelle des anticorps mais en en réduisant la taille, comme les fragments d’anticorps classiques (Fab, scFv) ou ceux provenant des camélidés ou des requins (VHH, V-NAR). La deuxième famille a été développée en partant des propriétés finales désirées et notamment la stabilité en milieu extrême et la productivité en système simple et économique de production comme l’utilisation de bactéries et en y greffant des propriétés de liaison comparables aux anticorps par des méthodes d’évolution moléculaire dirigéein vitro. Cette mini-revue se concentrera sur les molécules les plus avancées, mais le domaine est en très forte et rapide expansion. Il faut noter que beaucoup de ces molécules, voire ces approches, sont couvertes par des brevets et sont souvent développées dans le cadre de jeunes sociétés innovantes dont certaines ont déjà été rachetées par de grands groupes de la pharmacie.
Améliorer le ciblage tissulaire des anticorps thérapeutiques par de nouveaux formats
Authors: Lafaye Pierre , Lesuisse Dominique , Declèves Xavier
Published: January 2020

De nombreuses pathologies cérébrales neurodégénératives ou tumorales devraient pourvoir bénéficier des progrès thérapeutiques impressionnants des anticorps médicaments. Malheureusement, en raison de leur très faible passage dans le cerveau, de nombreux développements cliniques d’anticorps dont la cible thérapeutique se situe dans le parenchyme cérébral ont été arrêtés par manque d’efficacité. La barrière hémato-encéphalique (BHE), douée de propriétés extrêmement sélectives et restrictives, est à l’origine de la faible pénétration cérébrale des molécules de haute masse moléculaire, telles que les anticorps thérapeutiques. L’objectif de cette revue est de présenter les propriétés de la BHE et les dernières avancées dans le domaine de l’ingénierie de nouveaux formats d’anticorps susceptibles d’améliorer leur passage intracérébral.
Effects of a Simulated Game on Pitching Kinematics in Youth Softball Pitcher
Authors: Downs Jessica , Friesen Kenzie , W. Anz Adam , Dugas Jeffrey R. , R. Andrews James , Oliver Gretchen D.
Published: January 2020

Despite evidence that overuse is the most common mechanism of injury, softball pitchers currently have no pitch count regulations. Pain has been associated with certain pitching pathomechanics, and some reports indicate increased pain following a single pitching bout. Therefore, the purpose of this study was to examine trunk and lower extremity kinematics during the first and last inning of a game, as well as last inning pitch volume in youth softball pitchers. Thirty-two youth softball pitchers (12.4±1.6 years, 159.4±8.9 cm, 62.0±13.6 kg) pitched a simulated game. Three fastballs were averaged and analyzed from the first and last inning. Kinematic data were collected at 100Hz using an electromagnetic system, synced with motion analysis software. A Wilcoxon signed rank test revealed pitchers exhibited less trunk rotation toward their pitching arm side in the last inning. A bivariate Pearson’s correlation showed volume of pitches was correlated with stride length (r=0.367, p=0.039) and center of mass (r=0.364, p=0.041) at the start of the pitch, and trunk flexion at top of pitch (r=−0.392, p=0.026), foot contact (r=−0.413, p=0.019), and follow-through (r=−0.436, p=0.013). This study found that pitching a simulated game did result in altered pitching mechanics, meanwhile pitch volume was also correlated with pitching mechanics.
Smith–Magenis Syndrome: Clues in the Clinic
Authors: Akkus Nejmiye , Kilic Betul , Cubuk Pelin Ozyavuz
Published: January 2020

As a multisystemic congenital mental retardation disorder/anomaly, Smith–Magenis syndrome (SMS) is commonly aroused from de novo interstitial deletion of the 17p11.2 chromosome. The deletion of this chromosome results with haploinsufficiency for the retinoic acid-induced 1 (RAI1) gene. In this article, we present three cases, who were diagnosed with SMS with mental retardation and behavioral problems such as self-hugging and sleeping disturbances. During the evaluation of the patients, it has been found that there was a 3.4-Mb deletion in the 17p11.2 chromosome region of these patients. This deletion includes RAI1 that is a critically involved gene in SMS.
Hypocalcemia following Neridronate Administration in Pediatric Patients with Osteogenesis Imperfecta: A Prospective Observational Study
Authors: Maines Evelina , Tadiotto Elisa , Morandi Grazia , Fedrizzi Michela , Gaudino Rossella , Cavarzere Paolo , Guzzo Alessandra , Antoniazzi Franco
Published: January 2020

