bluuebunny/crossref_metadata_2025_split huggingface dataset:
In vivo gastric detection of α‐synuclein inclusions in Parkinson's disease
Authors: Sánchez‐Ferro Álvaro , Rábano Alberto , Catalán María José , Rodríguez‐Valcárcel Fernando Canga , Díez Servando Fernández , Herreros‐Rodríguez Jaime , García‐Cobos Elvira , Álvarez‐Santullano Marina Mata , López‐Manzanares Lydia , Mosqueira Antonio J. , Desojo Lydia Vela , López‐Lozano Juan José , López‐Valdés Eva , Sánchez‐Sánchez Rafael , Molina‐Arjona José Antonio 
Published: August 2014

Abstractα‐Synuclein inclusions have been identified in the brain and some parts of the enteric nervous system in Parkinson's disease cases. We aimed to assess these inclusions in gastric mucosa samples from patients with symptomatic Parkinson's disease. Random biopsies were performed by gastroscopy in 28 patients with Parkinson's disease and in 29 age‐ and sex‐matched controls. Gastroscopy was performed to start enteral levodopa (l‐dopa) therapy in cases and for diagnostic purposes in controls (gastroesophageal reflux, anemia, and abdominal pain were the main indications). The clinical definition of cases and controls was made a priori. Six controls had data suggestive of “mild presymptomatic parkinsonism”. Biopsy specimens were immunostained for α‐synuclein. The neuropathological diagnosis was established post hoc. No differences were found in the baseline characteristics of the groups. Positive fibers for the α‐synuclein protein were observed in 17 of 28 (60.7%) Parkinson's disease patients, 1 of 23 controls (4.3%), and 1 of 6 (16.7%) cases of incident “mild presymptomatic parkinsonism.” Neuropathological diagnosis based on α‐synuclein immunostaining showed a sensitivity of 85% (95% confidence interval [CI] 62.1‐96.8), specificity of 95% (95% CI 76.2‐99.9) and area under the receiver operating characteristics curve (AUC) of 0.90 (95% CI 0.80‐1.00). No adverse events occurred. Detection of α‐synuclein inclusions in the gastric mucosa is a useful and safe tool providing in vivo evidence of the underlying neurodegenerative peripheral involvement linked to Parkinson's disease. Further studies are warranted to determine its pathophysiological implications. © 2014 International Parkinson and Movement Disorder Society
Simultaneous T1 and T2 quantification of the myocardium using cardiac balanced‐SSFP inversion recovery with interleaved sampling acquisition (CABIRIA)
Authors: Santini Francesco , Kawel‐Boehm N. , Greiser A. , Bremerich J. , Bieri O. 
Published: August 2014

PurposeTo develop a novel sequence for simultaneous quantification of T1 and T2 relaxation times in the myocardium based on the transient phase of the balanced steady‐state free precession.MethodsA new prototype sequence, named “cardiac balanced‐SSFP inversion recovery with interleaved sampling acquisition” (CABIRIA) was developed based on a single‐shot bSSFP readout following an inversion pulse. With this method, T1 and T2 values can be calculated from the analysis of signal evolution. The scan duration for a single slice in vivo was 8 heartbeats, thus feasible in a breath‐hold. The sequence was validated both in vitro by comparing it to conventional inversion recovery and multi‐echo spin‐echo methods and in 5 healthy volunteers by comparing it to the Modified Look‐Locker Inversion Recovery (MOLLI) sequence and to a T2 quantification sequence based on multi‐T2‐prepared bSSFP.ResultsThe method showed good agreement with conventional methods for both T1 and T2 measurements (concordance correlation coefficient ≥ 0.99) in vitro. In healthy volunteers the measured T1 values were 1227 ± 68 ms and T2 values 37.9 ± 2.4 ms, with similar inter‐ and intrasubject variability with respect to existing methods.ConclusionThe proposed CABIRIA method enables simultaneous quantification of myocardial T1 and T2 values with good accuracy and precision. Magn Reson Med 74:365–371, 2015. © 2014 Wiley Periodicals, Inc.
Power spectral changes of the superimposed M wave during isometric voluntary contractions of increasing strength
Authors: Rodriguez‐Falces Javier , Place Nicolas 
Published: August 2014

