bluuebunny/crossref_metadata_2025_split huggingface dataset:
HPCA confirmed as a genetic cause of DYT2‐like dystonia phenotype
Authors: Atasu Burcu , Hanagasi Hasmet , Bilgic Basar , Pak Meltem , Erginel‐Unaltuna Nihan , Hauser Ann‐Kathrin , Guven Gamze , Simón‐Sánchez Javier , Heutink Peter , Gasser Thomas , Lohmann Ebba 
Published: August 2018

Abstract
Background: HPCA (hippocalcin) is one of the underlying genetic causes of autosomal‐recessively inherited forms of dystonia. Here, we describe two consanguineous Turkish DYT‐HPCA families carrying the novel HPCA mutations.
Methods: After detailed clinical and neurological examination, whole‐exome sequencing was performed.
Results: Whole‐exome sequencing analysis revealed two homozygous novel truncating mutations (p.W103* and p.P10PfsTer80) in the HPCA gene in two unrelated Turkish dystonia families presenting with complex dystonia.
Conclusions: After identification of HPCA as a genetic cause of DYT‐HPCA‐like dystonia by Charlesworth et al, this is the second report in the scientific literature that describes dystonia families harboring HPCA mutations. Our findings confirm that HPCA leads to recessively inherited dystonia. © 2018 International Parkinson and Movement Disorder Society
Projections of prevalence, lifetime risk, and life expectancy of Parkinson's disease (2010‐2030) in France
Authors: Wanneveich Mathilde , Moisan Frédéric , Jacqmin‐Gadda Hélène , Elbaz Alexis , Joly Pierre 
Published: August 2018

ABSTRACTBackground: Previous studies on the number of Parkinson's disease (PD) patients in the future based on projections of population size underestimated PD burden because they did not take into account the improvement of life expectancy over time.Objective: The objective of this study was to assess PD progression from 2010 to 2030 in France in terms of prevalent patient numbers, prevalence rates, lifetime risk, and life expectancy with PD, accounting for projections of overall mortality and increased risk of death of PD patients.Methods: To provide projections of PD burden, we applied a multistate approach considering age and calendar time to incidence and prevalence rates of PD (France 2010) based on drug claims and national demographic data.Results: The number of PD patients will increase by ∼65% between 2010 (n = 155,000) and 2030 (n ∼ 260,000), mainly for individuals older than 65 years; the prevalence rate of PD after age 45 will increase from 0.59% in 2010 to ∼0.80% in 2030. We project an extension of ∼3 years of the life expectancy of PD patients at 65 years between 2010 (women, 14.8 years; men, 13.0 years) and 2030 (women, 17.8 years; men, 16.1 years), and a relative increase of about 10% of the lifetime risk of PD at 45 years between 2010 (women, 5.5%; men, 6.0%) and 2030 (women, 6.3%; men, 7.4%).Conclusions: The number of PD patients is predicted to grow substantially in future years as a consequence of population aging and life expectancy improvement. The assessment of the future PD burden is an important step for planning resources needed for patient care in aging societies. © 2018 International Parkinson and Movement Disorder Society
Lactobacillus plantarum C29‐Fermented Soybean (DW2009) Alleviates Memory Impairment in 5XFAD Transgenic Mice by Regulating Microglia Activation and Gut Microbiota Composition
Authors: Lee Hae‐Ji , Hwang Yun‐Ha , Kim Dong‐Hyun 
Published: August 2018

ScopeThe study aims to determine whether Lactobacillus plantarum C29‐fermented defatted soybean (FDS, DW2009) can attenuate memory impairment in 5XFAD transgenic (Tg) mice.Methods and resultsOral administration of FDS or C29 increases cognitive function in Tg mice in passive avoidance, Y‐maze, novel object recognition, and Morris water maze tasks. FDS or C29 treatment significantly suppresses amyloid‐β, β/γ‐secretases, caspase‐3 expression, and NF‐κB activation, and activates microglia and apoptotic neuron cell populations, and increases BDNF expression in the brain. FDS or C29 treatment suppresses blood and fecal lipopolysaccharide levels and Enterobacteriaceae population and increases lactobacilli/bifidobacteria populations.ConclusionFDS and C29 alleviates the decrease in cognitive function and inhibited amyloid‐β expression in Tg mice by regulating microglia activation and gut microbiota composition.
A more fine‐grained complexity analysis of finding the most vital edges for undirected shortest paths
Authors: Bazgan Cristina , Fluschnik Till , Nichterlein André , Niedermeier Rolf , Stahlberg Maximilian 
Published: August 2018

