bluuebunny/crossref_metadata_2025_split huggingface dataset:
Dilated cardiomyopathy secondary to rickets-related hypocalcaemia: eight case reports and a review of the literature
Authors: Yilmaz Osman , Olgun Hasim , Ciftel Murat , Kilic Omer , Kartal Ibrahim , Iskenderoglu Nebahat Y. , Laloglu Fuat , Ceviz Naci 
Published: December 2013

AbstractIntroduction: Dilated cardiomyopathy is usually idiopathic and may arise secondary to infections or metabolic or genetic causes. Another rare cause is hypocalcaemia. Owing to the fact that calcium plays an essential role in excitation and contraction of myocardial muscle, myocardial contractility may decline in patients with hypocalcaemia. Materials and Methods: Patients with symptoms of congestive heart failure and rickets-related hypocalcaemia were assessed clinically and by echocardiography in a paediatric cardiology clinic. Echocardiography was performed for all patients. Rickets was diagnosed according to the clinical, laboratory, and radiologic findings. Maternal lifestyle and living conditions were investigated, and the maternal 25-OH vitamin D3 blood level was measured. Results: We evaluated eight patients who developed heart failure as a result of severe hypocalcaemia associated with rickets between August, 1999 and June, 2012. The age distribution of the patients was 3–12 months. Laboratory results were consistent with advanced-stage rickets. Severe hypocalcaemia was detected in all patients. The maternal 25-OH vitamin D3 levels were low. Echocardiography revealed increased pre-treatment left ventricle end-systolic and end-diastolic diameters for age and reduced ejection fraction and fractional shortening. After clinical improvement, the patients were discharged. Conclusions: Severe hypocalcaemia associated with rickets must always be kept in mind among the causes of dilated cardiomyopathy and impaired cardiac function in infants. If diagnosed and treated in time, dilated cardiomyopathy and severe heart failure related to rickets respond well.
Dis-assembling Traditions: Deconstructing Tasan via Matteo Ricci
Authors: CAWLEY KEVIN N. 
Published: December 2013

AbstractChŏng Yagyong 丁若鏞 (1762–1836), commonly known by the penname Tasan 茶山, was infamous for his involvement in the early Catholic Church, which was formed by his close friends and his brothers. This Church was responsible for its self-evangelisation based on the ideas found in Matteo Ricci's Tianzhu shiyi 天主實義 (The True Meaning of the Lord of Heaven). The effects of this very controversial aspect of his life, and the influence of this precarious context—fraught with danger, narrowly escaping execution and exiled for 18 years—has been under-valued. This paper highlights the effects of such a context on Tasan's ideas by engaging with “deconstruction”, drawing on the ideas of Jacques Derrida. I outline how Tasan embroidered Ricci's deconstructive strategies into the deepest fabric of his own deconstruction of “original” Confucianism, or his dis-assembling of traditions. The paper uncovers Christian traces silhouetted in Tasan's theistic commentaries, leading him towards a Post-Confucian conceptualisation of humanity (仁) vis-à-vis a personal, monotheistic, creator God.
Determining the optimum morphology in high-performance polymer-fullerene organic photovoltaic cells
Authors: Hedley Gordon J. , Ward Alexander J. , Alekseev Alexander , Howells Calvyn T. , Martins Emiliano R. , Serrano Luis A. , Cooke Graeme , Ruseckas Arvydas , Samuel Ifor D. W. 
Published: December 2013

AbstractThe morphology of bulk heterojunction organic photovoltaic cells controls many of the performance characteristics of devices. However, measuring this morphology is challenging because of the small length-scales and low contrast between organic materials. Here we use nanoscale photocurrent mapping, ultrafast fluorescence and exciton diffusion to observe the detailed morphology of a high-performance blend of PTB7:PC71BM. We show that optimized blends consist of elongated fullerene-rich and polymer-rich fibre-like domains, which are 10–50 nm wide and 200–400 nm long. These elongated domains provide a concentration gradient for directional charge diffusion that helps in the extraction of charge pairs with 80% efficiency. In contrast, blends with agglomerated fullerene domains show a much lower efficiency of charge extraction of ~45%, which is attributed to poor electron and hole transport. Our results show that the formation of narrow and elongated domains is desirable for efficient bulk heterojunction solar cells.
A rare functional cardioprotective APOC3 variant has risen in frequency in distinct population isolates
Authors: Tachmazidou Ioanna , Dedoussis George , Southam Lorraine , Farmaki Aliki-Eleni , Ritchie Graham R. S. , Xifara Dionysia K. , Matchan Angela , Hatzikotoulas Konstantinos , Rayner Nigel W. , Chen Yuan , Pollin Toni I. , O’Connell Jeffrey R. , Yerges-Armstrong Laura M. , Kiagiadaki Chrysoula , Panoutsopoulou Kalliope , Schwartzentruber Jeremy , Moutsianas Loukas ,   UK10K consortium, Tsafantakis Emmanouil , Tyler-Smith Chris , McVean Gil , Xue Yali , Zeggini Eleftheria 
Published: December 2013

