bluuebunny/crossref_metadata_2025_split huggingface dataset:
How Could It Happen? Learning From Adolescents as They Engage Critical Literacy Skills of History
Authors: Schall‐Leckrone Laura , Barron Debra , Konuk Joan , Kain Nick 
Published: April 2018

AbstractOften coauthored by university‐ and school‐based educators, preservice teachers, and youths, this department column considers how literacies are best developed through context‐crossing partnerships among university, school, and community constituents.
Genetic and Epigenetic Defects at the GNAS Locus Lead to Distinct Patterns of Skeletal Growth but Similar Early-Onset Obesity
Authors: Hanna Patrick , Grybek Virginie , Perez de Nanclares Guiomar , Tran Léa C , de Sanctis Luisa , Elli Francesca , Errea Javier , Francou Bruno , Kamenicky Peter , Linglart Léa , Pereda Arrate , Rothenbuhler Anya , Tessaris Daniele , Thiele Susanne , Usardi Alessia , Shoemaker Ashley H , Kottler Marie-Laure , Jüppner Harald , Mantovani Giovanna , Linglart Agnès 
Published: April 2018

ABSTRACT

Pseudohypoparathyroidism type 1A (PHP1A), pseudoPHP (PPHP), and PHP type 1B (PHP1B) are caused by maternal and paternal GNAS mutations and abnormal methylation at maternal GNAS promoter(s), respectively. Adult PHP1A patients are reportedly obese and short, whereas most PPHP patients are born small. In addition to parathyroid hormone (PTH) resistance, PHP1A and PHP1B patients may display early-onset obesity. Because early-onset and severe obesity and short stature are daily burdens for PHP1A patients, we aimed at improving knowledge on the contribution of the GNAS transcripts to fetal and postnatal growth and fat storage. Through an international collaboration, we collected growth and weight data from birth until adulthood for 306 PHP1A/PPHP and 220 PHP1B patients. PHP1A/PPHP patients were smaller at birth than healthy controls, especially PPHP (length Z-score: PHP1A –1.1 ± 1.8; PPHP –3.0 ± 1.5). Short stature is observed in 64% and 59% of adult PHP1A and PPHP patients. PHP1B patients displayed early postnatal overgrowth (height Z-score at 1 year: 2.2 ± 1.3 and 1.3 ± 1.5 in autosomal dominant and sporadic PHP1B) followed by a gradual decrease in growth velocity resulting in normal adult height (Z-score for both: –0.4 ± 1.1). Early-onset obesity characterizes GNAS alterations and is associated with significant overweight and obesity in adults (bodey mass index [BMI] Z-score: 1.4 ± 2.6, 2.1 ± 2.0, and 1.4 ± 1.9 in PPHP, PHP1A, and PHP1B, respectively), indicating that reduced Gsα expression is a contributing factor. The growth impairment in PHP1A/PPHP may be due to Gsα haploinsufficiency in the growth plates; the paternal XLαs transcript likely contributes to prenatal growth; for all disease variants, a reduced pubertal growth spurt may be due to accelerated growth plate closure. Consequently, early diagnosis and close follow-up is needed in patients with GNAS defects to screen and intervene in case of early-onset obesity and decreased growth velocity. © 2018 The Authors. Journal of Bone and Mineral Research Published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research (ASBMR).
Prediction of Fractures in Men Using Bone Microarchitectural Parameters Assessed by High-Resolution Peripheral Quantitative Computed Tomography—The Prospective STRAMBO Study
Authors: Szulc Pawel , Boutroy Stéphanie , Chapurlat Roland 
Published: April 2018