AbstractWe study the NP‐hard shortest path most vital edges problem arising in the context of analyzing network robustness. For an undirected graph with positive integer edge lengths and two designated vertices s and t, the goal is to delete as few edges as possible in order to increase the length of the (new) shortest st‐path as much as possible. This scenario has been studied from the viewpoint of parameterized complexity and approximation algorithms. We contribute to this line of research by providing refined computational tractability as well as hardness results. We achieve this by a systematic investigation of various problem‐specific parameters and their influence on the computational complexity. Charting the border between tractability and intractability, we also identify numerous challenges for future research.
Alterations in honey bee gut microorganisms caused by Nosema spp. and pest control methods
Authors: Diaz Tsiri , del‐Val Ek , Ayala Ricardo , Larsen John 
Published: August 2018

AbstractBACKGROUNDHoney bees are associated with gut microorganisms essential for their nutrition and health. The composition of the microbial community can be used as a biological health indicator and is characterized using biomarker fatty acids. Commonly, gut microorganisms are exposed to pathogens and to an array of chemical and biological pest control methods.RESULTSWe found a strong negative effect on microbial gut community composition when exposed to the bee pest control chemicals oxytetracycline, oxalic acid and imidacloprid, and when inoculated with the bee pest Nosema spp. and the potential bee pest biocontrol agent Lactobacillus plantarum. Results from the in vitro test with bee pest chemicals showed a differential response of Lactobacillus spp. At the community level, some taxonomic groups were more affected depending on treatment, but sharp changes in the microbial structure were caused by compounds generally considered as bee safe.CONCLUSIONOur results show that pests such as Nosema spp. and pest control methods alter the composition of bee gut microorganisms, which may have severe consequences for pathogen defense, physiology and general honey bee health. In addition, L. plantarum has potential as a biocontrol agent against Nosema spp. © 2018 Society of Chemical Industry
Hierarchical 3D All‐Carbon Composite Structure Modified with N‐Doped Graphene Quantum Dots for High‐Performance Flexible Supercapacitors
Authors: Li Zhen , Liu Xiang , Wang Liang , Bu Fan , Wei Junjie , Pan Dengyu , Wu Minghong 
Published: August 2018

AbstractFlexible supercapacitors have shown enormous potential for portable electronic devices. Herein, hierarchical 3D all‐carbon electrode materials are prepared by assembling N‐doped graphene quantum dots (N‐GQDs) on carbonized MOF materials (cZIF‐8) interweaved with carbon nanotubes (CNTs) for flexible all‐solid‐state supercapacitors. In this ternary electrode, cZIF‐8 provides a large accessible surface area, CNTs act as the electrical conductive network, and N‐GQDs serve as highly pseudocapactive materials. Due to the synergistic effect and hierarchical assembly of these components, N‐GQD@cZIF‐8/CNT electrodes exhibit a high specific capacitance of 540 F g−1 at 0.5 A g−1 in a 1 m H2SO4 electrolyte and excellent cycle stability with 90.9% capacity retention over 8000 cycles. The assembled supercapacitor possesses an energy density of 18.75 Wh kg−1 with a power density of 108.7 W kg−1. Meanwhile, three supercapacitors connected in series can power light‐emitting diodes for 20 min. All‐solid‐state N‐GQD@cZIF‐8/CNT flexible supercapacitor exhibits an energy density of 14 Wh kg−1 with a power density of 89.3 W kg−1, while the capacitance retention after 5000 cycles reaches 82%. This work provides an effective way to construct novel electrode materials with high energy storage density as well as good cycling performance and power density for high‐performance energy storage devices via the rational design.
Peroxisome proliferator‐activated receptor alpha accelerates neuronal differentiation and this might involve the mitogen‐activated protein kinase pathway
Authors: Lin Chingju , Chen Pei‐Yi , Chan Hsu‐Chin , Huang Yi‐Ping , Chang Nai Wen 
Published: August 2018

