bluuebunny/crossref_metadata_2025_split huggingface dataset:
A Clear Difference Between the Outcomes After a Major Hepatectomy With and Without an Extrahepatic Bile Duct Resection
Authors: Takagi Takehiro , Yokoyama Yukihiro , Kokuryo Toshio , Ebata Tomoki , Ando Masahiko , Nagino Masato 
Published: October 2016

AbstractBackgroundThe procedure of a simple hepatectomy and a hepatectomy with an extrahepatic bile duct resection and subsequent choledocho‐jejunostomy is largely different. However, these two procedures are sometimes included in the same category. There are no studies comparing postoperative course and liver regeneration rate after a major hepatectomy with and without an extrahepatic bile duct resection.MethodsWe retrospectively reviewed medical records of 245 patients who underwent a right hepatectomy (RH, n = 55) or RH with an extrahepatic bile duct resection (RHEBR, n = 190). Postoperative complications, including incidence of posthepatectomy liver failure (PHLF) and hepatic regeneration rates after surgery, were evaluated.ResultsThe incidence of PHLF was considerably higher in the RHEBR group than in the RH group (39.5 vs. 16.4 %, p = 0.001). The percentage of newly regenerated liver volume after the hepatectomies on postoperative days 6–8 was significantly lower in the RHEBR group than in the RH group (14.0 % in the RH; 7.9 % in the RHEBR group, p < 0.001). Especially type of surgery (RHEBR) was the only independent risk factor for an impaired liver regeneration rate by univariate and multivariate analyses. Furthermore, estimated hepatic regeneration rate by stepwise linear regression analysis in the RHEBR group was 7.1 % lower (95 % confidence interval 1.8–12.3, p = 0.011) than in the RH group.ConclusionThese results suggest that the procedure of extrahepatic bile duct resection has a possibility of adverse impact on the postoperative outcome after major hepatectomy.
Cardiomyopathy as presenting sign of glycogenin‐1 deficiency—report of three cases and review of the literature
Authors: Hedberg‐Oldfors Carola , Glamuzina Emma , Ruygrok Peter , Anderson Lisa J. , Elliott Perry , Watkinson Oliver , Occleshaw Chris , Abernathy Malcolm , Turner Clinton , Kingston Nicola , Murphy Elaine , Oldfors Anders 
Published: October 2016

AbstractWe describe a new type of cardiomyopathy caused by a mutation in the glycogenin‐1 gene (GYG1). Three unrelated male patients aged 34 to 52 years with cardiomyopathy and abnormal glycogen storage on endomyocardial biopsy were homozygous for the missense mutation p.Asp102His in GYG1. The mutated glycogenin‐1 protein was expressed in cardiac tissue but had lost its ability to autoglucosylate as demonstrated by an in vitro assay and western blot analysis. It was therefore unable to form the primer for normal glycogen synthesis. Two of the patients showed similar patterns of heart dilatation, reduced ejection fraction and extensive late gadolinium enhancement on cardiac magnetic resonance imaging. These two patients were severely affected, necessitating cardiac transplantation. The cardiomyocyte storage material was characterized by large inclusions of periodic acid and Schiff positive material that was partly resistant to alpha‐amylase treatment consistent with polyglucosan. The storage material had, unlike normal glycogen, a partly fibrillar structure by electron microscopy. None of the patients showed signs or symptoms of muscle weakness but a skeletal muscle biopsy in one case revealed muscle fibres with abnormal glycogen storage. Glycogenin‐1 deficiency is known as a rare cause of skeletal muscle glycogen storage disease, usually without cardiomyopathy. We demonstrate that it may also be the cause of severe cardiomyopathy and cardiac failure without skeletal muscle weakness. GYG1 should be included in cardiomyopathy gene panels.
Newborn screening for mucopolysaccharidoses: a pilot study of measurement of glycosaminoglycans by tandem mass spectrometry
Authors: Kubaski Francyne , Mason Robert W. , Nakatomi Akiko , Shintaku Haruo , Xie Li , van Vlies Naomi N. , Church Heather , Giugliani Roberto , Kobayashi Hironori , Yamaguchi Seiji , Suzuki Yasuyuki , Orii Tadao , Fukao Toshiyuki , Montaño Adriana M. , Tomatsu Shunji 
Published: October 2016

