bluuebunny/crossref_metadata_2025_split huggingface dataset:
Beclin1 Modulates Bone Homeostasis by Regulating Osteoclast and Chondrocyte Differentiation
Authors: Arai Atsushi , Kim Sol , Goldshteyn Vadim , Kim Terresa , Park No-Hee , Wang Cun-Yu , Kim Reuben H. 
Published: May 2019

ABSTRACT
Autophagy (ATG), an important cellular recycling process whereby macromolecules or organelles are encapsulated by autophagosome and degraded upon merging with lysosome, has recently been shown to play an essential role in bone biology. However, the involvement of ATG in bone and bone-related cells remains unclear. Here, we show that Beclin1, an ATG-related protein involved in ATG initiation, plays a pivotal role in osteoclasts. ATG was activated during osteoclast differentiation in vitro. Beclin1 was enhanced and required for osteoclast differentiation. Mechanistically, we found that TRAF6-mediated ubiquitination of Beclin1 at K117, but not ULK1-mediated phosphorylation, is required for RANKL-stimulated osteoclast differentiation. In vivo, mice lacking Beclin1 in CstK-expressing cells exhibited an increased cortical bone thickness caused by impaired osteoclasts’ function. Interestingly, these mice also exhibited diminished trabecular bone mass, which was associated with a defect in cartilage formation and chondrocyte differentiation. Collectively, our study highlights the functional importance of ATG in osteoclasts and chondrocytes, and identifies ATG as a potential therapeutic target for managing bone-related diseases. © 2019 American Society for Bone and Mineral Research.
The CtBP1–p300–FOXO3a transcriptional complex represses the expression of the apoptotic regulators Bax and Bim in human osteosarcoma cells
Authors: Li Chen , Xiao Xiao‐qing , Qian Yi‐hong , Zhou Zhi‐yong 
Published: May 2019

AbstractC‐terminal binding protein 1 (CtBP1), a well‐known transcriptional corepressor, functions as an oncogene in multiple cancer types, including osteosarcoma, by modulating the transcription of many tumor suppressors, such as cadherin 1 (CDH1), phosphatase and tensin homolog (PTEN), Bcl2‐associated X (Bax), Bcl‐2‐interacting mediator (Bim), and cyclin‐dependent kinase inhibitor 1A (CDKN1A). However, it is still unclear how CtBP1 regulates the expression of these downstream targets. Here, we identified that CtBP1 is overexpressed in osteosarcoma cells and found that CtBP1 directly interacts with the transcription factor forkhead box O3 (FOXO3a) and the histone acetyltransferase p300 in vivo and in vitro. Through microarray analysis, we found that CtBP1 negatively regulates FOXO3a levels. In contrast to the CtBP1 level, the FOXO3a expression level was found to be significantly reduced in osteosarcoma cells. Knockdown of CtBP1 or overexpression of FOXO3a in U2OS cells resulted in different gene expression patterns, and the former caused upregulation of CtBP1 downstream target genes such as CDH1, PTEN, Bax, Bim, and CDKN1A, whereas the latter caused upregulation of Bax and Bim, but not CDH1, PTEN, and CDKN1A. Further analysis indicated that the CtBP1–p300–FOXO3a transcriptional complex specifically binds to the promoters of Bax and Bim. Inhibition of CtBP1 by the constitutive expression of Pep1‐E1AWT peptide in U2OS and OSA cells reversed oncogenic phenotypes, including colony formation, cellular proliferation, and migration, and limited tumor growth in vivo. Together our results demonstrated that the CtBP1–p300–FOXO3a transcriptional complex represses the expression of the apoptotic regulators Bax and Bim in human osteosarcoma cells and that targeting CtBP1‐mediated transcriptional events might be a potential therapeutic strategy for the osteosarcoma treatment.
Effects of overwintering temperatures on the escape response in cunner (Tautogolabrus adspersus)
Authors: Moran Clinton J. , Young Caroline M. , Gerry Shannon P. 
Published: May 2019