ABSTRACTIntroduction: We examined the power spectral changes of the compound muscle action potential (M wave) evoked during isometric contractions of increasing strength. Methods: Surface electromyography (sEMG) of the vastus lateralis and medialis was recorded from 20 volunteers who performed 4‐s step‐wise isometric contractions of different intensities. A maximal M wave was elicited by a single stimulus to the femoral nerve and superimposed on the voluntary contractions. The spectral characteristics (Fmean and Fmedian) of sEMG and M‐wave signals were calculated. Results: M‐wave spectral indicators increased systematically with contraction intensity up to 60% MVC and then leveled off at higher forces. Over the 10–60% MVC range, the increase in spectral indicators was 3 times higher for M waves (36%) than for sEMG (12%). Conclusions: The consistent increase in M‐wave spectral characteristics with force is due to the fact that the number of motor units recruited by the superimposed supramaximal stimulus is approximately stable. Muscle Nerve 51: 580–591, 2015
The National ALS Registry: A recruitment tool for research
Authors: Malek Angela M. , Stickler David E. , Antao Vinicius C. , Horton D. Kevin 
Published: August 2014

ABSTRACTIntroduction: Subject recruitment is critical for understanding fatal diseases like ALS, however linking patients with researchers can be challenging. The U.S. population‐based National ALS Registry allows recruitment of persons with ALS (PALS) for research opportunities. Methods: The Registry's Research Notification Mechanism was used to recruit PALS aged ≥21 years; participants completed a Web‐based epidemiologic survey. PALS (n = 2,232) were sent an email describing the study, and 268 surveys were completed. Results: The mean age (± SD) of eligible participants was 57.7 ± 9.3 years for men and 61.5 ± 8.9 for women. Most were men (63%) and Caucasian (92%). Of 256 potentially eligible participants, 37.5% (n = 96) returned an authorization to disclose protected health information. ALS was confirmed for 94% (83/88) from physician responses. Conclusions: This analysis demonstrates the National ALS Registry's usefulness in recruiting PALS for research. This recruitment source can potentially foster the discovery of better treatment options and therapies, and of prevention strategies. Muscle Nerve 50: 830–834, 2014
Structural and mutational studies on an aldo‐keto reductase AKR5C3 from Gluconobacter oxydans
Authors: Liu Xu , Wang Chao , Zhang Lujia , Yao Zhiqiang , Cui Dongbing , Wu Liang , Lin Jinping , Yuan Yu‐Ren Adam , Wei Dongzhi 
Published: August 2014

AbstractAn aldo‐keto reductase AKR5C3 from Gluconobacter oxydans (designated as Gox0644) is a useful enzyme with various substrates, including aldehydes, diacetyl, keto esters, and α‐ketocarbonyl compounds. The crystal structures of AKR5C3 in apoform in complex with NADPH and the D53A mutant (AKR5C3‐D53A) in complex with NADPH are presented herein. Structure comparison and site‐directed mutagenesis combined with biochemical kinetics analysis reveal that the conserved Asp53 in the AKR5C3 catalytic tetrad has a crucial role in securing active pocket conformation. The gain‐of‐function Asp53 to Ala mutation triggers conformational changes on the Trp30 and Trp191 side chains, improving NADPH affinity to AKR5C3, which helps increase catalytic efficiency. The highly conserved Trp30 and Trp191 residues interact with the nicotinamide moiety of NADPH and help form the NADPH‐binding pocket. The AKR5C3‐W30A and AKR5C3‐W191Y mutants show decreased activities, confirming that both residues facilitate catalysis. Residue Trp191 is in the loop structure, and the AKR5C3‐W191Y mutant does not react with benzaldehyde, which might also determine substrate recognition. Arg192, which is involved in the substrate binding, is another important residue. The introduction of R192G increases substrate‐binding affinity by improving hydrophobicity in the substrate‐binding pocket. These results not only supplement the AKRs superfamily with crystal structures but also provide useful information for understanding the catalytic properties of AKR5C3 and guiding further engineering of this enzyme.
Grip it and rip it: Structural mechanisms of DNA helicase substrate binding and unwinding
Authors: Bhattacharyya Basudeb , Keck James L. 
Published: August 2014