AbstractBackgroundMucopolysaccharidoses (MPS) are a group of inborn errors of metabolism that are progressive and usually result in irreversible skeletal, visceral, and/or brain damage, highlighting a need for early diagnosis.MethodsThis pilot study analyzed 2862 dried blood spots (DBS) from newborns and 14 DBS from newborn patients with MPS (MPS I, n = 7; MPS II, n = 2; MPS III, n = 5). Disaccharides were produced from polymer GAGs by digestion with chondroitinase B, heparitinase, and keratanase II. Heparan sulfate (0S, NS), dermatan sulfate (DS) and mono‐ and di‐sulfated KS were measured by liquid chromatography tandem mass spectrometry (LC‐MS/MS). Median absolute deviation (MAD) was used to determine cutoffs to distinguish patients from controls. Cutoffs were defined as median + 7× MAD from general newborns.ResultsThe cutoffs were as follows: HS‐0S > 90 ng/mL; HS‐NS > 23 ng/mL, DS > 88 ng/mL; mono‐sulfated KS > 445 ng/mL; di‐sulfated KS > 89 ng/mL and ratio di‐KS in total KS > 32 %. All MPS I and II samples were above the cutoffs for HS‐0S, HS‐NS, and DS, and all MPS III samples were above cutoffs for HS‐0S and HS‐NS. The rate of false positives for MPS I and II was 0.03 % based on a combination of HS‐0S, HS‐NS, and DS, and for MPS III was 0.9 % based upon a combination of HS‐0S and HS‐NS.ConclusionsCombination of levels of two or more different GAGs improves separation of MPS patients from unaffected controls, indicating that GAG measurements are potentially valuable biomarkers for newborn screening for MPS.
A Pilot Study of Fragile X Syndrome Screening in Pregnant Women and Women Planning Pregnancy: Implementation, Acceptance, Awareness, and Geographic Factors
Authors: Alfaro Arenas Ramona , Rosell Andreo Jordi , Heine Suñer Damián ,   Group for the study of FXS in the Balearic Islands
Published: October 2016

AbstractWe report herein results of a study performed in the Balearic Islands which had the following goals: 1) Determine the proportion of pregnant or non‐pregnant women planning pregnancy, who would choose to undergo a screening test for Fragile X Syndrome (FXS), if it is accompanied by the appropriate information; 2) Assess satisfaction and any increase in stress among women who participate in screening; 3) Collect epidemiological information about the incidence of the disease in our population; and 4) Collect demographic and health history data and assess participants’ awareness of the disease. Screening was performed on 3,731 pregnant and non‐pregnant women of childbearing age and the results indicate: a very high voluntary rate of participation; a high level of self‐reported satisfaction and low levels of stress because of the test; a very high incidence of premutation (1/106) in our population; and a low level of awareness about the existence of FXS (25 %). Additional findings indicate no significant correlation between self‐reported health history and premutation detection, and the high premutation incidence does not seem to be specific to the indigenous Balearic population. Based on these results, we discuss the pros and cons of an implementation of preconception and pregnant women screening for FXS within a public health screening program.
A single-machine scheduling problem with multiple unavailability constraints: A mathematical model and an enhanced variable neighborhood search approach
Authors: Yazdani Maziar , Khalili Seyed Mohammad , Babagolzadeh Mahla , Jolai Fariborz 
Published: October 2016

AbstractThis research focuses on a scheduling problem with multiple unavailability periods and distinct due dates. The objective is to minimize the sum of maximum earliness and tardiness of jobs. In order to optimize the problem exactly a mathematical model is proposed. However due to computational difficulties for large instances of the considered problem a modified variable neighborhood search (VNS) is developed. In basic VNS, the searching process to achieve to global optimum or near global optimum solution is totally random, and it is known as one of the weaknesses of this algorithm. To tackle this weakness, a VNS algorithm is combined with a knowledge module. In the proposed VNS, knowledge module extracts the knowledge of good solution and save them in memory and feed it back to the algorithm during the search process. Computational results show that the proposed algorithm is efficient and effective.Highlights A scheduling problem with multiple unavailability is proposed and formulated. A knowledge-based variable neighborhood search algorithm is proposed. Extensive computational tests confirm the good capability of the proposed model and algorithm.
Spatio-temporal changes in glacier-wide mass balance quantified by optical remote sensing on 30 glaciers in the French Alps for the period 1983–2014
Authors: RABATEL ANTOINE , DEDIEU JEAN PIERRE , VINCENT CHRISTIAN 
Published: October 2016

