bluuebunny/crossref_metadata_2025_split huggingface dataset:
Lysosomal degradation of GMPPB is associated with limb‐girdle muscular dystrophy type 2T
Authors: Tian Wo‐Tu , Zhou Hai‐Yan , Zhan Fei‐Xia , Zhu Ze‐Yu , Yang Jie , Chen Sheng‐Di , Luan Xing‐Hua , Cao Li 
Published: May 2019

AbstractObjectiveGDP‐mannose pyrophosphorylase B (GMPPB) related phenotype spectrum ranges widely from congenital myasthenic syndrome (CMS), limb‐girdle muscular dystrophy type 2T (LGMD 2T) to severe congenital muscle‐eye‐brain syndrome. Our study investigates the clinicopathologic features of a patient with novel GMPPB mutations and explores the pathogenetic mechanism.MethodsThe patient was a 22‐year‐old woman with chronic proximal limb weakness for 9 years without cognitive deterioration. Weakness became worse after fatigue. Elevated serum creatine kinase and decrements on repetitive nerve stimulation test were recorded. MRI showed fatty infiltration in muscles of lower limbs and shoulder girdle on T1 sequence. Open muscle biopsy and genetic analysis were performed.ResultsMuscle biopsy showed myogenic changes. Two missense mutations in GMPPB gene (c.803T>C and c.1060G>A) were identified in the patient. Western blotting and immunostaining showed GMPPB and α‐dystroglycan deficiency in the patient's muscle. In vitro, mutant GMPPB forming cytoplasmic aggregates completely colocalized with microtubule‐associated protein 1 light chain 3‐II (LC3‐II), a classical marker of autophagosome. Degradation of GMPPB was accompanied by an upregulation of LC3‐II, which could be restored by lysosomal inhibitor leupeptin.InterpretationWe identified two novel GMPPB mutations causing overlap phenotype between LGMD 2T and CMS. We provided the initial evidence that mutant GMPPB colocalizes with autophagosome at subcellular level. GMPPB mutants degraded by autophagy‐lysosome pathway is associated with LGMD 2T. This study shed the light into the enzyme replacement which could become one of the therapeutic targets in the future study.
Blood Pressure Trends in Patients With Seropositive Rheumatoid Arthritis Compared With Controls Without Rheumatoid Arthritis: A Retrospective Cohort Study
Authors: Boersma Peter , McElwee Matthew K. , Hashmi Hiba , Schreiner Pamela , Demmer Ryan T. , Shmagel Anna 
Published: May 2019

ObjectiveRheumatoid arthritis (RA) conveys an increased risk of cardiovascular disease (CVD), making it imperative that traditional CVD risk factors are well controlled. This study compared blood pressure (BP) trends over 13 years among patients with seropositive RA and patients without RA who received care within a large health care system in Minnesota.MethodsThis retrospective cohort study compared 774 patients with seropositive RA and 3254 patients without RA who were matched on sex and year of birth (±5 years) and observed between 2005 and 2017. Generalized estimating equation models were used for longitudinal analyses, with adjustment for demographics, body mass index, smoking status, Charlson Comorbidity Index, number of BP measurements, and number of antihypertensive and oral glucocorticoid medications.ResultsPatients both with and without RA had a mean age of 55 and were predominately female (78% with RA; 79% without RA). The mean follow‐up was 6.3 (SD 3.4) years for patients with RA and 7.2 (SD 3.3) years for patients without RA. Overall, systolic BP, diastolic BP, and the number of prescribed antihypertensive medications did not differ between groups. Patients with RA were more likely to be current smokers compared with patients without RA (23% vs 11%; P < 0.01) and were less likely to have serum lipid measurements (75% vs 85%; P < 0.01).ConclusionBP was similarly controlled in patients with seropositive RA and patients without RA. However, diastolic BP in patients with RA was trending up in most recent years. Patients with RA were also more likely to smoke compared with controls and were less likely to have serum lipid measurements.
Temperature‐ and Light‐Regulated Gas Transport in a Liquid Crystal Polymer Network
Authors: Cao Anping , van Raak Roel J. H. , Pan Xinglong , Broer Dirk J. 
Published: May 2019