The use of intravenous bisphosphonates has been linked to hypocalcemia both in children and adults with osteogenesis imperfecta (OI). The aims of this study were: (1) to investigate the incidence of hypocalcemia in the first 48 hours (T48) after neridronate infusion in a pediatric population with OI and (2) to assess any correlation between the baseline values of calcium, vitamin D (25-hydroxyvitamin D) and bone turnover markers, and the postinfusion calcium values. We conducted a prospective observational study on 37 pediatric patients. All patients were treated with a single infusion of neridronate at a dose of 1 to 2 mg/kg. The study provided two postinfusion reassessments: 24 hours (T24) and T48 after neridronate administration. Hypocalcemia was observed in 11% of patients at T24 and in 50% of patients at T48 from neridronate infusion. We observed a positive linear correlation between the baseline vitamin D values and postinfusion calcium values, both at baseline and at T24 and T48. Hypocalcemia was mild and asymptomatic in all cases. Postinfusion calcium levels were related to baseline vitamin D levels. Consequently, low vitamin D levels should be considered a significant risk factor for hypocalcemia and should be carefully investigated and treated before neridronate infusion.
Ophthalmologic Impairment and Intellectual Disability in a Girl Presenting Kenny-Caffey Syndrome Type 2
Authors: Deconte Desirée , Kreusch Tulia Cristina , Salvaro Bruna Pavan , Perin Wagner Fernando , Ferreira Maria Angélica Tosi , Kopacek Cristiane , da Rosa Ernani Bohrer , Heringer Jane Iândora , Ligabue-Braun Rodrigo , Zen Paulo Ricardo Gazzola , Rosa Rafael Fabiano Machado , Fiegenbaum Marilu
Published: January 2020

Kenny-Caffey syndrome (KCS) is a rare genetic condition characterized by growth retardation, bone abnormalities, and hypoparathyroidism. Herein, we report an unusual case of a 10-year-old girl with Kenny-Caffey syndrome type 2 (KCS2) presenting with vision impairment-suspected maculopathy and intellectual disability. Endocrine evaluation showed low calcium and high phosphorus plasma levels. Radiographic evaluation revealed short metacarpal bones and delayed bone age. Sequencing analysis showed a missense variant in FAM111A (R569H), unidentified in her parents. Better understanding of potential neurological and ophthalmological findings in KCS2 patients is important to improve quality of life of these patients as usually they exhibit long survival.
Peruvian Newborn Male with 3p13 Deletion Syndrome Encompassing the FOXP1 Gene: Review of the Literature
Authors: Abarca-Barriga Hugo H. , Trubnykova Milana , Chavesta-Velásquez Félix , Barletta-Carrillo Claudia , Ordoñez-Linares Marco , Rondón-Abuhadba Andrea
Published: January 2020

Copy number variation in loss of 3p13 is an infrequently reported entity characterized by hypertelorism, aniridia, microphthalmia, high palate, neurosensorial deafness, camptodactyly, heart malformation, development delay, autism spectrum disorder, seizures, and choanal atresia. The entity is caused probably by haploinsufficiency for FOXP1, UBA3, FAM19A1, and MITF. We report a newborn male with hypotonia, facial dysmorphism, heart malformation, and without clinical diagnosis; nevertheless, the use of appropriate genetic test, such us the chromosomal microarray analysis allowed identification of a copy number variant in loss of 5.5 Mb at chromosome 3 (p13-p14.1), that included 54 genes, encompassing FOXP1 gene. We compare the findings in our Peruvian patient to those of earlier reported patients; furthermore, add new signs for this entity.
The impact of the carrier gas composition of non-thermal atmospheric pressure plasma jet for bacteria sterilization
Authors: Lotfy Khaled
Published: January 2020