AbstractMaintenance and faithful transmission of genomic information depends on the efficient execution of numerous DNA replication, recombination, and repair pathways. Many of the enzymes that catalyze steps within these pathways require access to sequence information that is buried in the interior of the DNA double helix, which makes DNA unwinding an essential cellular reaction. The unwinding process is mediated by specialized molecular motors called DNA helicases that couple the chemical energy derived from nucleoside triphosphate hydrolysis to the otherwise non‐spontaneous unwinding reaction. An impressive number of high‐resolution helicase structures are now available that, together with equally important mechanistic studies, have begun to define the features that allow this class of enzymes to function as molecular motors. In this review, we explore the structural features within DNA helicases that are used to bind and unwind DNA. We focus in particular on “aromatic‐rich loops” that allow some helicases to couple single‐stranded DNA binding to ATP hydrolysis and “wedge/pin” elements that provide mechanical tools for DNA strand separation when connected to translocating motor domains.
Conformational and aggregation properties of the 1–93 fragment of apolipoprotein A‐I
Authors: Petrlova Jitka , Bhattacherjee Arnab , Boomsma Wouter , Wallin Stefan , Lagerstedt Jens O. , Irbäck Anders 
Published: August 2014

AbstractSeveral disease‐linked mutations of apolipoprotein A‐I, the major protein in high‐density lipoprotein (HDL), are known to be amyloidogenic, and the fibrils often contain N‐terminal fragments of the protein. Here, we present a combined computational and experimental study of the fibril‐associated disordered 1–93 fragment of this protein, in wild‐type and mutated (G26R, S36A, K40L, W50R) forms. In atomic‐level Monte Carlo simulations of the free monomer, validated by circular dichroism spectroscopy, we observe changes in the position‐dependent β‐strand probability induced by mutations. We find that these conformational shifts match well with the effects of these mutations in thioflavin T fluorescence and transmission electron microscopy experiments. Together, our results point to molecular mechanisms that may have a key role in disease‐linked aggregation of apolipoprotein A‐I.
A novel comprehensive steganalysis of transmission control protocol/Internet protocol covert channels based on protocol behaviors and support vector machine
Authors: Shen Yao , Huang Liusheng , Lu Xiaorong , Yang Wei 
Published: August 2014

AbstractCovert channels are malicious conversations disguised in legitimate network communications, allowing information leak to the unauthorized or unknown receiver. Various network steganographic schemes that modify the header fields of transmission control protocol/Internet protocol (TCP/IP) have been proposed in recent years. People before conducted detection research based on the surface content of the header field and did not take into account the differences between the behavior characters of covert channels and the inherent behavior regularities of the header fields. Up to date, there is little comprehensive research on the steganalysis against the storage covert channels. In this paper, we focus on the detection of storage covert channels and introduce a novel comprehensive detection method based on the protocol behaviors. The protocol behavior characters are utilized to evaluate the regularities or correlations of header fields between adjacent packets according to the conventional use. First, the behavior features of the header fields in TCP/IP are extracted; a support vector machine is then applied to the behavior feature sets for discovering the existence of covert channels. Some recognized covert channel tools are detected in our detection experiment. Experimental results and discussion show that our detection method is of effectiveness. Copyright © 2014 John Wiley & Sons, Ltd.
Concise Review: Understanding Clonal Dynamics in Homeostasis and Injury Through Multicolor Lineage Tracing
Authors: Roy Edwige , Neufeld Zoltan , Livet Jean , Khosrotehrani Kiarash 
Published: August 2014

Abstract
Lineage tracing is an essential tool to study stem cell fate. Although traditional lineage tracing techniques have considerably advanced our understanding of stem cell behavior, they pose significant limitations for identification and longitudinal tracking of the progeny of individual stem cells, to compare their behaviors. This is of importance given the well-established heterogeneity among stem cells both in terms of potentialities and proliferative capacities. The recent development of multicolor genetic reporters addressable to specific cell populations largely overcomes these issues. These new “rainbow” technologies provide increased resolution in clonal identification and offer the possibility to study the relative distribution, contacts, tiled arrangement, and competitive interactions among cells or groups of cells of the same type. Stem Cells 2014;32:3046–3054
The effect of online collaboration on middle school student science misconceptions as an aspect of science literacy
Authors: Wendt Jillian L. , Rockinson‐Szapkiw Amanda 
Published: August 2014