ABSTRACT

Areal bone mineral density (aBMD) poorly identifies men at high fracture risk. Our aim was to assess prediction of fractures in men by bone microarchitectural measures. At baseline, 825 men aged 60 to 87 years had the assessment of bone microarchitecture at distal radius and distal tibia by high-resolution peripheral QCT (HR-pQCT; XtremeCT-I, Scanco Medical, Brüttisellen, Switzerland). Bone strength was estimated by micro-finite element analysis. During the prospective 8-year follow-up, 105 men sustained fractures (59 vertebral fractures in 49 men and 70 nonvertebral fractures in 68 men). After adjustment for age, body mass index (BMI), prior falls, and fractures, most HR-pQCT measures at both skeletal sites predicted fractures. After further adjustment for aBMD, low distal radius trabecular number (Tb.N) was most strongly associated with higher fracture risk (hazard ratio [HR] = 1.63 per SD, 95% confidence interval [CI] 1.31–2.03, p &lt; 0.001). In similar models, low Tb.N was associated with higher risk of major osteoporotic fracture (HR = 1.80 per SD, p &lt; 0.001), vertebral fracture (HR = 1.78 per SD, p &lt; 0.01) and nonvertebral fracture (HR = 1.46 per SD, p &lt; 0.01). In comparison with the reference model (age, BMI, falls, fractures, aBMD), the adjustment for distal radius Tb.N increased the estimated fracture probability in men who sustained fractures versus those who did not have ones (difference = 4.1%, 95% CI 1.9–6.3%, p &lt; 0.001). However, the adjustment for distal radius Tb.N did not increase the area under the curve (AUC, p = 0.37). Similar results were found for distal radius trabecular separation (Tb.Sp) and connectivity density (Conn. D). They were predictive of all fracture types and increased the estimated fracture risk, but not AUC, in men who had incident fractures. Thus, poor distal radius trabecular microarchitecture is predictive of fracture after adjustment for age, BMI, falls, fractures, and aBMD. Although distal radius Tb.N, Conn. D, and Tb.Sp improve the discrimination between men who will or who will not have fracture, they do not provide clinically relevant improvement of fracture prediction in older men. © 2018 American Society for Bone and Mineral Research.
Sclerostin Neutralizing Antibody Treatment Enhances Bone Formation but Does Not Rescue Mechanically Induced Delayed Healing
Authors: Kruck Bettina , Zimmermann Elizabeth A , Damerow Sophie , Figge Christine , Julien Catherine , Wulsten Dag , Thiele Tobias , Martin Madge , Hamdy Reggie , Reumann Marie K , Duda Georg N , Checa Sara , Willie Bettina M 
Published: April 2018

ABSTRACT

During bone healing, tissue formation processes are governed by mechanical strain. Sost/sclerostin, a key Wnt signaling inhibitor and mechano-sensitive pathway, is downregulated in response to mechanical loading. Sclerostin neutralizing antibody (SclAb) increases bone formation. Nevertheless, it remains unclear whether sclerostin inhibition can rescue bone healing in situations of mechanical instability, which otherwise delay healing. We investigated SclAb's influence on tissue formation in a mouse femoral osteotomy, stabilized with rigid or semirigid external fixation. The different fixations allowed different magnitudes of interfragmentary movement during weight bearing, thereby influencing healing outcome. SclAb or vehicle (veh) was administeredand bone healing was assessed at multiple time points up to day 21 postoperatively by in vivo micro-computed tomography, histomorphometry, biomechanical testing, immunohistochemistry, and gene expression. Our results show that SclAb treatment caused a greater bone volume than veh. However, SclAb could not overcome the characteristic delayed healing of semirigid fixation. Indeed, semirigid fixation resulted in delayed healing with a prolonged endochondral ossification phase characterized by increased cartilage, lower bone volume fraction, and less bony bridging across the osteotomy gap than rigid fixation. In a control setting, SclAb negatively affected later stages of healing under rigid fixation, evidenced by the high degree of endosteal bridging at 21 days in the rigid-SclAb group compared with rigid-veh, indicating delayed fracture callus remodeling and bone marrow reconstitution. Under rigid fixation, Sost and sclerostin expression at the gene and protein level, respectively, were increased in SclAb compared with veh-treated bones, suggesting a negative feedback mechanism. Our results suggest that SclAb could be used to enhance overall bone mass but should be carefully considered in bone healing. SclAb may help to increase bone formation early in the healing process but not during advanced stages of fracture callus remodeling and not to overcome delayed healing in semirigid fixation. © 2018 American Society for Bone and Mineral Research.
Respiratory disease, behavior, and survival of mountain goat kids
Authors: Blanchong Julie A. , Anderson Christopher A. , Clark Nicholas J. , Klaver Robert W. , Plummer Paul J. , Cox Mike , McAdoo Caleb , Wolff Peregrine L. 
Published: April 2018