AbstractAzobenzene‐containing liquid crystal polymer networks (LCNs) are developed for temperature‐ and light‐regulated gas permeation. The order in a chiral‐nematic LCN (LCN*) is found to be essential to couple the unique structure of the membrane and its gas permeation responses to external stimuli such as temperature and varying irradiation conditions. An LCN membrane polymerized in the isotropic phase exhibits enhanced N2 permeation with increasing temperature, like most traditional polymers, but barely responds to exposure with 455 and 365 nm light. In sharp contrast, a reversible decrease of N2 transport is observed for the LCN* membrane of exactly the same chemical composition, but molecularly ordered, when submitted to an elevated temperature. More importantly, alternating in situ illumination with 455 and 365 nm light modulates reversibly N2 permeation performance of the LCN* membrane, through the trans–cis isomerization of azo moieties. The authors postulate that, besides the anisotropic deformation of LCN*, the decreased order in LCN* membrane caused by external stimuli (i.e., increasing temperature or UV light illumination) is responsible for an inhibition of gas permeation. These results show potential applications of liquid crystal polymers in the gas transport and separation, and also contribute to the development of “smart” membranes.
Compositional and Dimensional Control of 2D and Quasi‐2D Lead Halide Perovskites in Water
Authors: Jana Atanu , Ba Qiankai , Kim Kwang S. 
Published: May 2019

AbstractBlue light emitting two dimensional (2D) and quasi‐2D layered halide perovskites (LHPs) are gaining attention in solid‐state lighting applications but their fragile stability in humid condition is one of the most pressing issues for their practical applications. Though water is much greener and cost effective, organic solvents must be used during synthesis as well as the device fabrication process for these LHPs due to their water‐sensitivity/instability and consequently, water‐stable blue‐light emitting 2D and quasi‐2D LHPs have not been documented yet. Here, water‐mediated facile and cost‐effective syntheses, characterizations, and optical properties of 16 organic–inorganic hybrid compounds are reported including 2D (A′)2PbX4 (A′ = butylammonium, X = Cl/Br/I) (8 compounds), 3D perovskites (4), and quasi‐2D (A′)pAx−1BxX3x+1 LHPs (A = methylammonium) (4) in water. Here, both composition and dimension of LHPs are tuned in water, which has never been explored yet. Furthermore, the dual emissive nature is observed in quasi‐2D perovskites, where the intensity of two photoluminescence (PL) peaks are governed by 2D and 3D inorganic layers. The Pb(OH)2‐coated 2D and quasi‐2D perovskites are highly stable in water even after several months. In addition, single particle imaging is performed to correlate structural–optical property of these LHPs.
Defect Engineering in Two Common Types of Dielectric Materials for Electromagnetic Absorption Applications
Authors: Quan Bin , Shi Wenhao , Ong Samuel Jun Hoong , Lu Xiaochi , Wang Paul Luyuan , Ji Guangbin , Guo Yufeng , Zheng Lirong , Xu Zhichuan J. 
Published: May 2019

AbstractDielectric materials are greatly desired for electromagnetic absorption applications. Lots of research shows that conduction loss and polarization are two of the most important factors determining complex permittivity. However, the detailed dissipation mechanisms for the improved microwave absorption performance are often based on semiempirical rules, lacking practical data relationships between conduction loss/polarization and dielectric behaviors. Here, a strategy of introducing point defects is used to understand such underlying relationships, where polarizability and conductivity are adjustable by manipulating oxygen deficiency or heteroatoms. Based on first principles calculations and the applied oxygen‐deficient strategy, dielectric polarization is shown to be dominant in determining the permittivity behaviors in semiconductors. Meanwhile, the presented nitrogen doping strategy shows that conduction loss is dominant in determining the permittivity behavior in graphitized carbon materials. The validity of the methods for using point defects to explore the underlying relations between conduction loss/polarization and dielectric behaviors in semiconductor and graphitized carbon are demonstrated for the first time, which are of great importance in optimizing the microwave absorption performance by defect engineering and electronic structure tailoring.
Treefrog Toe Pad‐Inspired Micropatterning for High‐Power Triboelectric Nanogenerator
Authors: Bui Van‐Tien , Zhou Qitao , Kim Jong‐Nam , Oh Jung‐Hwan , Han Kwan‐Woo , Choi Ho‐Suk , Kim Sang‐Woo , Oh Il‐Kwon 
Published: May 2019