In this work, the bacteria inactivation using the nonthermal atmospheric pressure plasma has been studied. The bacteria inactivation was conducted using a self-design nonthermal atmospheric pressure plasma jet system. During this experiment, Escherichia coli was used as an objective microorganism. The primary operating gas for the plasma jet used in this work is helium, and small fractions of oxygen or nitrogen (0.2%) were used as the secondary gas. The three plasma jet cases were operated at 3.5 kV, 14 l/m, and 7 mm, which represented the applied voltage, gas flow rate, and distance from the nozzle, respectively. The types of reactive species have been examined using optical emission spectroscopy. The gas temperature and optical emission spectrum were measured under the same condition. The active species of OH, OII, OI, N21+, N22+, and He are indented in the UV-vis wavelength range. The inactivation of E. coli bacteria has occurred after 20 s of nonthermal plasma treatment, whether the carrier gas is pure helium or helium + nitrogen or helium + oxygen. The results revealed that the impact of helium is less than that of helium + 0.2% nitrogen which is less than that of helium + 0.2% oxygen. The current results of this experiment could be utilized in improving the nonthermal plasma jet for extended surface decontamination.
Field-effect transistor based on surface negative refraction in Weyl nanowire
Authors: Chen Guangze , Chen Wei , Zilberberg Oded
Published: January 2020

Weyl semimetals are characterized by their bulk Weyl points—conical band touching points that carry a topological monopole charge—and Fermi arc states that span between the Weyl points on the surface of the material. Recently, significant progress has been made toward the understanding and measurement of the physical properties of Weyl semimetals. Yet, potential applications remain relatively sparse. Here we propose Weyl semimetal nanowires as field-effect transistors, dubbed WEYLFETs. Specifically, applying gradient gate voltage along the nanowire, an electric field is generated, which effectively tilts the open surfaces, thus, varying the relative orientation between Fermi arcs on different surfaces. As a result, perfect negative refraction between adjacent surfaces can occur and longitudinal conductance along the wire is suppressed. The WEYLFET offers a high on/off ratio with low power consumption. Adverse effects due to dispersive Fermi arcs and surface disorder are studied.
Epitaxial strain and the magnetic properties of canted antiferromagnetic perovskite NaNiF3 thin films
Authors: Morley Sophie A. , Marquez Humberto , Lederman David
Published: January 2020

The perovskite crystal structure is known to exhibit a multitude of interesting physical phenomena owing to the intricate coupling of the electronic and magnetic properties to the structure. Fluoroperovskites offer an alternative chemistry to the much more widely studied oxide materials, which may prove advantageous for applications. It is demonstrated here for the first time that the antiferromagnetic perovskite fluoride, NaNiF3, can be synthesized in thin film form. The films were grown via molecular beam epitaxy on SrTiO3 (100) substrates to produce high quality epitaxial films in the thickness range of 5–50 nm. The Pnma structure of the films was confirmed by x-ray diffraction. There was a decrease in the out-of-plane lattice spacing from the bulk value corresponding to a maximum strain of 1.7% in the thinnest film. Canted antiferromagnetism was measured in all films using magnetometry and a negative change in the antiferromagnetic ordering temperature of ΔTN = - 9.1 ± 0.7 K was observed with increasing strain.
A compact photoelectric measurement system for harmonic vibrating table
Authors: Ma Ling , Lin Guan , Duan Fajie
Published: January 2020

A compact measurement system for harmonic vibration based on photoelectric array is proposed. Cooperating with a planar reflective mirror, which is fixed on the measured vibrating table, the photoelectric array outputs a vibration-sensitive signal. Theoretical model and simulation are discussed for the optical sensor. A frequency-tracking algorithm is proposed to realize effective measurement of vibration amplitude and frequency. Experiments are carried out on a precision vibrating table at different vibrating frequencies and different drive voltages, and reasonable results are achieved. Finally, taking the vibration with maximum amplitude as an example, the measurement results by laser Doppler interferometer are a frequency of 10 Hz and an amplitude of 72.25 μm, the measurement results by the optical sensor are a frequency of 10 Hz and an amplitude of 72.05 μm. Theoretical and experimental results show the feasibility of this system in harmonic vibration parameter measurements.
A study on experiment and simulation to predict the spring-back of SS400 steel sheet in large radius of V-bending process
Authors: Gia Hai Vuong , Thi Hong Minh Nguyen , Nguyen Duc Toan
Published: January 2020