AbstractActivation of peroxisome proliferator‐activated receptor alpha (PPARα) has been reported to modulate cell proliferation, migration, and differentiation in astrocytes. In this study, we used a retinoic acid (RA)‐induced differentiation model of NTERA‐2/clone D1 (NT2) cells to explore the functional significance of PPARα in neuronal differentiation. We found that activating PPARα by Wy14643 accelerated neuronal differentiation via regulating the expression of neuronal markers. RT‐PCR assays showed a significant increase in NeuroD expression and a decrease in nestin expression in cells treated concomitantly with RA and Wy14643 for 2 days compared to the levels in cells treated with RA alone. Expression of MAP2 protein, a mature neuronal marker, was markedly upregulated at day 10 of Wy14643 treatment, which was maintained after 21 days of neuronal formation. Corresponding to the changes in MAP2 expression, the expression of Cdk5 was upregulated with Wy14643 exposure from day 10 to day 21. Moreover, cells treated with Wy14643 displayed higher expression levels of phospho‐ERK and phospho‐p38 in the differentiation process than cell treated with RA alone. These results indicated that activation of PPARα accelerated neuronal differentiation through upregulating the expression of NeuroD, MAP2, and Cdk5 and downregulating the expression of nestin. MAPK signals, ERK and p38, might contribute to the accelerated differentiation process. These findings suggest that PPARα plays a role in regulating neuronal differentiation and may be beneficial for functional recovery from neurological disorders.
The sensitivity of sensitivity analysis
Authors: Plümper Thomas , Traunmüller Richard 
Published: August 2018

AbstractThis article evaluates the reliability of sensitivity tests. Using Monte Carlo methods we show that, first, the definition of robustness exerts a large influence on the robustness of variables. Second and more importantly, our results also demonstrate that inferences based on sensitivity tests are most likely to be valid if determinants and confounders are almost uncorrelated and if the variables included in the true model exert a strong influence on outcomes. Third, no definition of robustness reliably avoids both false positives and false negatives. We find that for a wide variety of data-generating processes, rarely used definitions of robustness perform better than the frequently used model averaging rule suggested by Sala-i-Martin. Fourth, our results also suggest that Leamer’s extreme bounds analysis and Bayesian model averaging are extremely unlikely to generate false positives. Thus, if based on these inferential criteria a variable is robust, it is almost certain to belong into the empirical model. Fifth and finally, we also show that researchers should avoid drawing inferences based on lack of robustness.
Associations of plasma very-long-chain SFA and the metabolic syndrome in adults
Authors: Zhao Jing , Li Xiaofan , Li Xiang , Chu Qianqian , Zhou Yunhua , Li Zi , Zhang Hong , Brenna Thomas J. , Song Yiqing , Gao Ying 
Published: August 2018

AbstractPlasma levels of very-long-chain SFA (VLCSFA) are associated with the metabolic syndrome (MetS). However, the associations may vary by different biological activities of individual VLCSFA or population characteristics. We aimed to examine the associations of VLCSFA and MetS risk in Chinese adults. Totally, 2008 Chinese population aged 35–59 years were recruited and followed up from 2010 to 2012. Baseline MetS status and plasma fatty acids data were available for 1729 individuals without serious diseases. Among 899 initially metabolically healthy individuals, we identified 212 incident MetS during the follow-up. Logistic regression analysis was used to estimate OR and 95 % CI. Cross-sectionally, each VLCSFA was inversely associated with MetS risk; comparing with the lowest quartile, the multivariate-adjusted OR for the highest quartile were 0·18 (95 % CI 0·13, 0·25) for C20 : 0, 0·26 (95 % CI 0·18, 0·35) for C22 : 0, 0·19 (95 % CI 0·13, 0·26) for C24 : 0 and 0·16 (0·11, 0·22) for total VLCSFA (allPfor trend<0·001). The associations remained significant after further adjusting for C16 : 0, C18 : 0, C18 : 3n-3, C22 : 6n-3,n-6 PUFA and MUFA, respectively. Based on follow-up data, C20 : 0 or C22 : 0 was also inversely associated with incident MetS risk. Among the five individual MetS components, higher levels of VLCSFA were most strongly inversely associated with elevated TAG (≥1·7 mmol/l). Plasma levels of VLCSFA were significantly and inversely associated with MetS risk and individual MetS components, especially TAG. Further studies are warranted to confirm the findings and explore underlying mechanisms.
Beyond Opportunity Costs: Campaign Messages, Anger and Turnout among the Unemployed
Authors: Aytaç S. Erdem , Rau Eli Gavin , Stokes Susan 
Published: August 2018