AbstractInspired by treefrog's toe pads that show superior frictional properties, herein, an industrially compatible approach is reported to make an efficient dielectric tribosurface design using customizable nonclose‐packed microbead arrays, mimicking the friction pads of treefrogs, in order to significantly enhance electrification performance and reliability of triboelectric nanogenerator (TENG). The approach involves using an engineering polymer to prepare a highly ordered large‐area concave film, and subsequently the molding of a convex patterned triboreplica in which the concave film is exploited as a reusable master mold. A nature‐inspired TENG based on the patterned polydimethylsiloxane (PDMS) paired with flat aluminum (Al) can generate a relatively high power density of 8.1 W m−2 even if a very small force of ≈6.5 N is applied. Moreover, the convex patterned PDMS‐based TENG possesses exceptional durability and reliability over 25 000 cycles of contact–separation. Considering the significant improvements in power generation of TENG; particularly at very small force, together with cost‐effectiveness and possibility of mass production, the present methodology may pave the way for large‐scale blue energy harvesting and commercialization of TENGs for many practical applications.
Photoelectric Synaptic Plasticity Realized by 2D Perovskite
Authors: Sun Yilin , Qian Liu , Xie Dan , Lin Yuxuan , Sun Mengxing , Li Weiwei , Ding Liming , Ren Tianling , Palacios Tomás 
Published: May 2019

AbstractRecently, several light‐stimulated artificial synaptic devices have been proposed to mimic photonic synaptic plasticity for neuromorphic computing. Here, the photoelectric synaptic plasticity based on 2D lead‐free perovskite ((PEA)2SnI4) is demonstrated. The devices show a photocurrent activation in response to a light stimulus in a neuron‐like way and exhibit several essential synaptic functions such as short‐term plasticity (STP) and long‐term plasticity (LTP) as well as their transmission based on spike frequency control. The strength of synaptic connectivity can be effectively modulated by the duration, irradiance, and wavelength of light spikes. The ternary structure of (PEA)2SnI4 causes it to possess varied photoelectric properties by composition control, which enhances the complexity and freedoms required by neuromorphic computing. The physical mechanisms of the memory effect are attributed to two distinct lifetimes of photogenerated carrier trapping/detrapping processes modulated by controlling the proportion of Sn vacancies. This work demonstrates the great potential of (PEA)2SnI4 as a platform to develop future multifunctional artificial neuromorphic systems.
Printing Therapeutic Proteins in 3D using Nanoengineered Bioink to Control and Direct Cell Migration
Authors: Peak Charles W. , Singh Kanwar Abhay , Adlouni Mu'ath , Chen Jeffrey , Gaharwar Akhilesh K. 
Published: May 2019

AbstractA nanoengineered bioink loaded with therapeutic proteins is designed to direct cell function in a 3D printed construct. The bioink is developed from a hydrolytically degradable polymer and 2D synthetic nanoparticle. The synthesis of poly(ethylene glycol)‐dithiothreitol (PEGDTT) via a Michael‐like step growth polymerization results in acrylate terminated degradable macromer. The addition of 2D nanosilicates to PEGDTT results in formation of shear‐thinning bioinks with high printability and structural fidelity. The mechanical properties, swelling kinetics, and degradation rate of 3D printed constructs can be modulated by changing the ratio of PEG:PEGDTT and nanosilicates concentration. Due to high surface area and charged characteristic of nanosilicates, protein therapeutics can be sequestered in 3D printing structure for prolong duration. Sustained release of pro‐angiogenic therapeutics from 3D printed structure, promoted rapid migration of human endothelial umbilical vein cell. This approach to design biologically active inks to control and direct cell behavior can be used to engineer 3D complex tissue structure for regenerative medicine.
Grain Boundary Engineering Nanostructured SrTiO3 for Thermoelectric Applications
Authors: Dylla Maxwell T. , Kuo Jimmy Jiahong , Witting Ian , Snyder Gerald Jeffrey 
Published: May 2019

AbstractNanostructuring to reduce thermal conductivity is among the most promising strategies for designing next‐generation, high‐performance thermoelectric materials. In practice, electrical grain boundary resistance can overwhelm the thermal conductivity reduction induced by nanostructuring, which results in worse overall performance. Since a large body of work has characterized the transport of both polycrystalline ceramics and single crystals of SrTiO3, it is an ideal material system for conducting a case study of electrical grain boundary resistance. An effective mass model is used to characterize the transport signatures of electrical grain boundary resistance and evaluate thermodynamic design principles for controlling that resistance. Treating the grain boundary as a secondary phase to the bulk crystallites explains the transport phenomena. Considering that the interface can be engineered by controlling oxygen partial pressure, temperature, and the addition of extrinsic elements into the grain boundary phase, the outlook for SrTiO3 as a nanostructured thermoelectric is promising, and the zT could be greater than 0.5 at room temperature.
MnO/Metal/Carbon Nanohybrid Lithium‐Ion Battery Anode With Enhanced Electrochemical Performance: Universal Facile Scalable Synthesis and Fundamental Understanding
Authors: Wang Xiaoyan , Ma Liujia , Ji Qing , Meng Jian‐Qiang , Liang Suzhe , Xu Zhuijun , Wang Meimei , Zuo Xiuxia , Xiao Ying , Zhu Jin , Xia Yonggao , Müller‐Buschbaum Peter , Cheng Ya‐Jun 
Published: May 2019