AbstractIsolated populations can empower the identification of rare variation associated with complex traits through next generation association studies, but the generalizability of such findings remains unknown. Here we genotype 1,267 individuals from a Greek population isolate on the Illumina HumanExome Beadchip, in search of functional coding variants associated with lipids traits. We find genome-wide significant evidence for association between R19X, a functional variant in APOC3, with increased high-density lipoprotein and decreased triglycerides levels. Approximately 3.8% of individuals are heterozygous for this cardioprotective variant, which was previously thought to be private to the Amish founder population. R19X is rare (<0.05% frequency) in outbred European populations. The increased frequency of R19X enables discovery of this lipid traits signal at genome-wide significance in a small sample size. This work exemplifies the value of isolated populations in successfully detecting transferable rare variant associations of high medical relevance.
Active and secreted IgA-coated bacterial fractions from the human gut reveal an under-represented microbiota core
Authors: D'Auria Giuseppe , Peris-Bondia Francesc , Džunková Mária , Mira Alex , Collado Maria Carmen , Latorre Amparo , Moya Andrés 
Published: December 2013

AbstractHost-associated microbiota varies in distribution depending on the body area inhabited. Gut microbes are known to interact with the human immune system, maintaining gut homoeostasis. Thus, we studied whether secreted-IgA (S-IgA) coat specific microbial taxa without inducing strong immune responses. To do so, we fractionated gut microbiota by flow cytometry. We found that active and S-IgA-coated bacterial fractions were characterized by a higher diversity than those observed in raw faecal suspensions. A long-tail effect was observed in family distribution, revealing that rare bacteria represent up to 20% of total diversity. While Firmicutes was the most abundant phylum, the majority of its sequences were not assigned at the genus level. Finally, the single-cell-based approach enabled us to focus on active and S-IgA-coated bacteria. Thus, we revealed a microbiota core common to the healthy volunteers participating in the study. Interestingly, this core was composed mainly of low frequency taxa (e.g. Sphingomonadaceae).
The renin–angiotensin–aldosterone system in pre-eclampsia: the delicate balance between good and bad
Authors: Verdonk Koen , Visser Willy , Van Den Meiracker Anton H. , Danser A. H. Jan 
Published: December 2013

Pregnancy demands major changes of the cardiovascular system, and this involves, among others, activation of the RAAS (renin–angiotensin–aldosterone system), allowing an aldosterone-dependent increase in volume. Remarkably, a relative resistance to the pressor response of AngII (angiotensin II) develops simultaneously to prevent the increase in blood pressure that would normally accompany RAAS activation. The increase in volume, the degree of RAAS activation and the diminished pressor response to AngII are less pronounced in pre-eclampsia. However, animal models displaying excessive RAAS activation also result in a pre-eclampsia-like syndrome, and the aldosterone/renin ratio is elevated in pre-eclampsia compared with a normal pregnancy. New insights into the pathogenesis of pre-eclampsia have revealed a major role for VEGF (vascular endothelial growth factor), VEGF-inactivating sFlt-1 (soluble fms-like tyrosine kinase-1) and AT1 (angiotensin II type 1) receptor autoantibodies. The last mentioned activate AT1 receptors, thereby potentially suppressing circulating renin and aldosterone. VEGF, both directly and indirectly (by increasing capillary density), affects adrenal aldosterone synthesis. The present review summarizes all of the recent findings regarding RAAS regulation in pre-eclampsia compared with normal pregnancy, concluding that factors such as sFlt-1 and AT1 receptor autoantibodies disturb the delicate balance that normally results in a volume increase and a diminished vasoconstrictor response to AngII in pregnant women. It is possible that there are non-parallel changes in the circulating and renal RAAS in pre-eclampsia, which are potentially reflected by the urinary levels of renin.
Climate change effects on agriculture: Economic responses to biophysical shocks
Authors: Nelson Gerald C. , Valin Hugo , Sands Ronald D. , Havlík Petr , Ahammad Helal , Deryng Delphine , Elliott Joshua , Fujimori Shinichiro , Hasegawa Tomoko , Heyhoe Edwina , Kyle Page , Von Lampe Martin , Lotze-Campen Hermann , Mason d’Croz Daniel , van Meijl Hans , van der Mensbrugghe Dominique , Müller Christoph , Popp Alexander , Robertson Richard , Robinson Sherman , Schmid Erwin , Schmitz Christoph , Tabeau Andrzej , Willenbockel Dirk 
Published: December 2013