The spring-back phenomenon is one of the key problems in sheet metal forming process, especially with a complex shape. In this paper, the experiments of spring back have been first carried out to study the effect of punch strokes (different bending angles) on the spring-back angle for 6 (mm) thickness of SS400 sheet steel by V-bending process. The experimental results are then validated by finite element analysis (FEA) of ABAQUS software. To simulate spring-back of V-bending, deformed results of V-shape from a dynamic forming simulation in Abaqus/Explicit would be imported into Abaqus/Standard, and then a static analysis will calculate the spring-back of V-bending. Simulation studies have shown that there are different variations in the initial bending angle and the spring-back angle after the removal of the bending force based on isotropic and kinematic hardening models. Therefore, the combined hardening model has been proposed to overcome the mismatch between prediction results and the corresponding experiments. Based on the accuracy of FEM simulation, this study also verifies the effect of parameters such as a thickness of the sheet, punch radius and punch stroke on the spring-back angle in order to select the optimum input parameters by using Taguchi method.
Thermomechanical behavior of graphene nanoplatelets and bamboo micro filler incorporated epoxy hybrid composites
Authors: Gouda Krushna , Bhowmik Sumit , Das Biplab
Published: January 2020

The present study is focused on the development of micro bamboo filler/epoxy hybrid composite with the incorporation of varied weight percentage of graphene nanoplatelet (GNPs). To check the effect of inclusion of dual fillers on the structural x-ray diffraction (XRD), morphological analysis by Scanning electron microscope (SEM) and thermomechanical analysis (TMA) are carried out. The micro bamboo and GNPs filler in the epoxy polymer are incorporated to eradicate the problem associated with natural and synthetic fibers alignment, delamination, and anisotropic property in the thermoset composite materials. Results revealed that with the inclusion of graphene nanoplatelet with bamboo filler in epoxy composite improves the synergetic effect, which in turn increases the tensile, flexural, loss modulus and storage modulus of developed hybrid composite material. SEM analysis confirmed the proper distribution of fillers and their presence from XRD analysis. All fabricated hybrid composite displayed improved thermal conductivity value and a marginal increase in the corrosion rate. The overall result predicts that the improvement is quite better compared to neat or solo bamboo filler based epoxy composite. The improvement is ascribed due to the proper interfacial bonding or cross-link between micro bamboo filler/epoxy polymer with the addition of GNPs. Developed filler based hybrid composite may be utilized for the application of thermal interface material, circuit board, electronic packaging, etc.
Influence of Ba and Mo co-doping on the structural, electrical, magnetic and optical properties of BiFeO3 ceramics
Authors: Sen Aungkan , Hasan Md Khalid , Islam Zahidul , Hassan Md. Rahat Al , Zaman Tasmia , Matin M A , Gulshan Fahmida
Published: January 2020

In this work,

Bi

0.8

Ba

0.2

Fe

1
−
x

Mo

x

O

3

(x = 0, 0.5, 0.10 and 0.15) ceramics were synthesized by conventional solid-state reaction to evaluate the influence of Ba2+ and Mo6+ co-doping on the structure, morphology, electrical, magnetic and optical properties of

BiFeO

3

ceramic. Rietveld refinement of x-ray diffraction data was done to obtain the subtle structural information. A tetragonal structure of P4mm type was revealed for

Bi

0.8

Ba

0.2

FeO

3

(x = 0) ceramic. Evolution of rhombohedral (R3c) phase was observed with Mo6+ doping and a complete transformation to R3c phase from P4mm was found for 15 wt% Mo6+ doping. This type of transformation causes distortion in the structure and results in changing bond angle. Magnetization was found to be improved with increasing the percentage of Mo6+ up to 10 wt%. Canting of spin due to the change in Fe-O-Fe bond angle is believed to be the main reason behind this improvement. One secondary phase

BaMoO

4

was found and becomes prominent with Mo6+ doping. Possible formation of this impurity and its correlation with properties are explained. Microstructural analysis was done to observe the Ba2+ and Mo6+ co-doping effect on grain size and distribution. A correlation of grain size with electric and magnetic properties is drawn and elucidated. Dielectric constant shows an increasing trend with Mo6+ doping. Reduction in oxygen vacancy, due to charge compensation upon high charged Mo6+ addition, believed to be the staple reason behind the dielectric constant increment. Lastly, optical band gap energy found to be decreased with the addition of Mo6+ and possible reasons behind this are evaluated. Overall, co-doping of Ba2+ and Mo6+ found to have a positive influence over materials electrical, optical as well as magnetic property.
Optimized foam filling configuration in bi-tubular crush boxes; a comprehensive experimental and numerical analysis
Authors: Salaripoor Hamidreza , Azimi Mohammadbagher B , Asgari Masoud
Published: January 2020