AbstractMnO holds a great promise as an alternative lithium‐ion battery anode. It is crucial to improve the cyclic stability and rate capability of MnO‐based anodes. A facile scalable strategy to incorporate metal nanoparticles into the MnO/carbon anodes is developed as demonstrated by the MnO/Ag/C and MnO/Ni/C nanohybrids. Difunctional methacrylate monomers are used as solvent and carbon source, where the precursors of MnO and metal are homogeneously mixed at the molecular level and converted into a thermosetting polymer. MnO and metal nanoparticles are in situ formed and homogeneously embedded in the in situ formed carbon matrix after the carbonization process. The influence of the metal nanoparticles on the structure and properties of the MnO‐based anodes is systematically investigated. The mass composition of the MnO phase within the nanohybrid is controlled to be at a relatively low level, which is helpful for maintaining a good cyclic stability at the expense of the reversible capacities. However, the reversible capacities are increased by the incorporation of the metal nanoparticles due to enhanced electrochemical kinetics, where both excellent cyclic stability and rate performance are exhibited simultaneously. The mechanism responsible for the performance improvement is explored by electrochemical impedance spectroscopy, cyclic voltammetry, and temperature‐dependent resistivity measurements.
Multicolor Semiconductor Lasers
Authors: Zhuang Xiujuan , Ouyang Yu , Wang Xiaoxia , Pan Anlian 
Published: May 2019

AbstractThe ever‐increasing demand for information density and accuracy in highly integrated photonic devices calls for miniaturization of a coherent light source with broadband tunability and high stability. Wavelength tuning range, cavity Q factor, and threshold are three indicators of evaluating the performance of a nanoscale laser. Herein, the latest, effective materials and fundamental structures for multicolor semiconductor lasers including low dimensional II–VI semiconductors, III–V semiconductors, and lead halide perovskites are reviewed. Composition and bandgaps, novel cavity designs, lasing wavelength, Q factors of resonators, and lasing thresholds of the multicolor semiconductor lasers are compared and discussed. Finally, a summary and a future perspective of broadband tunable semiconductor lasers are given.
Arsenic Trioxide Impacts Viral Latency and Delays Viral Rebound after Termination of ART in Chronically SIV‐Infected Macaques
Authors: Yang Qing , Feng Fengling , Li Pingchao , Pan Enxiang , Wu Chunxiu , He Yizi , Zhang Fan , Zhao Jin , Li Ruiting , Feng Liqiang , Hu Fengyu , Li Linghua , Zou Huachun , Cai Weiping , Lehner Thomas , Sun Caijun , Chen Ling 
Published: May 2019

AbstractThe latent viral reservoir is the source of viral rebound after interruption of antiretroviral therapy (ART) and is the major obstacle in eradicating the latent human immunodeficiency virus‐1 (HIV‐1). In this study, arsenic class of mineral, arsenic trioxide, clinically approved for treating acute promyelocytic leukemia, is demonstrated to reactivate latent provirus in CD4+ T cells from HIV‐1 patients and Simian immunodeficiency virus (SIV)‐infected macaques, without significant systemic T cell activation and inflammatory responses. In a proof‐of‐concept study using chronically SIVmac239‐infected macaques, arsenic trioxide combined with ART delays viral rebound after ART termination, reduces the integrated SIV DNA copies in CD4+ T cells, and restores CD4+ T cells counts in vivo. Most importantly, half of arsenic trioxide‐treated macaques show no detectable viral rebound in the plasma for at least 80 days after ART discontinuation. Mechanistically, the study reveals that CD4 receptors and CCR5 co‐receptors of CD4+ T cells are significantly downregulated by arsenic trioxide treatment, which reduces susceptibility to infection after provirus reactivation. Furthermore, an increase in SIV‐specific immune responses after arsenic trioxide treatment may contribute to suppression of viral rebound. This work suggests that arsenic trioxide in combination with ART is a novel regimen in down‐sizing or even eradicating latent HIV‐1 reservoir.
Mesoporous Decoration of Freestanding Palladium Nanotube Arrays Boosts the Electrocatalysis Capabilities toward Formic Acid and Formate Oxidation
Authors: Ding Jia , Liu Zhi , Liu Xiaorui , Liu Jie , Deng Yida , Han Xiaopeng , Zhong Cheng , Hu Wenbin 
Published: May 2019