Significance
Plausible estimates of climate change impacts on agriculture require integrated use of climate, crop, and economic models. We investigate the contribution of economic models to uncertainty in this impact chain. In the nine economic models included, the direction of management intensity, area, consumption, and international trade responses to harmonized crop yield shocks from climate change are similar. However, the magnitudes differ significantly. The differences depend on model structure, in particular the specification of endogenous yield effects, land use change, and propensity to trade. These results highlight where future research on modeling climate change impacts on agriculture should focus.
Hydrological droughts in the 21st century, hotspots and uncertainties from a global multimodel ensemble experiment
Authors: Prudhomme Christel , Giuntoli Ignazio , Robinson Emma L. , Clark Douglas B. , Arnell Nigel W. , Dankers Rutger , Fekete Balázs M. , Franssen Wietse , Gerten Dieter , Gosling Simon N. , Hagemann Stefan , Hannah David M. , Kim Hyungjun , Masaki Yoshimitsu , Satoh Yusuke , Stacke Tobias , Wada Yoshihide , Wisser Dominik 
Published: December 2013

Significance
Increasing concentrations of greenhouse gases in the atmosphere are widely expected to influence global climate over the coming century. The impact on drought is uncertain because of the complexity of the processes but can be estimated using outputs from an ensemble of global models (hydrological and climate models). Using an ensemble of 35 simulations, we show a likely increase in the global severity of drought by the end of 21st century, with regional hotspots including South America and Central and Western Europe in which the frequency of drought increases by more than 20%. The main source of uncertainty in the results comes from the hydrological models, with climate models contributing to a substantial but smaller amount of uncertainty.
Global water resources affected by human interventions and climate change
Authors: Haddeland Ingjerd , Heinke Jens , Biemans Hester , Eisner Stephanie , Flörke Martina , Hanasaki Naota , Konzmann Markus , Ludwig Fulco , Masaki Yoshimitsu , Schewe Jacob , Stacke Tobias , Tessler Zachary D. , Wada Yoshihide , Wisser Dominik 
Published: December 2013

Significance
Humans alter the water cycle by constructing dams and through water withdrawals. Climate change is expected to additionally affect water supply and demand. Here, model analyses of climate change and direct human impacts on the terrestrial water cycle are presented. The results indicate that the impact of man-made reservoirs and water withdrawals on the long-term global terrestrial water balance is small. However, in some river basins, impacts of human interventions are significant. In parts of Asia and the United States, the effects of human interventions exceed the impacts expected for moderate levels of global warming. This study also identifies areas where irrigation water is currently scarce, and where increases in irrigation water scarcity are projected.
Mangarevan invention of binary steps for easier calculation
Authors: Bender Andrea , Beller Sieghard 
Published: December 2013

Significance
The paper describes the mixed counting system in Mangarevan, which is unique in that it had three binary steps superposed onto a decimal structure. In showing how these steps affect calculation, our analysis yields important insights for theorizing on numerical cognition: counting systems serve as complex cultural tools for numerical cognition, apparently unwieldy systems may in fact be cognitively advantageous, and such advantageous systems can be—and have been—developed by nonindustrialized societies and in the absence of notational systems. These insights also help to dismiss simple notions of cultural complexity as a homogenous state and emphasize that investigating cultural diversity is not merely an optional extra, but a must.
FMRP and Ataxin-2 function together in long-term olfactory habituation and neuronal translational control
Authors: Sudhakaran Indulekha P. , Hillebrand Jens , Dervan Adrian , Das Sudeshna , Holohan Eimear E. , Hülsmeier Jörn , Sarov Mihail , Parker Roy , VijayRaghavan K. , Ramaswami Mani 
Published: December 2013