AbstractCunner (Tautogolabrus adspersus) are a temperate labrid species that inhabit the Western Atlantic and experience temperatures ranging from 0°C to 25°C. During autumn, once temperatures drop below 10°C in Long Island Sound, cunner find shelter and enter a state of quiescence. Previous work has shown that acclimation to low temperatures limits the performance of locomotor musculature, which significantly lowers steady swimming capabilities. We aimed to understand how the escape response (C‐start) might be impacted by temperatures experienced by cunner in Long Island Sound over the course of a year. Escape responses were recorded at 250 frames/s at 20°C, 15°C, 10°C, and 5°C. Average peak velocities and accelerations were faster in fish acclimated to 20°C than to 5°C and 10°C. Despite taking a similar turn angle to 10°C and 15°C fish, the 5°C treatment group took longer to complete the C‐start, which might make them more susceptible to predation at this temperature. Based on these results it appears that the escape response is reduced at cold temperatures. Previous research has shown that locomotor musculature performance is significantly reduced at cold temperatures, which could explain the results seen here. The decrease in escape performance at cold temperatures could explain their state of extended torpor as the slowed C‐start at these cold temperatures might make them more susceptible to predation.
A novel mutation in VPS33B gene causing a milder ARC syndrome phenotype with prolonged survival
Authors: del Brío Castillo Rodrigo , Squires James E. , McKiernan Patrick J. 
Published: May 2019

AbstractIntroductionARC (arthrogryposis, renal dysfunction, and cholestasis) syndrome is an uncommon multisystem disorder that entails a very poor prognosis. It is caused by mutations in either VPS33B or VIPAS39 gene, both playing a key role in intracellular trafficking. We report two siblings born to first cousin parents with a novel mutation in VPS33B who have both shown prolonged survival.Cases PresentationThe index patient presented with bilateral hip dysplasia and arthrogryposis, failure to thrive, undernourishment, developmental delay, and low gamma‐glutamyl transferase cholestasis. She at age 2 years underwent external biliary diversion with improvement in pruritus but liver disease continued to progress. She developed stomal bleeding at 7 years of age and liver biopsy displayed cirrhosis. Her 3‐year‐old sibling showed a similar trajectory as well as he had ichthyotic skin with excoriations. Their renal involvement was mild and stable. Genetic analysis in both patients revealed a novel homozygous mutation in NM_018668.4 (VPS33B):c.1157A > C (p.His386Pro).ConclusionsARC syndrome is a severe disorder with few patients reported to survive beyond 12 months of age. This report discloses a novel mutation in the VPS33B gene and describes a phenotype with prolonged survival, mild renal involvement, and progressive liver disease.
Glycogen storage disease presenting as Cushing syndrome
Authors: Stefater Margaret A. , Wolfsdorf Joseph I. , Ma Nina S. , Majzoub Joseph A. 
Published: May 2019

AbstractImpaired growth is common in patients with glycogen storage disease (GSD), who also may have “cherubic” facies similar to the “moon” facies of Cushing syndrome (CS). An infant presented with moon facies, growth failure, and obesity. Laboratory evaluation of the hypothalamic‐pituitary‐adrenal (HPA) axis was consistent with CS. He was subsequently found to have liver disease, hypoglycemia, and a pathogenic variant in PHKA2, leading to the diagnosis of GSD type IXa. The cushingoid appearance, poor linear growth and hypercortisolemia improved after treatment to prevent recurrent hypoglycemia. We suspect this child's HPA axis activation was “appropriate” and caused by chronic hypoglycemic stress, leading to increased glucocorticoid secretion that may have contributed to his poor growth and excessive weight gain. This is in contrast to typical CS, which is due to excessive adrenocorticotropic hormone (ACTH) or cortisol secretion from neoplastic pituitary or adrenal glands, ectopic secretion of ACTH or corticotropin‐releasing hormone (CRH), or exogenous administration of corticosteroid or ACTH. Pseudo‐CS is a third cause of excessive glucocorticoid secretion, has no HPA axis pathology, is most often associated with underlying psychiatric disorders or obesity in children and, by itself, is thought to be benign. We speculate that some diseases, including chronic hypoglycemic disorders such as the GSDs, may have biochemical features and pathologic consequences of CS. We propose that excessive glucocorticoid secretion due to chronic stress be termed “stress‐induced Cushing (SIC) syndrome” to distinguish it from the other causes of CS and pseudo‐CS, and that evaluation of children with chronic hypoglycemia and poor statural growth include evaluation for CS.
Fabrication of flexible paper‐based Surface‐enhanced Raman scattering substrate from Au nanocubes monolayer for trace detection of crystal violet on shell
Authors: Lin Shuang , Lin Xiang , Song Xueying , Han Siqingaowa , Wang Li , Hasi Wuliji 
Published: May 2019

