bluuebunny/crossref_metadata_2025_split huggingface dataset:
The phenotypic spectrum of DYT24 due to ANO3 mutations
Authors: Stamelou Maria , Charlesworth Gavin , Cordivari Carla , Schneider Susanne A. , Kägi Georg , Sheerin Una‐Marie , Rubio‐Agusti Ignacio , Batla Amit , Houlden Henry , Wood Nicholas W. , Bhatia Kailash P. 
Published: January 2014

ABSTRACTGenes causing primary dystonia are rare. Recently, pathogenic mutations in the anoctamin 3 gene (ANO3) have been identified to cause autosomal dominant craniocervical dystonia and have been assigned to the dystonia locus dystonia‐24 (DYT24). Here, we expand on the phenotypic spectrum of DYT24 and provide demonstrative videos. Moreover, tremor recordings were performed, and back‐averaged electroencephalography, sensory evoked potentials, and C‐reflex studies were carried out in two individuals who carried two different mutations in ANO3. Ten patients from three families are described. The age at onset ranged from early childhood to the forties. Cervical dystonia was the most common site of onset followed by laryngeal dystonia. The characteristic feature in all affected individuals was the presence of tremor, which contrasts DYT24 from the typical DYT6 phenotype. Tremor was the sole initial manifestation in some individuals with ANO3 mutations, leading to misdiagnosis as essential tremor. Electrophysiology in two patients with two different mutations showed co‐contraction of antagonist muscles, confirming dystonia, and a 6‐Hz arm tremor at rest, which increased in amplitude during action. In one of the studied patients, clinically superimposed myoclonus was observed. The duration of the myoclonus was in the range of 250 msec at about 3 Hz, which is more consistent with subcortical myoclonus. In summary, ANO3 causes a varied phenotype of young‐onset or adult‐onset craniocervical dystonia with tremor and/or myoclonic jerks. Patients with familial cervical dystonia who also have myoclonus‐dystonia as well as patients with prominent tremor and mild dystonia should be tested for ANO3 mutations. © 2014 The Authors. Movement Disorders published by International Parkinson and Movement Disorder Society
Acceleration of high angular and spatial resolution diffusion imaging using compressed sensing with multichannel spiral data
Authors: Mani Merry , Jacob Mathews , Guidon Arnaud , Magnotta Vincent , Zhong Jianhui 
Published: January 2014

PurposeTo accelerate the acquisition of simultaneously high spatial and angular resolution diffusion imaging.MethodsAccelerated imaging is achieved by recovering the diffusion signal at all voxels simultaneously from under‐sampled k‐q space data using a compressed sensing algorithm. The diffusion signal at each voxel is modeled as a sparse complex Gaussian mixture model. The joint recovery scheme enables incoherent under‐sampling of the 5‐D k‐q space, obtained by randomly skipping interleaves of a multishot variable density spiral trajectory. This sampling and reconstruction strategy is observed to provide considerably improved reconstructions than classical k‐q under‐sampling and reconstruction schemes. The complex model enables to account for the noise statistics without compromising the computational efficiency and theoretical convergence guarantees. The reconstruction framework also incorporates compensation of motion induced phase errors that result from the multishot acquisition.ResultsReconstructions of the diffusion signal from under‐sampled data using the proposed method yields accurate results with errors less that 5% for different accelerations and b‐values. The proposed method is also shown to perform better than standard k‐q acceleration schemes.ConclusionsThe proposed scheme can significantly accelerate the acquisition of high spatial and angular resolution diffusion imaging by accurately reconstructing crossing fiber architectures from under‐sampled data. Magn Reson Med 73:126–138, 2015. © 2014 Wiley Periodicals, Inc.
The Equine PeptideAtlas: A resource for developing proteomics‐based veterinary research
Authors: Bundgaard Louise , Jacobsen Stine , Sørensen Mette A. , Sun Zhi , Deutsch Eric W. , Moritz Robert L. , Bendixen Emøke 
Published: January 2014

Progress in MS‐based methods for veterinary research and diagnostics is lagging behind compared to the human research, and proteome data of domestic animals is still not well represented in open source data repositories. This is particularly true for the equine species. Here we present a first Equine PeptideAtlas encompassing high‐resolution tandem MS analyses of 51 samples representing a selection of equine tissues and body fluids from healthy and diseased animals. The raw data were processed through the Trans‐Proteomic Pipeline to yield high quality identification of proteins and peptides. The current release comprises 24 131 distinct peptides representing 2636 canonical proteins observed at false discovery rates of 0.2% at the peptide level and 1.4% at the protein level. Data from the Equine PeptideAtlas are available for experimental planning, validation of new datasets, and as a proteomic data mining resource. The advantages of the Equine PeptideAtlas are demonstrated by examples of mining the contents for information on potential and well‐known equine acute phase proteins, which have extensive general interest in the veterinary clinic. The extracted information will support further analyses, and emphasizes the value of the Equine PeptideAtlas as a resource for the design of targeted quantitative proteomic studies.
Objective and subjective socioeconomic status and health symptoms in patients following hematopoietic stem cell transplantation
Authors: Brown‐Iannuzzi Jazmin L. , Payne B. Keith , Rini Christine , DuHamel Katherine N. , Redd William H. 
Published: January 2014