ABSTRACTBacterial pneumonia is a threat to bighorn sheep (Ovis canadensis) populations. Bighorn sheep in the East Humboldt Mountain Range (EHR), Nevada, USA, experienced a pneumonia epizootic in 2009–2010. Testing of mountain goats (Oreamnos americanus) that were captured or found dead on this range during and after the epizootic detected bacteria commonly associated with bighorn sheep pneumonia die‐offs. Additionally, in years subsequent to the bighorn sheep epizootic, the mountain goat population had low kid:adult ratios, a common outcome for bighorn sheep populations that have experienced a pneumonia epizootic. We hypothesized that pneumonia was present and negatively affecting mountain goat kids in the EHR. From June–August 2013–2015, we attempted to observe mountain goat kids with marked adult females in the EHR at least once per week to document signs of respiratory disease; identify associations between respiratory disease, activity levels, and subsequent disappearance (i.e., death); and estimate weekly survival. Each time we observed a kid with a marked adult female, we recorded any signs of respiratory disease and collected behavior data that we fit to a 3‐state discrete hidden Markov model (HMM) to predict a kid's state (active vs. sedentary) and its probability of disappearing. We first observed clinical signs of respiratory disease in kids in late July–early August each summer. We observed 8 of 31 kids with marked adult females with signs of respiratory disease on 13 occasions. On 11 of these occasions, the HMM predicted that kids were in the sedentary state, which was associated with increased probability of subsequent death. We estimated overall probability of kid survival from June–August to be 0.19 (95% CI = 0.08–0.38), which was lower than has been reported in other mountain goat populations. We concluded that respiratory disease was present in the mountain goat kids in the EHR and negatively affected their activity levels and survival. Our results raise concerns about potential effects of pneumonia to mountain goat populations and the potential for disease transmission between mountain goats and bighorn sheep where the species are sympatric. © 2018 This article is a U.S. Government work and is in the public domain in the USA.
Phenotypes in siblings with homozygous mutations of TRAPPC9 and/or MCPH1 support a bifunctional model of MCPH1
Authors: Duerinckx Sarah , Meuwissen Marije , Perazzolo Camille , Desmyter Laurence , Pirson Isabelle , Abramowicz Marc 
Published: April 2018

AbstractBackgroundAutosomal recessive intellectual disability (ARID) is vastly heterogeneous. Truncating mutations of TRAPPC9 were reported in 8 ARID families. Autosomal recessive primary microcephaly (MCPH) represents another subgroup of ARID, itself very heterogeneous, where the size of the brain is very small since birth. MCPH1 plays a role at the centrosome via a BRCT1 domain, and in DNA Damage Repair (DDR) via BRCT2 and BRCT3, and it is not clear which of these two mechanisms causes MCPH in man.MethodsWe studied the phenotype and sequenced the exome in two siblings with MCPH and their unaffected sister.ResultsHomozygous mutations of TRAPPC9 (p.Leu178Pro) and of MCPH1 (p.Arg741X) were found in both affected siblings. Brain MRI showed anomalies previously associated with TRAPPC9 defects, supporting the implication of TRAPPC9 in the phenotype. Importantly, the asymptomatic sister with normal head size was homozygous for the MCPH1 truncating mutation and heterozygous for the TRAPPC9 mutation.ConclusionThe affected siblings represent the first ARID cases with a TRAPPC9 missense mutation and with microcephaly of prenatal onset of. Furthermore, their unaffected sister represents strong evidence that the lack of MCPH1 BRCT3 domain does not cause MCPH in man, supporting a bifunctional model of MCPH1 where the centrosomal function is involved in brain volumic development and not the DDR function.
Meta‐analysis of the incidence and patterns of second neoplasms after photon craniospinal irradiation in children with medulloblastoma
Authors: Bavle Abhishek , Tewari Sayani , Sisson Amy , Chintagumpala Murali , Anderson Michael , Paulino Arnold C. 
Published: April 2018

AbstractBackgroundSecond neoplasms (SNs) are a well‐established long‐term adverse effect of radiation therapy (RT), but there are limited data regarding their incidence and location relative to the radiation field, specific to medulloblastoma (MB) survivors after craniospinal irradiation (CSI).MethodsA systematic literature review, per Preferred Reporting Items for Systematic Reviews and Meta‐Analyses, identified six studies reporting the incidence and locations of SNs for 1,114 patients with MB, after CSI, with a median follow‐up of ∼9 years (7.6–15.4 years). The study‐specific cumulative incidence (CI) of SNs, second benign neoplasms (SBNs), and second malignant neoplasms (SMNs) were standardized to a 10‐year time frame. Meta‐analysis was performed using random effects models, with pooled data from selected studies and an institutional cohort of 55 patients.ResultsThe 10‐year CI was 6.1% for all SNs (excluding skin cancer and leukemia), 3.1% for SBNs, and 3.7% for SMNs. Fifty‐eight percent of SNs were malignant; high‐grade glioma was the most common SMN (15/33; 45%) and meningioma, the most common SBN (16/24; 67%). Forty percent of SNs occurred outside the target central nervous system (CNS) field, with a majority in areas of exit RT dose. Seventy‐four percent of extra‐CNS tumors (17/23) were malignant, most commonly thyroid carcinoma (7/17; 41%) and bone and soft‐tissue tumors (6/17, 35%).ConclusionsSurvivors of MB are at risk of SNs both within and outside the CNS. A significant proportion of SNs occur in areas of exit RT dose. Studies are needed to determine whether the use of proton therapy, which has no exit RT dose, is associated with a lower incidence of SNs.
Addressing policy challenges in implementing Sustainable Development Goals through an adaptive governance approach: A view from transitional China
Authors: Xue Lan , Weng Lingfei , Yu Hanzhi 
Published: April 2018