AbstractSurface‐enhanced Raman scattering (SERS) substrate is the key to SERS technology and it is still a basic focus to fabricate desirable SERS substrates offering multiple advantages of strong Raman enhancement and good reproducibility and portability. A flexible paper‐based SERS substrate were fabricated involving the use of Au nanocubes (NCs) monolayer. Au NCs were generated with tunable size in the range of 24 to 63 nm through seed‐mediated growth method. The effect of support on SERS properties of Au NCs–assembled monolayer was systemically explored. As a result, more excellent SERS performance could be obtained by assembling Au NCs on the filter paper. Besides, the influence of Au NCs' size on SERS‐enhanced effect of paper‐based substrate was also investigated. Our results clearly indicated that paper‐based substrate with 39 nm Au NCs possessed the optimal SERS enhancement. This SERS substrate exhibited high sensitivity with enhancement factor (EF) up to 9.75 × 107 and demonstrated good uniformity and time stability. Furthermore, based on this superior paper‐based SERS substrate with advantage of powerful sample extraction performance, the measurement of crystal violet (CV) on shell surface were successfully carried out with detection limit down to 2.17 × 10−7 M. And linear quantification were achieved in the range from 5 × 10−5 to 5 × 10−7 M. To sum up, the as‐prepared SERS substrate is applicable for on‐site detection and analysis of CV on various shell surfaces.
Fabric phase sorptive extraction/GC‐MS method for rapid determination of broad polarity spectrum multi‐class emerging pollutants in various aqueous samples
Authors: Kaur Ramandeep , Kaur Ripneel , Grover Aman , Rani Susheela , Malik Ashok Kumar , Kabir Abuzar , Furton Kenneth G. 
Published: May 2019

AbstractA rapid extraction and cleanup method using selective fabric phase sorptive extraction combined with gas chromatography and mass spectrometry has been developed and validated for the determination of broad polarity spectrum emerging pollutants, ethyl paraben, butyl paraben, diethyl phthalate, dibutyl phthalate, lidocaine, prilocaine, triclosan, and bisphenol A in various aqueous samples. Some important parameters of fabric phase sorptive extraction such as extraction time, matrix pH, stirring speed, type and volume of desorption solvent were investigated and optimized. Calibration curves were obtained in the concentration range 0.05–500 ng/mL. Under the optimum conditions, the limits of detection were in the range 0.009 –0.021 ng/mL. This method was validated by analyzing the compounds in spiked aqueous samples at different levels with recoveries of 93 to 99% and relative standard deviations of <6%. The developed method was applied for the determination of the emerging contaminants in tap water, municipal water, ground water, sewage water, and sludge water samples. The results demonstrate that fabric phase sorptive extraction has great potential in the preconcentration of trace analytes in complex matrix.
Functional Modules of Pharyngeal Swallowing Mechanics
Authors: Hosseini Pouria , Tadavarthi Yasasvi , Martin‐Harris Bonnie , Pearson William G. 
Published: May 2019

ObjectivesThe present retrospective cohort study aims to test the hypothesis that elements of swallowing mechanics including hyoid movement, laryngeal elevation, tongue base retraction, pharyngeal shortening, pharyngeal constriction, and head and neck extension can be grouped into functional modules, and that these modules are predictably altered in disease states.MethodsModified barium swallow video clips of a thick and a thin liquid swallow from 40 normal patients and 10 dysphagic post‐treatment oropharyngeal head‐and‐neck cancer (HNC) patients were used in this study. Coordinate locations of 12 anatomical landmarks mapping pharyngeal swallowing mechanics were tracked on every frame during the pharyngeal phase of each swallow using a custom‐made MATLAB tool. Morphometric modularity hypothesis testing was performed on these coordinate data to characterize the modular elements of swallowing function in each cohort using MorphoJ software.ResultsThe elements of normal swallowing can be grouped into four functional modules including bolus propulsion, pharyngeal shortening, airway protection, and head and neck posture. Modularity in HNC patient showed an intact airway protection module but altered bolus propulsion and pharyngeal shortening modules. To cross‐validate the alteration in modules, a post hoc analysis was performed, which showed significantly increased vallecular (P < .04) and piriform (P < .05) residue but no significant change in aspiration status in the HNC cohort versus controls.ConclusionsThis study suggests that while pharyngeal swallowing mechanics is highly complex, the system is organized into functional modules, and that changes in modularity impacts swallowing performance. This approach to understanding swallowing function may help the patient care team better address swallowing difficulties.Level of Evidence2b
Aligned Tubular Conjugated Microporous Polymer Films for the Aggregation‐Induced Emission‐Based Sensing of Explosives
Authors: Ryu Sang Hyun , Lee Doo Hun , Ko Yoon‐Joo , Lee Sang Moon , Kim Hae Jin , Ko Kyoung Chul , Son Seung Uk 
Published: May 2019