Significance
This work explores the endogenous functions of two disease- and RNA-associated proteins, FMRP (fragile X protein) and Ataxin-2, in memory, synaptic plasticity, RNA regulation, and messenger ribo-nucleoprotein particle assembly. By documenting an array of common phenotypic consequences from loss of Atx2 or dFMR1, the results argue that both proteins have similar in vivo functions and that differences in spinocerebellar ataxia 2 and fragile X disease pathologies may arise from the distinct features of the respective disease causative mutations. This work provides insight into the in vivo mechanisms of long-term memory (LTM)-associated synaptic plasticity and into the roles of FMRP and Atx2 in neuronal translational control.
JMJD5 regulates PKM2 nuclear translocation and reprograms HIF-1α–mediated glucose metabolism
Authors: Wang Hung-Jung , Hsieh Ya-Ju , Cheng Wen-Chi , Lin Chun-Pu , Lin Yu-shan , Yang So-Fang , Chen Chung-Ching , Izumiya Yoshihiro , Yu Jau-Song , Kung Hsing-Jien , Wang Wen-Ching 
Published: December 2013

Significance
Cancer cells favor high rates of aerobic glycolysis, or the Warburg effect, which is mediated by a key molecule, pyruvate kinase muscle isozyme (PKM)2. PKM2 functions both as a cytosolic enzyme and a nuclear factor in tumor cells. This report shows that PKM2’s nuclear translocation is regulated by Jumonji C domain-containing dioxygenase (JMJD)5 via direct physical binding. JMJD5 hinders the PKM2 tetrameric assembly and facilitates PKM2’s nuclear translocation. Together, they modulate hypoxia-inducible factor 1α-mediated transcriptional reprogramming of metabolic genes. These results reveal a mechanism whereby PKM2’s activity can be modulated by a dioxygenase/demethylase.
Rewiring yeast sugar transporter preference through modifying a conserved protein motif
Authors: Young Eric M. , Tong Alice , Bui Hang , Spofford Caitlin , Alper Hal S. 
Published: December 2013

Significance
The quest for an optimal xylose pathway in yeast is of utmost importance along the way to realizing the potential of lignocellulosic biomass conversion into fuels and chemicals. An often-overlooked aspect of this catabolic pathway is the molecular transporter of this sugar. Here we demonstrate that sugar transport preference and kinetics can be rewired through the programming of a specific sequence motif. The result is a study to rationally alter the sugar preference of a protein through defined sequence-level modifications. In these cases, primary hexose transporters were rewired into xylose transporters.
Initial stages of calcium uptake and mineral deposition in sea urchin embryos
Authors: Vidavsky Netta , Addadi Sefi , Mahamid Julia , Shimoni Eyal , Ben-Ezra David , Shpigel Muki , Weiner Steve , Addadi Lia 
Published: December 2013

Significance
With the onset of gastrulation, sea urchin embryos deposit a calcium carbonate endoskeleton consisting of two spicules. Sea water is the source for the mineral ions, but the specific stages of the transport and deposition pathway are not well understood. This study shows that the first-formed mineral is deposited inside intracellular micrometer-size vesicles as solid nanospheres. Surprisingly, the initial deposits are distributed widely inside the embryonic cells, including epithelial cells. The possibility that the whole embryo is geared toward depositing mineral for spicule formation or other purposes, may contribute to the understanding of biomineralization processes in general.
How risk management can prevent future wildfire disasters in the wildland-urban interface
Authors: Calkin David E. , Cohen Jack D. , Finney Mark A. , Thompson Matthew P. 
Published: December 2013

SignificanceRecent wildfire events throughout the world have highlighted the consequences of residential development in the wildland-urban interface (WUI) including hundreds to thousands of homes burned during a single wildfire to, more tragically, firefighter and homeowner fatalities. Despite substantial investments in modifying wildland fuels near populated areas, losses appear to be increasing. In this article, we examine the conditions under which WUI wildfire disasters occur and introduce a wildfire risk assessment framework. By using this framework, we examine how prefire mitigation activities failed to prevent significant structure loss during the Fourmile Canyon fire outside Boulder, CO. In light of these results, we suggest the need to reevaluate and restructure wildfire mitigation programs aimed at reducing residential losses from wildfire.
Early Pleistocene third metacarpal from Kenya and the evolution of modern human-like hand morphology
Authors: Ward Carol V. , Tocheri Matthew W. , Plavcan J. Michael , Brown Francis H. , Manthi Fredrick Kyalo 
Published: December 2013