AbstractIn 2015, the 17 Sustainable Development Goals (SDGs) were adopted by all member states of the United Nations. This paper argues that actually implementing the SDGs is far more challenging than reaching agreement to adopt them. To overcome these challenges, a Four‐Step Adaptive Governance Framework was set up to facilitate implementation of these goals. Two‐level policy tools are presented to illustrate the policy evolution of China's development transitions, within the framework of “adaptive governance.” This study found that the Four‐Step Adaptive Governance Approach is deeply embedded in China's policy process, and it has contributed significantly to China's development during the past four decades. The Adaptive Governance Framework and this example of its application in China suggests it could be a useful tool for developing countries that are formulating their national strategies to achieve their SDGs by 2030.
Graphene‐Oxide‐Supported SO3H‐Functionalized Imidazolium‐Based Ionic Liquid: Efficient and Recyclable Heterogeneous Catalyst for Alcoholysis and Aminolysis Reactions
Authors: Nakhate Akhil V. , Yadav Ganapati D. 
Published: April 2018

AbstractIonic liquids have been established as solvents and catalysts in a number of reactions due to their unique properties. In this study, we have synthesized sulfonic acid functionalized imidazolium based strongly acidic ionic liquid supported on graphene oxide (PTS−Im‐3@GO). PTS−Im‐3@GO was synthesized by grafting 1‐(4‐sulfobutyl)‐3‐(3‐propyltriethoxysilyl)imidazolium hydrogen sulfate onto graphene oxide (GO) by covalent bonds. The prepared PTS−Im‐3@GO was used for regio‐selective ring opening reaction of styrene oxide with isopropyl alcohol giving 95% conversion and 100% regio‐selectivity towards 2‐isopropoxy‐2‐phenylethan‐1‐ol at 50 °C. The activity of the said catalyst was compared with various heterogeneous catalysts such as GO, PTS−Im‐1@GO, PTS−Im‐2@GO, PTS−Im‐1, PTS−Im‐2, PTS−Im‐3, Hβ‐zeolite, and montmorillonite K‐10. Among all catalysts, PTS−Im‐3@GO showed good conversion towards the desired product. PTS−Im‐3@GO was characterized by FTIR, SEM, EDS, TGA, XRD, 29Si NMR, XPS and CHNS analysis. Systematic studies demonstrated that PTS−Im‐3@GO had excellent catalytic activity and regio‐selectivity for the alcoholysis of epoxides rendering β‐alkoxy alcohols and aminolysis of epoxide giving β‐amino alcohols in very good yields. The protocol is clean and green.
Room‐Temperature Synthesis and Enhanced Photocatalytic Performance of BiOCl Microsphere
Authors: Li Jin , Li Yuanrui 
Published: April 2018

AbstractHierarchical BiOCl microspheres assembled from nanosheets with predominated (110) facets were facilely fabricated by precipitation at room temperature and characterized systematically. The effects of the solvents, chlorine precursors and reaction time on the growth of the BiOCl microspheres have been investigated in detail. Rhodamine B (RhB) was selected to evaluate the photocatalytic activity of the as‐obtained samples under visible‐light irradiation, and the results revealed that the BiOCl microspheres synthesized with WCl6 exhibited superior RhB removal efficiency through a photosensitization process, and the degradation efficiency was 99% within 25 min and was still kept over 97% after five runs. The enhanced photocatalytic activities were mainly attributed to their hierarchical structure, high specific surface area as well as more exposed (110) facet. The superoxide radicals (•O2−) were demonstrated to be the main active species in the photodegradation process. Additionally, photocatalysis mechanism was proposed based on experimental results.
Enhanced Electrochemical Performances of LiMnPO4/C via Liquid Crystal Template Pathway
Authors: Xiao Zhenghui , He Yang , Li Xueliang , Zhang Luyao , Ding Zhongqiang 
Published: April 2018