AbstractThis work shows the sensing performance of conjugated microporous polymer (CMP) tubes. Aligned tubular CMP films (CMP‐AT) are synthesized by a template method. The Sonogashira coupling of tetra(4‐ethynyl)phenylethylene with 1,4‐diiodobenzene in the cylindrical pores of anodic aluminum oxide (AAO) plates and the etching of templates result in the CMP‐AT films. Due to the tetraphenylethylene moieties in the materials, the CMP‐AT films show aggregation‐induced emission (AIE). Based on emission‐quenching behavior, the sensing performance of CMP‐AT films toward model explosives, nitrotoluenes, is studied. The CMP‐AT films having longer CMP tubes with thinner wall thickness show better sensing performance with the Stern–Volmer constant (Ksv) values up to 92 400 M−1 toward 2,4‐dinitrotoluene. The reduced diffusion pathway of substrates by the thin wall of the CMP tubes is critical for the AIE quenching‐based sensing of nitrotoluenes. These observations indicate that the functionality of CMP materials can be further enhanced by their morphological engineering. Due to the chemical stability of CMP materials, the CMP‐AT‐5 film can be recycled at least five times, maintaining the original sensing performance and tubular morphology.
Exome sequencing identifies a KRT9 pathogenic variant in a Chinese pedigree with epidermolytic palmoplantar keratoderma
Authors: Li Changxing , Chen Pingjiao , Sun Silong , Zeng Kang , Liang Jingyao , Wang Qi , Zhang Sanquan , Xu Meinian , Li Zhijia , Zhang Xibao 
Published: May 2019

AbstractBackgroundEpidermolytic palmoplantar keratoderma (EPPK) is a rare skin disorder and its pathogenesis and inheritability are unknown.ObjectiveTo investigate the inheritance and pathogenesis of EPPK.MethodsTwo EPPK cases occurred in a three‐generation Chinese family. Patient–parents trio EPPK was carried out and the identified candidate variants were confirmed by Sanger sequencing.ResultsA heterozygous missense pathogenic variant, c.488G > A (p.Arg163Gln), in the keratin (KRT) 9 gene was detected in the proband and his son via targeted exome sequencing, and then validated by Sanger sequencing. This pathogenic variant cosegregated with the EPPK in extended family members, and was predicted to be pathogenic by SIFT, PolyPhen2, PROVEAN, and Mutation Taster. This heterozygous variation was not evident in 100 healthy controls.ConclusionThis report describes a KRT9 c.488G > A (p.Arg163Gln) variant causing a diffuse phenotype of Chinese EPPK. The current results broaden the spectrum of KRT9 pathogenic variants responsible for EPPK and have important implications for molecular diagnosis, treatment, and genetic counseling for this family.
Medial approach to the peroneal vessels as recipients for free flap reconstruction of the leg
Authors: Elswick Sarah M. , Miglani Amar , Lettieri Salvatore C. 
Published: May 2019

AbstractThe anatomic variation in the branching pattern of the popliteal vessel has been well‐established. Little has been written in the literature regarding recipient vessel selection for microvascular reconstruction in the lower extremity as it pertains to aberrant vascular anatomy. We present the case of a 57‐year‐old male patient who sustained a closed right lower extremity pilon fracture in a motor vehicle accident. The patient was initially treated with external fixation, which was followed by definitive open reduction and internal fixation 3 weeks later. Over the next 2 weeks, the patient developed skin ischemia of the anterior ankle. A free‐tissue transfer was planned. A digital subtraction angiogram revealed absence of the posterior tibial (PT) vessels and an aberrant course of the peroneal (PR) artery. In the operating room, the medial distal leg was explored for the PT vessels, which were severely hypoplastic. Given the close proximity of the PR vessels, the dissection was extended proximally to assess these vessels for microvascular anastomosis. They were found to be suitable. A parascapular flap was harvested and transferred to the right leg wound without any significant stretch on the vessels for soft tissue coverage. The flap demonstrated excellent inflow and outflow, and the leg and foot remained well perfused. The patient's postoperative course was uncomplicated. The technique of exposing the peroneal vessels for microvascular anastomosis via a medial approach is described. This technique is an excellent option to have available when aberrant anatomy precludes the use more commonly utilized vessels.
Two‐level linearized and local uncoupled difference schemes for the two‐component evolutionary Korteweg‐de Vries system
Authors: Shen Jin‐Ye , Sun Zhi‐Zhong 
Published: May 2019