AbstractThis quantitative, quasi‐experimental pretest/posttest control group design examined the effects of online collaborative learning on middle school students' science literacy. For a 9‐week period, students in the control group participated in collaborative face‐to‐face activities whereas students in the experimental group participated in online collaborative activities using the Edmodo educational platform. Students at a public middle school in central Virginia completed both a pretest and a posttest consisting of the Misconceptions‐Oriented Standards‐Based Assessment Resources for Teachers (MOSART) assessment to measure science misconceptions as an aspect of science literacy. Results indicated that the students who participated in collaborative activities in the traditional classroom had fewer science misconceptions than students who participated in collaborative activities in the online environment. Moreover, from pretest to posttest, the students in the experimental group increased in their science misconceptions. Suggestions for practice and future research are discussed in light of these results. © 2014 Wiley Periodicals, Inc. J Res Sci Teach 51: 1103–1118, 2014
Wake modelling of a VAWT in dynamic stall: impact on the prediction of flow and induction fields
Authors: Zanon Alessandro , Giannattasio Pietro , Simão Ferreira Carlos J. 
Published: August 2014

AbstractDynamic stall is a relevant phenomenon in the design and operation of a vertical axis wind turbine (VAWT) as it impacts loading, control and wake dynamics. Although streamtube models and single‐wake vortex models are commonly used for VAWT simulation, they either do not explicitly simulate the distribution of vorticity in the wake (streamtube models) or simplify it into a single‐wake release point (single‐wake vortex models). This can lead to inaccurate predictions of the vorticity distribution and wake dynamics, and therefore of the induction field, rotor loading and wake development, including wake mixing and re‐energizing. In this work, we use a double‐wake panel model developed for the simulation of dynamic stall in a VAWT to analyse (i) what is the flow field in dynamic stall, including the induction field, (ii) what is the error due to assuming a simplified wake, in both vorticity distribution and induction and (iii) how an incorrect simulation of the vorticity distribution can affect the prediction of the dynamics of the near and far wake. The results demonstrate that for mild separation (tip speed ratio λ≥3), single‐wake models can produce acceptable results. However, for lower tip speed ratios (λ < 3), the inaccuracy in the prediction of loads, induction field and vorticity distribution becomes significant because of an inadequate representation of the wake dynamics. These results imply that using lower order models can lead to inaccurate estimations of loads, performance and power control requirements at low tip speed ratios. Copyright © 2014 John Wiley & Sons, Ltd.
Diurnal space use and seasonal movement patterns of greater sage‐grouse in Northeastern California
Authors: Davis Dawn M. , Reese Kerry P. , Gardner Scott C. 
Published: August 2014

ABSTRACTGreater sage‐grouse (Centrocercus urophasianus) populations have declined throughout their geographic range, are considered a candidate species under the U.S. Endangered Species Act, and are regarded as a species of special concern in California, USA. Demographic parameters have been well‐documented for this species, but populations occupying the western periphery of their range have largely remained unstudied. We examined diurnal space use and seasonal movement patterns of greater sage‐grouse in northeastern California. We collected 3,072 diurnal locations from 132 greater sage‐grouse radiomarked between 2007 and 2009. Greater sage‐grouse in our study used large areas and exhibited wide‐ranging seasonal movements. Female greater sage‐grouse sought suitable nesting habitat within 5 km of a lek; however, of the 27% (n = 20/74) of females nesting >5 km from an occupied lek site, 53% (n = 10/19) nested successfully. This suggests the proportion of nesting females located >5 km from an active lek is important for population viability in northeastern California. Moreover, the annual range of movements indicates that conserving greater sage‐grouse habitat within 5 km of active lek sites is insufficient to maintain long‐term population persistence. Larger areas of habitat are needed to conserve greater sage‐grouse in northeastern California to accommodate seasonal movements, annual variation in habitat requirements, and to maintain connectivity between lek complexes. © 2014 The Wildlife Society.
Ten years of the international Pompe survey: patient reported outcomes as a reliable tool for studying treated and untreated children and adults with non‐classic Pompe disease
Authors: van der Meijden J. C. , Güngör D. , Kruijshaar M. E. , Muir A. D. J. , Broekgaarden H. A. , van der Ploeg A. T. 
Published: August 2014