AbstractRemote sensing is a powerful method to reconstruct annual mass-balance series over past decades by exploiting archives of available images, as well as to study glaciers in inaccessible regions. We present the application of a methodological framework based only on optical satellite images to retrieve glacier-wide annual mass balances for 30 glaciers in the French Alps. The glacier-wide annual mass balance for the period 1983–2014 was reconstructed by combining changes in glacier volumes computed from remote-sensing derived DEMs with annual measurements of the snow line altitude on satellite images. Data from direct observations on two of the glaciers confirmed the accuracy of the annual mass balances quantified by remote sensing with an average difference of ~0.3 m w.e., within the uncertainty range of the methods. Our results confirm the significant increase in mass loss since the early 2000s, with a difference >1 m w.e. a−1 between the periods 1983–2002 and 2003–14. The region-wide mass balance for the French Alps over the period 1979–2011 was −0.66 ± 0.27 m w.e. a−1, close to that of the European Alps. We also show that changes in glacier surface area or length are not representative of changes in mass balance at the scale of a few decades.
Large-sized Early Permian “caninioid” corals from the Karavanke Mountains, Slovenia
Authors: Kossovaya Olga L. , Novak Matevž , Weyer Dieter 
Published: October 2016

AbstractA new monospecific “caninioid” genus,Preisingerellan. gen., from the lower Permian of the Karavanke Mountains (Southern Alps, Slovenia) is erected. The type species isPreisingerella stegovnikensisn. sp. The new taxon can be differentiated from otherCaninia-type genera by its specific ontogeny and features of its dissepimentarium. Corals with such morphology had a wide distribution during the Carboniferous and early Permian, occurring in mostly shallow-water carbonate rocks. The phylogenetic relationships within this group are mostly unclear due to similarities in the adult stages. The earlier stages reveal the main distinguishing features that are decisive for a generic assignment, but these have rarely been well preserved and properly considered. The new taxon is compared with related genera of the Cyathopsidae and species ofCaninellaGorskiy, 1938 characterized by lateral dissepiments. Large numbers of specimens of the new species, representing a monospecific assemblage, have been collected from the Born Formation at Mt. Stegovnik. Sedimentological and microfacies characteristics, as well as macro- and microfossil assemblages, underline this correlation. The fusulinoidean assemblage of the Born Formation, withSphaeroschwagerina carniolica(Kahler and Kahler, 1937), as the predominant species, corresponds to the time span between theSphaeroschwagerina moelleri-Schwagerina fecundaandPseudofusulina moellerizones, indicating a late Asselian to early Sakmarian age in the Southern Urals.
The phacopid trilobiteEchidnops taphomimusn. sp. from the Lower Devonian of Argentina: insights into infaunal molting, eye architecture and geographic distribution
Authors: Rustán Juan José , Balseiro Diego 
Published: October 2016

AbstractSome Silurian-Devonian Argentinian trilobites characterized by infaunal behavior during molting are considered. After a taxonomic reappraisal, a species previously referred to a lineage of the phacopidPaciphacopsis proposed asEchidnops taphomimusnew species, from the Lower Devonian (probably late Lochkovian) of the Talacasto Formation, Argentine Precordillera. The visual surface ofE.taphomimusindicates that a irregular pattern of lens arrangement, typical of early phacopids such as the OrdovicianOrmathops, can also be recognized in more derived Devonian relatives, providing new insights on some evolutionary aspects of visual development.Echidnopsis recognized in Australia and Argentina, recording an unusual distribution pattern in trilobites from the Lower Devonian of southern South America, otherwise mostly linked to faunas of related Gondwanan, austral circum-polar Devonian basins of the Malvinokaffric Realm. In accordance with proposed increasing predation pressure in the context of the Mid Paleozoic Marine Revolution, evidence indicates that the infaunal molting behavior, as defensive strategy, arose in several trilobite groups during Silurian-Devonian times, rather than in a single endemic lineage ofPaciphacopsspecies.
Theory of pulsar magnetosphere and wind
Authors: Pétri Jérôme 
Published: October 2016