SignificanceA newly discovered metacarpal from Kaitio, Kenya, dates to 1.42 Mya and provides evidence for the evolution of the modern human hand more than 600,000 y earlier than previously documented. This bone displays a styloid process, which is part of a distinctively human rearrangement of the wrist associated with enhanced hand function when making and using tools. Prior to this discovery, the first evidence of this anatomy was found only in post–Homo erectusarchaic humans. This new find documents its appearance much earlier in time and suggests that an increased reliance on manipulatory behaviors indicated by the archeological record early in the Pleistocene selected for the modern human hand early in the evolution of the genusHomo.
Structure of Est3 reveals a bimodal surface with differential roles in telomere replication
Authors: Rao Timsi , Lubin Johnathan W. , Armstrong Geoffrey S. , Tucey Timothy M. , Lundblad Victoria , Wuttke Deborah S. 
Published: December 2013

Significance
Despite the central role that budding yeast has played in telomere biology, structural analysis of the subunits of the yeast telomerase complex has proven to be challenging. We present here the structure of a yeast telomerase protein, Est3, using the resolution-adapted structural recombination Rosetta strategy that combines NMR experimental data with database-derived conformational sampling. A comprehensive in vivo analysis of the experimentally determined Est3 protein surface has identified two functionally important surfaces, opening up the possibility of a similar discovery in the structurally similar human TPP1 protein.
Quantum mechanical calculations suggest that lytic polysaccharide monooxygenases use a copper-oxyl, oxygen-rebound mechanism
Authors: Kim Seonah , Ståhlberg Jerry , Sandgren Mats , Paton Robert S. , Beckham Gregg T. 
Published: December 2013

Significance
Plant cell walls contain significant amounts of the polysaccharides cellulose and hemicellulose, which can be depolymerized by enzymes to sugars and upgraded to renewable fuels and chemicals. Traditionally, enzymes for biomass depolymerization were based on naturally occurring hydrolytic enzymes, until the recent discovery of another natural enzymatic paradigm for carbohydrate deconstruction. Namely, lytic polysaccharide monooxygenases (LPMOs), long thought to be hydrolases or carbohydrate-binding modules, were revealed to be oxidative, copper-containing enzymes. These enzymes are receiving significant attention as they could revolutionize biomass deconstruction to upgradeable intermediates for renewable energy applications. Here, we apply quantum mechanical calculations to elucidate the oxidative reaction mechanism to offer predictions into how LPMOs function.
Evidence supporting an intentional Neandertal burial at La Chapelle-aux-Saints
Authors: Rendu William , Beauval Cédric , Crevecoeur Isabelle , Bayle Priscilla , Balzeau Antoine , Bismuth Thierry , Bourguignon Laurence , Delfour Géraldine , Faivre Jean-Philippe , Lacrampe-Cuyaubère François , Tavormina Carlotta , Todisco Dominique , Turq Alain , Maureille Bruno 
Published: December 2013

Significance
For several decades, scholars have questioned the existence of burial in Western Europe prior to the arrival of Anatomically Modern Humans. Therefore, an approach combining a global field recovery and the reexamination of the previously discovered Neandertal remains has been undertaken in the site of La Chapelle-aux-Saints (France), where the hypothesis of a Neandertal burial was raised for the first time. This project has concluded that the Neandertal of La Chapelle-aux-Saints was deposit in a pit dug by other members of its group and protected by a rapid covering from any disturbance. These discoveries attest the existence of West European Neandertal burial and of the Neandertal cognitive capacity to produce it.
Quantitation of the DNA tethering effect in long-range DNA looping in vivo and in vitro using the Lac and λ repressors
Authors: Priest David G. , Cui Lun , Kumar Sandip , Dunlap David D. , Dodd Ian B. , Shearwin Keith E. 
Published: December 2013

Significance
Proteins bound to DNA often interact with proteins bound elsewhere on the same DNA to regulate gene expression. The intervening DNA tethers the proteins near each other, making their interaction efficient and specific, but the importance of this tethering effect is poorly understood at large DNA separations. We quantitated tethering inside bacterial cells, using two different proteins at separations up to 10,000 bp, to show that tethering is strong enough to drive efficient interactions over these distances. The same interactions were ∼10-fold weaker outside cells, implying that cellular factors enhance tethering. However, tethering was lost at a DNA separation of 500,000 bp inside bacteria, indicating special mechanisms inside eukaryotic cells to provide efficient and specific interactions over such distances.