AbstractPompe disease is a rare, progressive lysosomal storage disorder for which enzyme therapy (ERT) became available in 2006. Four years earlier, the IPA/Erasmus MC survey, an international longitudinal prospective survey, was established to collect information on the natural course of the disease and its burden on patients. The survey is a collaboration between Erasmus MC University Medical Center and the International Pompe Association (IPA) and comprises an annual questionnaire that was specifically designed to assess the symptoms and problems of the disease. Here we review our results of over 10 years of follow‐up, and discuss the survey's contribution to the field. Tracking 408 Pompe patients between 2002 and 2013, the cumulative data reveals the broad range of clinical manifestations that interfere with patients’ lives. The survey allowed us to quantify the rate of disease progression and the positive effects of ERT on patients’ quality of life, fatigue, and participation in daily life. Furthermore, it showed for the first time that survival is reduced in adult Pompe disease and improved by ERT. Our results show that a patient survey can serve as a valuable and reliable tool for obtaining quantifiable information on the natural course of a rare disease and on the effects of therapy in a large cohort over a very long time. Most importantly, by working with patient reported outcomes, the survey provides the data that is truly relevant to the patient and complementary to clinical datasets.
Adenylosuccinate lyase deficiency
Authors: Jurecka Agnieszka , Zikanova Marie , Kmoch Stanislav , Tylki‐Szymańska Anna 
Published: August 2014

AbstractAdenylosuccinate lyase ADSL) deficiency is a defect of purine metabolism affecting purinosome assembly and reducing metabolite fluxes through purine de novo synthesis and purine nucleotide recycling pathways. Biochemically this defect manifests by the presence in the biologic fluids of two dephosphorylated substrates of ADSL enzyme: succinylaminoimidazole carboxamide riboside (SAICAr) and succinyladenosine (S‐Ado). More than 80 individuals with ADSL deficiency have been identified, but incidence of the disease remains unknown. The disorder shows a wide spectrum of symptoms from slowly to rapidly progressing forms. The fatal neonatal form has onset from birth and presents with fatal neonatal encephalopathy with a lack of spontaneous movement, respiratory failure, and intractable seizures resulting in early death within the first weeks of life. Patients with type I (severe form) present with a purely neurologic clinical picture characterized by severe psychomotor retardation, microcephaly, early onset of seizures, and autistic features. A more slowly progressing form has also been described (type II, moderate or mild form), as having later onset, usually within the first years of life, slight to moderate psychomotor retardation and transient contact disturbances. Diagnosis is facilitated by demonstration of SAICAr and S‐Ado in extracellular fluids such as plasma, cerebrospinal fluid and/or followed by genomic and/or cDNA sequencing and characterization of mutant proteins. Over 50 ADSL mutations have been identified and their effects on protein biogenesis, structural stability and activity as well as on purinosome assembly were characterized. To date there is no specific and effective therapy for ADSL deficiency.
Assessment of fitness costs of resistance against the parasitoid Leptopilina victoriae in Drosophila bipectinata
Authors: Takigahira Tomohiro , Suwito Awit , Kimura Masahito T. 
Published: August 2014

AbstractHow insects evolve resistance or counter‐resistance against antagonists is a basic issue in the study of host‐parasitoid coevolution. One of the factors that affect their coevolution is fitness costs of resistance and counter‐resistance. Here, we assess fitness costs of resistance against the parasitoid Leptopilina victoriae in Drosophila bipectinata on the basis of selection experiments. We made a base population by mixing three geographic fly populations that differed in resistance. The established base population was divided into four populations, two for selection of resistance against a L. victoriae population and two for control. Resistance increased rapidly in response to selection and reached a very high level within four generations in the selected populations, while resistance of the control populations remained low during 20 generations. High resistance of the selected populations was maintained at least for 10 generations after selection was stopped. Both selected populations had lower female longevity than the control populations, and at least one of the selected populations had shorter thorax length, lower female desiccation tolerance and adult heat tolerance than both or either of the control populations. On the other hand, the selected populations had higher male starvation tolerance and longevity than the control populations. There were no significant differences in resistance against another population of L. victoriae and two other parasitoid species between the selected and control populations. These results suggest that the resistance against the L. victoriae population in D. bipectinata may incur some but not so high costs and act parasitoid‐species‐ and/or parasitoid‐population‐specifically.