AbstractObjectiveRecent research indicates that subjective socioeconomic status (SES) – the perception of one's own SES compared with other people – is an important predictor of cancer‐related health outcomes. Subjective SES may function as a psychosocial mechanism by which objective SES affects health, well‐being, and, more broadly, quality of life among cancer survivors. This study tested whether the association between objective SES and indicators of quality of life was mediated by subjective SES in a sample of cancer survivors who had undergone hematopoietic stem cell transplantation.MethodsHematopoietic stem cell transplantation survivors (N = 268) completed measures of objective and subjective SES, along with four measures related to quality of life (depressive symptoms, health‐related quality of life, symptoms of generalized distress, and posttraumatic stress disorder symptoms).ResultsHigher objective SES was associated with greater quality of life across all four measures. Subjective SES mediated the relationship between objective SES and depressive symptoms (total indirect effect b = −0.09, 95% confidence interval [CI] [−0.15, −0.05]), generalized distress (total indirect effect b = −0.08, 95% CI [−0.13, −0.04]), health‐related quality of life (total indirect effect b = 0.10, 95% CI [0.06, 0.17]), and posttraumatic stress disorder (total indirect effect b = −0.08, 95% CI [−0.14, −0.04]).ConclusionsFindings extend work on subjective SES to cancer and suggest that SES gradients in patient outcomes after cancer may reflect not only material resources but also psychosocial factors related to rank within social hierarchies. Further research may provide insights useful for reducing disparities in this population. Copyright © 2014 John Wiley & Sons, Ltd.
Helicobacter pylori RNA polymerase α‐subunit C‐terminal domain shows features unique to ɛ‐proteobacteria and binds NikR/DNA complexes
Authors: Borin Brendan N. , Tang Wei , Krezel Andrzej M. 
Published: January 2014

AbstractBacterial RNA polymerase is a large, multi‐subunit enzyme responsible for transcription of genomic information. The C‐terminal domain of the α subunit of RNA polymerase (αCTD) functions as a DNA and protein recognition element localizing the polymerase on certain promoter sequences and is essential in all bacteria. Although αCTD is part of RNA polymerase, it is thought to have once been a separate transcription factor, and its primary role is the recruitment of RNA polymerase to various promoters. Despite the conservation of the subunits of RNA polymerase among bacteria, the mechanisms of regulation of transcription vary significantly. We have determined the tertiary structure of Helicobacter pylori αCTD. It is larger than other structurally determined αCTDs due to an extra, highly amphipathic helix near the C‐terminal end. Residues within this helix are highly conserved among ɛ‐proteobacteria. The surface of the domain that binds A/T rich DNA sequences is conserved and showed binding to DNA similar to αCTDs of other bacteria. Using several NikR dependent promoter sequences, we observed cooperative binding of H. pylori αCTD to NikR:DNA complexes. We also produced αCTD lacking the 19 C‐terminal residues, which showed greatly decreased stability, but maintained the core domain structure and binding affinity to NikR:DNA at low temperatures. The modeling of H. pylori αCTD into the context of transcriptional complexes suggests that the additional amphipathic helix mediates interactions with transcriptional regulators.
Sequence variation and structural conservation allows development of novel function and immune evasion in parasite surface protein families
Authors: Higgins Matthew K. , Carrington Mark 
Published: January 2014

AbstractTrypanosoma and Plasmodium species are unicellular, eukaryotic pathogens that have evolved the capacity to survive and proliferate within a human host, causing sleeping sickness and malaria, respectively. They have very different survival strategies. African trypanosomes divide in blood and extracellular spaces, whereas Plasmodium species invade and proliferate within host cells. Interaction with host macromolecules is central to establishment and maintenance of an infection by both parasites. Proteins that mediate these interactions are under selection pressure to bind host ligands without compromising immune avoidance strategies. In both parasites, the expansion of genes encoding a small number of protein folds has established large protein families. This has permitted both diversification to form novel ligand binding sites and variation in sequence that contributes to avoidance of immune recognition. In this review we consider two such parasite surface protein families, one from each species. In each case, known structures demonstrate how extensive sequence variation around a conserved molecular architecture provides an adaptable protein scaffold that the parasites can mobilise to mediate interactions with their hosts.
An adaptive solution for robust control based on integral high‐order sliding mode concept
Authors: Taleb M. , Plestan F. , Bououlid B. 
Published: January 2014