AbstractFabricating high‐performance electrocatalysts is the most critical step in commercializing direct formic acid or formate fuel cells. In this work, a dual‐template electrodeposition method is used to create freestanding mesoporosity decorated palladium nanotube arrays (P‐PdNTA) as a bifunctional electrocatalyst toward formic acid and formate oxidation (FAO/FOR). The phytantriol‐based soft template modifies the superficial chemistry of aluminum anodic oxide inner surfaces, thereby facilitating the regulated electrodeposition of highly stable palladium nanotubes. The sacrifice of the soft template generates substantial mesoporosity on the nanotubes, resulting in a 189% increase in the electrochemically active surface area with respect to the mesopore‐free PdNTA baseline. In addition, the soft template significantly increases the density of catalytically active sites per unit area via perturbation on routine nanotube growth, as evidenced by the doubled areal catalytic activity of P‐PdNTA versus PdNTA. Remarkably, the P‐PdNTA delivered gravimetric catalytic currents of 3.65 and 3.87 A mg−1 for FAO and FOR, which are 8.5 and 6.5 times higher, respectively, than those of commercial Pt/C. These values are among the most favorable reported and benefit from the unique synergy of fast substance transport, large electrochemical active surface area and high areal population of catalytically active sites.
Spatiotemporal distribution of individuals as an indicator for the social system of Lepilemur sahamalaza
Authors: Mandl Isabella , Holderied Marc , Schwitzer Christoph 
Published: May 2019

AbstractPrimate social systems are highly diverse, complicating the classification of particularly elusive species that are difficult to observe. The spatial distribution of individuals over time is a critica lindicator for the social organization and long‐term studies are important to establish patterns of social interactions. In recent years, species of the cryptic, nocturnal sportive lemurs of the genus Lepilemur were found to live in pairs in which a single male and a single female share and defend a mutual home range. The present study aimed to forward research into this underrepresented genus by determining the social organization and structure of the Sahamalaza sportive lemur, L. sahamalaza. We collected 773.15 hr of behavioral and GPS data during a period of 10 months (between 2015 and 2016) on 14 individuals: eight females and six males. There was no evidence of pair‐specific home range use as intra‐ and intersexual home range overlap was high. No pattern of social interactions between focal individuals could be distinguished despite high range overlap. Individuals met and interacted infrequently, resulting in an interaction rate of 0.32 interactions/hr. Sleeping associations between adult individuals were never observed. While both sexes had access to multiple potential mating partners, range sizes or ranging distances did not increase in mating periods. Overall, the social system of Sahamalaza sportive lemurs exhibits aspects of a solitary social organization and structure with potential for the polygamous mating system. These findings underline the importance of detailed social ecology studies that can provide the basis for understanding potential environmental influences on social system variability of closely related species.
Combined Alkali‐Organoammonium Structure Direction of High‐Charge‐Density Heteroatom‐Containing Aluminophosphate Molecular Sieves
Authors: Seo Seungwan , Ahn Nak Ho , Lee Jeong Hwan , Knight Lisa M. , Moscoso Jaime G. , Sinkler Wharton A. , Prabhakar Sesh , Nicholas Christopher P. , Hong Suk Bong , Lewis Gregory J. 
Published: May 2019

AbstractThe charge density mismatch concept was applied to the synthesis of high‐charge‐density silicoaluminophosphate SAPO‐69 (OFF) and SAPO‐79 (ERI) and zincoaluminophosphate PST‐16 (CGS), PST‐17 (BPH), PST‐19 (SBS), and ZnAPO‐88 (MER) molecular sieves. Combined alkali‐organoammonium structure direction in these systems is thus enabled. Structure direction is treated from the perspective of stabilizing an ionic framework, the relationships between reaction charge density (OH−/H3PO4), alkali and organoammonium content, and ionicity of tetrahedral framework atoms in successful structure direction are presented.