AbstractAre people under economic stress more or less likely to vote, and why? With large observational datasets and a survey experiment involving unemployed Americans, we show that unemployment depresses participation. But it does so more powerfully when the unemployment rate is low, less powerfully when it is high. Whereas earlier studies have explained lower turnout among the unemployed by stressing the especially high opportunity costs these would-be voters face, our evidence points to the psychological effects of unemployment and of campaign messages about it. When unemployment is high, challengers have an incentive to blame the incumbent, thus eliciting anger among the unemployed. Psychologists have shown anger to be an approach or mobilizing emotion. When joblessness is low, campaigns tend to ignore it. The jobless thus remain in states of depression and self-blame, which are demobilizing emotions.
Developing a real-time PCR assay based on multiplex high-resolution melt-curve analysis: a pilot study in detection and discrimination of soil-transmitted helminth and schistosome species
Authors: Cunningham Lucas J. , Stothard J. Russell , Osei-Atweneboana Mike , Armoo Samuel , Verweij Jaco J. , Adams Emily R. 
Published: August 2018

AbstractWith the push towards control and elimination of soil-transmitted helminthiasis and schistosomiasis in low- and middle-income countries, there is a need to develop alternative diagnostic assays that complement the current in-country resources, preferably at a lower cost. Here, we describe a novel high-resolution melt (HRM) curve assay with six PCR primer pairs, designed to sub-regions of the nuclear ribosomal locus. Used within a single reaction and dye detection channel, they are able to discriminate Ancylostoma duodenale, Necator americanus, Strongyloides stercoralis, Ascaris lumbricoides, Trichuris trichiuria and Schistosoma spp. by HRM curve analysis. Here we describe the primers and the results of a pilot assessment whereby the HRM assay was tested against a selection of archived fecal samples from Ghanaian children as characterized by Kato–Katz and real-time PCR analysis with species-specific TaqMan hydrolysis probes. The resulting sensitivity and specificity of the HRM was 80 and 98.6% respectively. We judge the assay to be appropriate in modestly equipped and resourced laboratories. This method provides a potentially cheaper alternative to the TaqMan method for laboratories in lower resource settings. However, the assay requires a more extensive assessment as the samples used were not representative of all target organisms.
Barriers to Providing Prehospital Care to Ischemic Stroke Patients: Predictors and Impact on Care
Authors: Li Timmy , Cushman Jeremy T. , Shah Manish N. , Kelly Adam G. , Rich David Q. , Jones Courtney M. C. 
Published: August 2018

AbstractIntroductionIschemic stroke treatment is time-sensitive, and barriers to providing prehospital care encountered by Emergency Medical Services (EMS) providers have been under-studied.Hypothesis/ProblemThis study described barriers to providing prehospital care, identified predictors of these barriers, and assessed the impact of these barriers on EMS on-scene time and administration of tissue plasminogen activator (tPA) in the emergency department (ED).MethodsA retrospective cohort study was performed using the Get With The Guidelines-Stroke (GWTG-S; American Heart Association [AHA]; Dallas, Texas USA) registry at two hospitals to identify ischemic stroke patients arriving by EMS. Variables were abstracted from prehospital and hospital medical records and merged with registry data. Barriers to care were grouped into themes. Logistic regression was used to identify predictors of barriers to care, and bi-variate tests were used to assess differences in EMS on-scene time and the proportion of patients receiving tPA between patients with and without barriers.ResultsBarriers to providing prehospital care were documented for 15.5% of patients: 29.6% related to access, 26.7% communication, 23.0% extrication and transportation, 20.0% refusal, and 14.1% assessment/management. Non-white and non-black race (OR: 3.69; 95% CI, 1.63-8.36) and living alone (OR: 1.53; 95% CI, 1.05-2.23) were associated with greater odds of barriers to providing care. The EMS on-scene time was ≥15 minutes for 70.4% of patients who had a barrier to care, compared with 49.0% of patients who did not (P<.001). There was no significant difference in the proportion of patients who were administered tPA between those with and without barriers to care (14.1% vs 19.2%; P=.159).ConclusionsBarriers to providing prehospital care were documented for a sizable proportion of ischemic stroke patients, with the majority related to patient access and communication, and occurred more frequently among non-white and non-black patients and those living alone. Although EMS on-scene time was longer for patients with barriers to care, the proportion of patients receiving tPA in the ED did not differ.LiT, CushmanJT, ShahMN, KellyAG, RichDQ, JonesCMC. Barriers to providing prehospital care to ischemic stroke patients: predictors and impact on care. Prehosp Disaster Med.2018;33(5):501–507.
The Sacred as Secular: State Control and Mosques Neutrality in Post-Revolutionary Tunisia
Authors: Donker Teije H. 
Published: August 2018