AbstractThe two‐level linearized and local uncoupled spatial second order and compact difference schemes are derived for the two‐component evolutionary system of nonhomogeneous Korteweg‐de Vries equations. It is shown by the mathematical induction that these two schemes are uniquely solvable and convergent in a discrete L∞ norm with the convergence order of O(τ2 + h2) and O(τ2 + h4), respectively, where τ and h are the step sizes in time and space. Three numerical examples are given to confirm the theoretical results.
BRICS, the southern model, and the evolving landscape of development assistance: Toward a new taxonomy
Authors: Lauria Valeria , Fumagalli Corrado 
Published: May 2019

SummaryIn recent years, there has been an explosion of categories and labels to account for the expansion of forms of cooperation beyond the membership of the Development Assistance Committee. Such hype has led to the construction of the so‐called southern model as the archetype of development cooperation coming from non‐Development Assistance Committee countries that are somehow committed to the principles of the South–South cooperation. The present article challenges the idea of a southern model by providing an analysis of drivers, tools, and modality of development assistance.
A plasma‐based gas‐phase method for synthesis of gold nanoparticles
Authors: Izadi Alborz , Anthony Rebecca J. 
Published: May 2019

AbstractThere are many applications for gold nanoparticles (AuNPs) due to their interesting optoelectronic properties such as tunable optical absorption and plasmonic resonance behavior. Although synthesis and stabilization of colloidal AuNPs are well established, new synthesis routes can lead to enhanced versatility of applications for AuNPs, particularly if the methods allow avoidance of solution processes or surfactants. Here, we introduce a plasma‐based synthesis of AuNPs, using a consumable gold wire and a radiofrequency power source. The AuNPs are monodisperse, with an average diameter of 4 nm. Although production yield is low, the narrow size distribution of the AuNPs and the avoidance of solution processing in this method are promising for future syntheses of metal NPs based on plasmas.
Kinetic versus thermodynamic control of mutational effects on protein homeostasis: A perspective from computational modeling and experiment
Authors: Pobre Kristine Faye R. , Powers David L. , Ghosh Kingshuk , Gierasch Lila M. , Powers Evan T. 
Published: May 2019

AbstractThe effect of mutations in individual proteins on protein homeostasis, or “proteostasis,” can in principle depend on the mutations' effects on the thermodynamics or kinetics of folding, or both. Here, we explore this issue using a computational model of in vivo protein folding that we call FoldEcoSlim. Our model predicts that kinetic versus thermodynamic control of mutational effects on proteostasis hinges on the relationship between how fast a protein's folding reaction reaches equilibrium and a critical time scale that characterizes the lifetime of a protein in its environment: for rapidly dividing bacteria, this time scale is that of cell division; for proteins that are produced in heterologous expression systems, this time scale is the amount of time before the protein is harvested; for proteins that are synthesized in and then exported from the eukaryotic endoplasmic reticulum, this time scale is that of protein secretion, and so forth. This prediction was validated experimentally by examining the expression yields of the wild type and several destabilized mutants of a model protein, the mouse ortholog of cellular retinoic acid‐binding protein 1.
Evaluation of new chemical and biological nematicides for managing Meloidogyne javanica in tomato production and associated double‐crops in Florida
Authors: Desaeger Johan A , Watson Tristan T 
Published: May 2019

AbstractBACKGROUNDTwo field experiments were conducted to determine the efficacy and field performance of three new non‐fumigant chemical nematicides (fluensulfone, fluopyram, and fluazaindolizine) and two biological nematicides (Burkholderia rinojensis strain A396 and Purpureocillium lilacinus strain 251) for management of root‐knot nematodes (Meloidogyne javanica) on tomato and associated double‐crops in Florida.RESULTSIn experiment 1, soil fumigation with metam potassium increased plant growth and reduced root galling on tomato by 77% relative to that of the untreated soil. All non‐fumigant chemical nematicides reduced root galling on tomato (47–85% reduction); however, only fluensulfone showed a trend towards yield enhancement. In experiment 2, soil fumigation with chloropicrin increased plant growth and reduced root galling on tomato by 35% relative to that of the untreated soil; however, end‐of‐season populations of M. javanica in soil were larger than that of the non‐fumigated soil. Fluensulfone showed a trend towards reduced root galling and enhanced fruit yield, whereas other non‐fumigant nematicides did not. Double‐cropped cucumber was 69% more galled when planted into soil previously fumigated with chloropicrin relative to that of untreated soil, and also showed reduced plant vigor and fruit yield.CONCLUSIONFluensulfone shows significant potential to be a component of an integrated pest management strategy for tomato in Florida. © 2019 Society of Chemical Industry