SummaryA new high‐order sliding mode controller is proposed. The main features are gain adaptivity and the use of integral sliding mode concept. The gain adaptation allows a reduction of the chattering and gives a solution to control uncertain nonlinear systems whose the uncertainties/perturbations have unknown bounds. The concept of real high‐order sliding mode detector is introduced given that it plays a key role in the adaptation law of the gain. This new control approach is applied by simulation to an academic example to evaluate its efficiency. Copyright © 2014 John Wiley & Sons, Ltd.
An integer linear programming formulation for power equalization placement to limit jamming attack propagation in transparent optical networks
Authors: Skorin‐Kapov Nina , Jirattigalachote Amornrat , Wosinska Lena 
Published: January 2014

ABSTRACTTransparent optical networks can be highly vulnerable to various physical‐layer attacks, such as high‐power jamming, which can cause widespread service disruption and even service denial. The propagation of such attacks can be thwarted with wavelength‐selective attenuators, referred to as power equalizers, installed at the network nodes. However, employing all nodes with power equalization functionality can lead to substantial costs. In previous work, we proposed a heuristic approach for sparse power equalization placement to limit jamming attack propagation cost‐effectively. The approach provides suboptimal solutions quickly; however, it does not guarantee optimality. Because placement of such power equalization is a long‐term planning problem affecting the capital expenditures of the network operator, solution quality is more critical than execution time. Thus, in this paper, we propose an integer linear programming formulation for the problem to guarantee optimality in terms of the number of power equalizers placed. Evaluation results show that our proposed integer linear programming formulation is able to solve moderately sized problems in reasonable time. These results also support the efficiency of our previously proposed heuristic by confirming its ability to find optimal solutions for the cases tested. Copyright © 2014 John Wiley & Sons, Ltd.
Instandsetzung von Bauten des Kraftwerks Meliti, Griechenland, nach einem Brand
Authors: Vayas Ioannis , Spiliopoulos Andreas , Dasiou Maria‐Eleni , Dougka Georgia , Dimakogianni Danai 
Published: January 2014

AbstractDas Kraftwerk Meliti in Nordgriechenland ist ein Braunkohlekraftwerk mit einer Bruttoleistung von 330 MW und wird von der Public Power Corporation (PCC) betrieben. Am 25. Juli 2014 brach ein Feuer an einer Förderbandanlage aus, die den Brennstoff zum Bunkerschwerbau fördert. Es entstanden Schäden in der 240 m langen stählernen Förderbandbrücke sowie in dem angrenzenden Betonbunkerschwerbau. Die Förderbandbrücke wurde durch Auswechselung der beschädigten Stäbe und Verbindungsmittel ohne Einsatz von Lehrgerüsten zur temporären Abstützung instandgesetzt. Am Bunkerschwerbau entstanden lokale Schäden, die mit Hilfe von CFK‐Lamellen und ‐Gewebe behoben werden konnten. Nach zwei Monaten Instandsetzung wurde die Kraftwerksanlage wieder in Betrieb genommen.Structural rehabilitation of the power plant Meliti, Greece after fire. The power plant Meliti in Northern Greece is a 300 MW power station that uses lignite as fuel. On the 25th of July 2014 a fire broke out in the conveyor belt that transports the coal to the bunker. The fire resulted in damages in the 240 m long steel conveyor bridge and the concrete bunker structure. The conveyor bridge was repaired without scaffolding temporary supports by exchanging the damaged parts and the connection media with new. The bunker was subjected to local damages that were rehabilitated by application of FRP sheets and FRP wraps. The plant was in operation in two months after it was fully rehabilitated.
Berichtigung: Frames with unique global and local imperfection in the shape of the elastic buckling mode. Part 1. Stahlbau 82 (2013), H. 8, S. 609–617, Part 2. Stahlbau 83 (2013), H. 9, S. 684–694.
Authors: Chladný E. , Štujberová M. 
Published: January 2014