AbstractHighly ordered LiMnPO4 (LMP) and LiMnPO4/C (LMP/C) were successfully synthesized with a facile hydrothermal process via cholesteryl caprylate (C35H60O2) liquid crystal‐assisted routine. The structures and morphologies were characterized through X‐ray diffraction, X‐ray photoelectron spectroscopy, scanning electron microscopy and transmission electron microscopy. The electrochemical performances of LiMnPO4 and LiMnPO4/C were investigated by cyclic voltammetry, charge‐discharge and alternating current (AC) impedance test. As‐prepared orthorhombic LiMnPO4 presents a large number of (020) planes and minimizes the paths for Li diffusion. The ordered LiMnPO4/C possess the higher rate and cycling performances with a specific discharge capacity of 156 mA h g−1 at 0.1C, and the remained capacity of 145 mA h g−1 after 100 cycles. These excellent results show that cholesteryl caprylate liquid crystal plays a crucial role in forming the structure with more active (020) planes for efficient Li+ extraction and insertion.
Diversification of transcription factor–DNA interactions and the evolution of gene regulatory networks
Authors: Rogers Julia M. , Bulyk Martha L. 
Published: April 2018

Sequence‐specific transcription factors (TFs) bind short DNA sequences in the genome to regulate the expression of target genes. In the last decade, numerous technical advances have enabled the determination of the DNA‐binding specificities of many of these factors. Large‐scale screens of many TFs enabled the creation of databases of TF DNA‐binding specificities, typically represented as position weight matrices (PWMs). Although great progress has been made in determining and predicting binding specificities systematically, there are still many surprises to be found when studying a particular TF's interactions with DNA in detail. Paralogous TFs' binding specificities can differ in subtle ways, in a manner that is not immediately apparent from looking at their PWMs. These differences affect gene regulatory outputs and enable TFs to rewire transcriptional networks over evolutionary time. This review discusses recent observations made in the study of TF–DNA interactions that highlight the importance of continued in‐depth analysis of TF–DNA interactions and their inherent complexity.This article is categorized under:

Biological Mechanisms > Regulatory Biology
Adolescent predictors of social and coping drinking motives in early adulthood
Authors: Temmen Chelsie D. , Crockett Lisa J. 
Published: April 2018

ABSTRACTAlthough motivations to use alcohol have been shown to predict alcohol use and alcohol‐related problems, little is known about how drinking motives develop. This study identified antecedents in adolescence of social and coping motives for drinking in early adulthood. Data came from a longitudinal study of youths in the eastern U.S. (N = 451) followed from secondary school into early adulthood (52.4% female; Mage = 23.01; SD = 1.03). In a structural equation analysis, frequency of drunkenness and peer alcohol use positively predicted young adult social motives, whereas only frequency of drunkenness predicted coping motives. These findings indicate that alcohol use behaviors and social relationships in adolescence may contribute to the development of adult drinking motives.
THE EFFECTS OF BIOTECHNOLOGY ON PRODUCTIVITY AND INPUT DEMANDS IN U.S. AGRICULTURE
Authors: CHAVAS JEAN-PAUL , SHI GUANMING , NEHRING RICHARD , STIEGERT KYLE 
Published: April 2018

AbstractU.S. agriculture has seen a rapid adoption of biotechnology over the last two decades. This study investigates how biotechnology has affected U.S. farm input demand and agricultural productivity. The analysis relies on data at the national level and at the state level for selected states in the Corn Belt. It evaluates the rate of technological change and price elasticities of demand for agricultural inputs over time. The study documents the evolving biases in technological change in agriculture. It finds evidence that farm input demands have become more price inelastic.
HOW DID MARITIME PIRACY AFFECT TRADE IN SOUTHEAST ASIA?
Authors: Morabito Giacomo , Sergi Bruno S. 
Published: April 2018

AbstractOver the past 30 years, Southeast Asia has experienced rapid growth in intra-regional economic activity, but despite the remarkable diminution in the frequency and intensity of military conflict and crises, it has not been free of interstate disputes, such as maritime disputes. However, the struggle against maritime crime activities such as maritime piracy is a priority for all countries of the region, as well as one of the unavoidable prerequisites for the achievement of the regional economic security. This research note focuses on the impact of maritime piracy on the Southeast Asian countries’ trade. Bilateral trade flows among the Southeast Asian countries over the 1994 to 2013 period are used to estimate an augmented gravity model that includes various measures of maritime crime activities. The purpose is to find the evidence to indicate how maritime piracy has affected the volume of intra-regional trade.