This study focuses on introducing optimized foam filled double-walled energy absorbers. To this end, different geometries and foam densities is proposed with different foam configuration including foam inside the inner wall or between the tubes. A comprehensive experimental study has been done via construction and testing different unique structures. By extracting experimental force-displacement plots of all the structures, crashworthiness behavior of the structures is studied. Based on the decision making algorithm of COPRAS the best configuration is obtained using

F

max

,

SEA

and

CFE

as the decision functions. Finite element model of some selected structures are developed in LS-DYNA explicit dynamic software and validated using test results. Based on the best found configuration a new set of FE models are analyzed to create a meta-model by employing Response Surface Methodology (RSM) and the thicknesses of inner and outer walls are optimized via a multi-objective optimization algorithm.
The Lindelöf hypothesis for primes is equivalent to the Riemann hypothesis
Authors: Gonek Steven , Graham Sidney , Lee Yoonbok
Published: January 2020

We recast the classical Lindelöf hypothesis as an estimate for the sums

∑

n
≤
x

n

−
i
t

\sum _{n\leq x}n^{-it}

. This leads us to propose that a more general form of the Lindelöf hypothesis may be true, one involving estimates for sums of the type

∑

n
≤
x

n
∈

N

n

−
i
t

,

\begin{equation*} \sum _{ \substack {n\leq x \\ n\in \mathscr {N} }}n^{-it}, \end{equation*}

where

N

\mathscr {N}

can be a quite general sequence of real numbers. We support this with several examples and show that when

N

=

P

\mathscr {N}=\mathbb {P}

, the sequence of prime numbers, the truth of our conjecture is equivalent to the Riemann hypothesis. Moreover, if our conjecture holds for

N

=

P

(
a
,
q
)

\mathscr {N}=\mathbb {P}(a, q)

, the primes congruent to

a

(
mod

q
)

a \pmod q

, with

a
a

coprime to

q
q

, then the Riemann hypothesis holds for all Dirichlet

L
L

-functions with characters modulo

q
q

, and conversely. These results suggest that a general form of the Lindelöf hypothesis may be both true and more fundamental than the classical Lindelöf hypothesis and the Riemann hypothesis.
Strong multiplicity one theorems for locally homogeneous spaces of compact-type
Authors: Lauret Emilio , Miatello Roberto
Published: January 2020

Let

G
G

be a compact connected semisimple Lie group, let

K
K

be a closed subgroup of

G
G

, let

Γ
\Gamma

be a finite subgroup of

G
G

, and let

τ
\tau

be a finite-dimensional representation of

K
K

. For

π
\pi

in the unitary dual

G
^

\widehat G

of

G
G

, denote by

n
Γ

(
π
)

n_\Gamma (\pi )

its multiplicity in

L
2

(
Γ
∖
G
)

L^2(\Gamma \backslash G)

.

We prove a strong multiplicity one theorem in the spirit of Bhagwat and Rajan, for the

n
Γ

(
π
)

n_\Gamma (\pi )

for

π
\pi

in the set

G
^

τ

\widehat G_\tau

of irreducible

τ
\tau

-spherical representations of

G
G

. More precisely, for

Γ
\Gamma

and

Γ
′

\Gamma ’

finite subgroups of

G
G

, we prove that if

n

Γ

(
π
)
=

n

Γ
′

(
π
)

n_{\Gamma }(\pi )= n_{\Gamma ’}(\pi )

for all but finitely many

π
∈

G
^

τ

\pi \in \widehat G_\tau

, then

Γ
\Gamma

and

Γ
′

\Gamma ’

are

τ
\tau

-representation equivalent, that is,

n

Γ

(
π
)
=

n

Γ
′

(
π
)

n_{\Gamma }(\pi )=n_{\Gamma ’}(\pi )

for all

π
∈

G
^

τ

\pi \in \widehat G_\tau

.