Neutron stars are fascinating astrophysical objects immersed in strong gravitational and electromagnetic fields, at the edge of our current theories. These stars manifest themselves mostly as pulsars, emitting a timely very stable and regular electromagnetic signal. Even though discovered almost fifty years ago, they still remain mysterious compact stellar objects. In this review, we summarize the most fundamental theoretical aspects of neutron star magnetospheres and winds. The main competing models explaining their radiative properties like multi-wavelength pulse shapes and spectra and the underlying physical processes such as pair creation and radiation mechanisms are scrutinized. A global but still rather qualitative picture slowly emerges thanks to recent advances in numerical simulations on the largest scales. However considerations about pulsar magnetospheres remain speculative. For instance, the exact composition of the magnetospheric plasma is not yet known. Is it solely filled with a mixture of$e^{\pm }$ leptons or does it contain a non-negligible fraction of protons and/or ions? Is it almost entirely filled or mostly empty except for some small anecdotal plasma filled regions? Answers to these questions will strongly direct the description of the magnetosphere to seemingly contradictory results leading sometimes to inconsistencies. Nevertheless, accounts are given as to the latest developments in the theory of pulsar magnetospheres and winds, the existence of a possible electrosphere and physical insight obtained from related observational signatures of multi-wavelength pulsed emission.
An interdigit signalling centre instructs coordinate phalanx-joint formation governed by 5′Hoxd–Gli3 antagonism
Authors: Huang Bau-Lin , Trofka Anna , Furusawa Aki , Norrie Jacqueline L. , Rabinowitz Adam H. , Vokes Steven A. , Mark Taketo M. , Zakany Jozsef , Mackem Susan 
Published: October 2016

AbstractThe number of phalanges and joints are key features of digit ‘identity’ and are central to limb functionality and evolutionary adaptation. Prior chick work indicated that digit phalanges and their associated joints arise in a different manner than the more sparsely jointed long bones, and their identity is regulated by differential signalling from adjacent interdigits. Currently, there is no genetic evidence for this model, and the molecular mechanisms governing digit joint specification remain poorly understood. Using genetic approaches in mouse, here we show that functional 5′Hoxd–Gli3 antagonism acts indirectly, through Bmp signalling from the interdigital mesenchyme, to regulate specification of joint progenitors, which arise in conjunction with phalangeal precursors at the digit tip. Phalanx number, although co-regulated, can be uncoupled from joint specification. We propose that 5′Hoxd genes and Gli3 are part of an interdigital signalling centre that sets net Bmp signalling levels from different interdigits to coordinately regulate phalanx and joint formation.
Bimodal antagonism of PKA signalling by ARHGAP36
Authors: Eccles Rebecca L. , Czajkowski Maciej T. , Barth Carolin , Müller Paul Markus , McShane Erik , Grunwald Stephan , Beaudette Patrick , Mecklenburg Nora , Volkmer Rudolf , Zühlke Kerstin , Dittmar Gunnar , Selbach Matthias , Hammes Annette , Daumke Oliver , Klussmann Enno , Urbé Sylvie , Rocks Oliver 
Published: October 2016

AbstractProtein kinase A is a key mediator of cAMP signalling downstream of G-protein-coupled receptors, a signalling pathway conserved in all eukaryotes. cAMP binding to the regulatory subunits (PKAR) relieves their inhibition of the catalytic subunits (PKAC). Here we report that ARHGAP36 combines two distinct inhibitory mechanisms to antagonise PKA signalling. First, it blocks PKAC activity via a pseudosubstrate motif, akin to the mechanism employed by the protein kinase inhibitor proteins. Second, it targets PKAC for rapid ubiquitin-mediated lysosomal degradation, a pathway usually reserved for transmembrane receptors. ARHGAP36 thus dampens the sensitivity of cells to cAMP. We show that PKA inhibition by ARHGAP36 promotes derepression of the Hedgehog signalling pathway, thereby providing a simple rationale for the upregulation of ARHGAP36 in medulloblastoma. Our work reveals a new layer of PKA regulation that may play an important role in development and disease.
Striatal prediction errors support dynamic control of declarative memory decisions
Authors: Scimeca Jason M. , Katzman Perri L. , Badre David 
Published: October 2016