AbstractHow are the characteristics of state–religion relations defined? The following paper provides a critical response to the competition perspective in studies on secularization, secularism, and mobilized religion. It argues that actors differ in how religion and state should relate to public life, not the extent that they should be integral or separate from each other. This paper substantiates its argument by exploring how in Tunisia––in a context of revolutionary, social and political instability––a variety of positions were articulated regarding the preferred position of Islam in relation to, first, national identity and, second, state authority. This is done in direct reference to one particular contentious issue: State control over mosques in name of ensuring the partisan neutrality of religious spaces in the country. This paper builds on multiple fieldwork visits to Tunisia and specifically Sfax, during which 32 individuals were interviewed. In addition, this paper builds on hundreds of primary and secondary sources.
A New Appraisal of Lithospheric Structures of the Cordillera‐Craton Boundary Region in Western Canada
Authors: Chen Yunfeng , Gu Yu Jeffrey , Hung Shu‐Huei 
Published: August 2018

AbstractThe Western Canada Sedimentary Basin marks a boundary zone between the Precambrian North American craton and the Phanerozoic Cordillera. Its crystalline basement has documented more than 3 billion years of evolution history of western Laurentia. Here we conduct a high‐resolution survey of the mantle P and S wave velocities using finite‐frequency tomography. Our models show pronounced eastward increases of 4% P and 6% S wave velocities beneath the foreland region, which define a sharp seismic Cordillera‐Craton boundary. In the cratonic region, distinctive high‐ (>2%) velocity anomalies representing depleted mantle lithospheres are well correlated with major Precambrian crustal domains. The largest lithosphere thickness contrast coincides with the Snowbird Tectonic Zone, where the Hearne province extends down to ~300 km, nearly 100 km deeper than the Proterozoic terranes in northern Alberta. In the latter region, a pronounced cylindrical negative velocity anomaly extends subvertically from 75 to ~300‐km depth, which potentially results from significant tectonothermal modifications during subduction and/or plume activities. At the basin scale, mantle velocities show no apparent correlations with surface heat flux, suggesting a minimum mantle contribution to the regional thermal variability. Furthermore, the long‐wavelength isostatic gravity correlates negatively with the velocities, which confirms that the melt extraction from Precambrian cratons is responsible for the formation of highly depleted mantle lithospheres. Moreover, our model reveals the increased concentrations of kimberlites and lamproites near the zones of high horizontal velocity gradients. The distinct spatial pattern may reflect either preferential formation or eruption of potentially diamondiferous rocks at lithospheric weak zones near the western margin of Laurentia.
Kinematics of Late Quaternary Slip Along the Qishan‐Mazhao Fault: Implications for Tectonic Deformation on the Southwestern Ordos, China
Authors: Li Xinnan , Zhang Peizhen , Zheng Wenjun , Feng Xijie , Li Chuanyou , Pierce Ian K. D. , Xu Hongyan , Li Xiaoni , Ai Ming , Chen Gan , Dong Jinyuan , Liu Jinrui , Ren Guangxue 
Published: August 2018

AbstractThe northwest striking Qishan‐Mazhao fault (QMF) accommodates complex deformation in the Tibet‐Ordos transition zone. We studied the geologic and geomorphic expression of the QMF using interpretations of high‐resolution satellite images and structure‐from‐motion models combined with detailed field investigations. Displaced loess tablelands, stream channels, and fluvial terraces show that the QMF is predominately a left‐lateral strike‐slip fault with a minor normal component. The magnetic susceptibility and optically stimulated luminescence ages of offset fluvial terraces yield left‐lateral slip rates ranging from 0.5 to 1.0 mm/year. Regionally, the QMF and the Haiyuan fault (HYF) form a large right step, in which the Liupanshan Mountains are located. The QMF shares a similar orientation and sense of motion to the HYF, suggesting that the left‐lateral slip of the HYF is not completely absorbed as crustal shortening across the Liupanshan Mountains but is partially transferred to slip along the QMF.
Modeling the Effects of Turbulence on Hyporheic Exchange and Local‐to‐Global Nutrient Processing in Streams
Authors: Grant Stanley B. , Gomez‐Velez Jesus D. , Ghisalberti Marco 
Published: August 2018

AbstractNew experimental techniques are allowing, for the first time, direct visualization of mass and momentum transport across the sediment‐water interface in streams. These experimental insights are catalyzing a renaissance in our understanding of the role stream turbulence plays in a host of critical ecosystem services, including nutrient cycling. In this commentary, we briefly review the nature of stream turbulence and its role in hyporheic exchange and nutrient cycling in streams. A simple process‐based model, borrowed from biochemical engineering, provides the link between empirical relationships for grain‐scale turbulent mixing and nutrient processing at reach, catchment, continental, and global scales.