Moreover, when

G
^

τ

\widehat G_\tau

can be written as a finite union of strings of representations, we prove a finite version of the above result. For any finite subset

F
^

τ

\widehat {F}_{\tau }

of

G
^

τ

\widehat G_{\tau }

verifying some mild conditions, the values of the

n
Γ

(
π
)

n_\Gamma (\pi )

for

π
∈

F
^

τ

\pi \in \widehat F_{\tau }

determine the

n
Γ

(
π
)

n_\Gamma (\pi )

’s for all

π
∈

G
^

τ

\pi \in \widehat G_\tau

. In particular, for two finite subgroups

Γ
\Gamma

and

Γ
′

\Gamma ’

of

G
G

, if

n
Γ

(
π
)
=

n

Γ
′

(
π
)

n_\Gamma (\pi ) = n_{\Gamma ’}(\pi )

for all

π
∈

F
^

τ

\pi \in \widehat F_{\tau }

, then the equality holds for every

π
∈

G
^

τ

\pi \in \widehat G_\tau

. We use algebraic methods involving generating functions and some facts from the representation theory of

G
G

.
Strichartz estimates for the one-dimensional wave equation
Authors: Donninger Roland , Glogić Irfan
Published: January 2020

We study the hyperboloidal initial value problem for the one-dimensional wave equation perturbed by a smooth potential. We show that the evolution decomposes into a finite-dimensional spectral part and an infinite-dimensional radiation part. For the radiation part we prove a set of Strichartz estimates. As an application we study the long-time asymptotics of Yang-Mills fields on a wormhole spacetime.
Experimental animal models of coronary microvascular dysfunction
Authors: Sorop Oana , van de Wouw Jens , Chandler Selena , Ohanyan Vahagn , Tune Johnathan D , Chilian William M , Merkus Daphne , Bender Shawn B , Duncker Dirk J
Published: January 2020

Coronary microvascular dysfunction (CMD) is commonly present in patients with metabolic derangements and is increasingly recognized as an important contributor to myocardial ischaemia, both in the presence and absence of epicardial coronary atherosclerosis. The latter condition is termed ‘ischaemia and no obstructive coronary artery disease’ (INOCA). Notwithstanding the high prevalence of INOCA, effective treatment remains elusive. Although to date there is no animal model for INOCA, animal models of CMD, one of the hallmarks of INOCA, offer excellent test models for enhancing our understanding of the pathophysiology of CMD and for investigating novel therapies. This article presents an overview of currently available experimental models of CMD—with an emphasis on metabolic derangements as risk factors—in dogs, swine, rabbits, rats, and mice. In all available animal models, metabolic derangements are most often induced by a high-fat diet (HFD) and/or diabetes mellitus via injection of alloxan or streptozotocin, but there is also a wide variety of spontaneous as well as transgenic animal models which develop metabolic derangements. Depending on the number, severity, and duration of exposure to risk factors—all these animal models show perturbations in coronary microvascular (endothelial) function and structure, similar to what has been observed in patients with INOCA and comorbid conditions. The use of these animal models will be instrumental in identifying novel therapeutic targets and for the subsequent development and testing of novel therapeutic interventions to combat ischaemic heart disease, the number one cause of death worldwide.
Emergent TandemHeart-ECMO for acute severe mitral regurgitation with cardiogenic shock and hypoxaemia: a case series
Authors: DiVita Michael , Visveswaran Gautam K , Makam Kasaiah , Naji Peyman , Cohen Marc , Kapoor Saurabh , Saunders Craig R , Zucker Mark J
Published: January 2020

Background 

Acute severe mitral regurgitation (MR) associated with cardiogenic shock is a life-threatening emergency. Traditional teaching has focused on the need for emergent coronary angiography and/or intra-aortic balloon counterpulsation in preparation for emergent open-heart surgery for repair/replacement. Unfortunately, emergent open-heart surgery in patients with acute MR complicated by cardiogenic shock is associated with 25–46% perioperative mortality. New devices have provided additional options for stabilization prior to emergent surgery which facilitate improved outcomes.

Case summary 

We present two cases of acute severe MR resulting in cardiogenic shock and profound hypoxaemia. TandemHeart® mechanical circulatory support with an oxygenator spliced into the circuit, akin to veno-arterial extracorporeal membrane oxygenation (ECMO), facilitated haemodynamic stabilization and decongestion of the lungs facilitating successful bridge to mitral valve surgery. Successful discharge to home was achieved in both patients with good neurological outcomes and sustained long-term functional recovery at 18 and 14 months, respectively.

Discussion

Selective use of the TandemHeart®, with or without ECMO, facilitates management of the critically ill cardiogenic shock patient with acute severe MR.