AbstractAdaptive memory requires context-dependent control over how information is retrieved, evaluated and used to guide action, yet the signals that drive adjustments to memory decisions remain unknown. Here we show that prediction errors (PEs) coded by the striatum support control over memory decisions. Human participants completed a recognition memory test that incorporated biased feedback to influence participants’ recognition criterion. Using model-based fMRI, we find that PEs—the deviation between the outcome and expected value of a memory decision—correlate with striatal activity and predict individuals’ final criterion. Importantly, the striatal PEs are scaled relative to memory strength rather than the expected trial outcome. Follow-up experiments show that the learned recognition criterion transfers to free recall, and targeting biased feedback to experimentally manipulate the magnitude of PEs influences criterion consistent with PEs scaled relative to memory strength. This provides convergent evidence that declarative memory decisions can be regulated via striatally mediated reinforcement learning signals.
Thermally-nucleated self-assembly of water and alcohol into stable structures at hydrophobic interfaces
Authors: Voïtchovsky Kislon , Giofrè Daniele , José Segura Juan , Stellacci Francesco , Ceriotti Michele 
Published: October 2016

AbstractAt the interface with solids, the mobility of liquid molecules tends to be reduced compared with bulk, often resulting in increased local order due to interactions with the surface of the solid. At room temperature, liquids such as water and methanol can form solvation structures, but the molecules remain highly mobile, thus preventing the formation of long-lived supramolecular assemblies. Here we show that mixtures of water with methanol can form a novel type of interfaces with hydrophobic solids. Combiningin situatomic force microscopy and multiscale molecular dynamics simulations, we identify solid-like two-dimensional interfacial structures that nucleate thermally, and are held together by an extended network of hydrogen bonds. On graphite, nucleation occurs above ∼35 °C, resulting in robust, multilayered nanoscopic patterns. Our findings could have an impact on many fields where water-alcohol mixtures play an important role such as fuel cells, chemical synthesis, self-assembly, catalysis and surface treatments.
Nanoscale probing of electron-regulated structural transitions in silk proteins by near-field IR imaging and nano-spectroscopy
Authors: Qin Nan , Zhang Shaoqing , Jiang Jianjuan , Corder Stephanie Gilbert , Qian Zhigang , Zhou Zhitao , Lee Woonsoo , Liu Keyin , Wang Xiaohan , Li Xinxin , Shi Zhifeng , Mao Ying , Bechtel Hans A. , Martin Michael C. , Xia Xiaoxia , Marelli Benedetto , Kaplan David L. , Omenetto Fiorenzo G. , Liu Mengkun , Tao Tiger H. 
Published: October 2016

AbstractSilk protein fibres produced by silkworms and spiders are renowned for their unparalleled mechanical strength and extensibility arising from their high-β-sheet crystal contents as natural materials. Investigation of β-sheet-oriented conformational transitions in silk proteins at the nanoscale remains a challenge using conventional imaging techniques given their limitations in chemical sensitivity or limited spatial resolution. Here, we report on electron-regulated nanoscale polymorphic transitions in silk proteins revealed by near-field infrared imaging and nano-spectroscopy at resolutions approaching the molecular level. The ability to locally probe nanoscale protein structural transitions combined with nanometre-precision electron-beam lithography offers us the capability to finely control the structure of silk proteins in two and three dimensions. Our work paves the way for unlocking essential nanoscopic protein structures and critical conditions for electron-induced conformational transitions, offering new rules to design protein-based nanoarchitectures.
Non-enzymatic pyridine ring formation in the biosynthesis of the rubrolone tropolone alkaloids
Authors: Yan Yijun , Yang Jing , Yu Zhiyin , Yu Mingming , Ma Ya-Tuan , Wang Li , Su Can , Luo Jianying , Horsman Geoffrey P. , Huang Sheng-Xiong 
Published: October 2016

AbstractThe pyridine ring is a potent pharmacophore in alkaloid natural products. Nonetheless, its biosynthetic pathways are poorly understood. Rubrolones A and B are tropolone alkaloid natural products possessing a unique tetra-substituted pyridine moiety. Here, we report the gene cluster and propose a biosynthetic pathway for rubrolones, identifying a key intermediate that accumulates upon inactivation of sugar biosynthetic genes. Critically, this intermediate was converted to the aglycones of rubrolones by non-enzymatic condensation and cyclization with either ammonia or anthranilic acid to generate the respective pyridine rings. We propose that this non-enzymatic reaction occurs via hydrolysis of the key intermediate, which possesses a 1,5-dione moiety as an amine acceptor capable of cyclization. This study suggests that 1,5-dione moieties may represent a general strategy for pyridine ring biosynthesis, and more broadly highlights the utility of non-enzymatic diversification for exploring and expanding natural product chemical space.