AbstractComments
a) Comment to Table 1 in Stahlbau 2013/8
Formulae in Table 1 may be used for ­following purposes:
1. Determination of the position for the critical cross‐section m
2. Calculation of ηinit,m(x), ηinit,m,m and MIIhinit,m(x)
3. Elastic verification of the buckling ­resistance
4. Plastic verification of the buckling ­resistance
aa) For the elastic verification of the buckling resistance:
Use all the formulae with elastic cross‐section characteristics for purposes 1, 2, 3.
ab) For the plastic verification of the buckling resistance:
For purposes 1and 2 use all the ­formulae with elastic cross‐section characteristics
For purpose 4 use the formula (15) with MRd(x) = Mpl,Rd(x)
b) Comment to Table 4 and Table 6
Due to the error in 5.1.1.2 b) and c) shown in the table above the values of NIIhint and of MIIhint in the table 4 are 1,133‐times greater than the correct ones. But the difference in utilisation for frame columns shown in the column [5.2.2(3) b + 5.3.2(11)] in the table 6 is due to small contribution of imperfections effect negligible and conclusions in 6 are not INFluenced by this.
Wir bitten um Entschuldigung und ­Beachtung. Die Redaktion
Extraction and Identification of Phytosterols in Manketti (Schinziophyton rautanenii) Nut Oil
Authors: Gwatidzo Luke , Botha Ben M. , McCrindle Rob I. , Combrinck Sandra 
Published: January 2014

AbstractPhytosterols of manketti (Schinziophyton rautanenii) nut oil extracted by Soxhlet, mechanical shaking using hexane, screw press and supercritical carbon dioxide, were analyzed by gas chromatography with flame ionization detection and identified by gas chromatography–mass spectrometry. The presence of several phytosterols (campesterol, stigmasterol, β‐sitosterol, Δ5‐avenasterol, 22‐dihydrospinasterol and Δ7‐avenasterol) previously reported in manketti oil, was confirmed. In addition, another fourteen phytosterols (lanosterol, Δ5,23‐stigmastadienol, Δ7‐campesterol, clerosterol, obtusifoliol, Δ5,24(25)‐stigmastadienol, α‐amyrin, gramisterol, cycloeucalenol, cycloartenol, stigmasta‐8,24‐dienol‐3‐β‐ol, 28‐methylobtusifoliol, 24‐methylenecycloartenol and citrostadienol) were identified. The phytosterols, β‐sitosterol, Δ5‐avenasterol and campesterol, had the highest concentrations in oils extracted by all the methods, whereas stigmasterol and cycloartenol were abundant in oils extracted by mechanical shaking and supercritical carbon dioxide. Total phytosterols and the quantities of individual phytosterols differed significantly (p ≤ 0.05) in oils from the four extraction methods. Mechanical shaking extracted the highest levels of total sterols (22,100 mg/100 g oil), followed by supercritical fluid extraction (9,550 mg/100 g oil). Screw press and Soxhlet extracted oils contained the lowest levels of total sterols, 3,810 mg/100 g oil and 3,350 mg/100 g oil, respectively.
Clinicopathological analysis and survival outcome of duodenal adenocarcinoma
Authors: Kim Myung Jin , Choi Sae Byeol , Han Hyung Joon , Park Pyoung Jae , Kim Wan Bae , Song Tae Jin , Suh Sung Ock , Choi Sang Yong 
Published: January 2014

AbstractDuodenal adenocarcinoma is a rare cancer, contributing <10 % of periampullary carcinoma. This study reviews the single center experience of duodenal adenocarcinoma and analyzes the clinical and pathological factors to predict survival and recurrence. The records of 50 patients with duodenal adenocarcinoma who underwent surgical exploration or resection from 1995 to 2010 were reviewed retrospectively. Univariate and multivariate analyses were performed to identify the clinicopathological factors associated with survival and recurrence. There were 35 men and 15 women, with a mean age of 61 years. In multivariate analysis of 50 patients, R0 resection [p = 0.041, hazard ratio (HR) = 3.569, 95% confidence interval (CI) = 1.057–12.054] and symptom at initial admission (p = 0.025, HR = 11.210, 95% CI = 1.354–92.812) were independent prognostic factors for overall survival. Thirty‐six patients underwent curative resection (resectability 72%). The 5‐year survival rates for curative and noncurative resections were 46.4% and 0%, respectively. Univariate analysis of 36 patients who underwent R0 resection revealed that symptoms at initial admission (p = 0.023), presence of lymph node metastasis (p = 0.034), and perineural invasion (p = 0.025) were significant prognostic factors after curative resection. There was no significant factor for overall survival in the multivariate analysis. There was recurrence in 15 patients, mainly as liver metastasis. Multivariate analysis revealed that presence of symptom (p = 0.047, HR = 5.362, 95% CI = 1.021–28.149) and ulcerative tumor (p = 0.036, HR = 5.668, 95% CI = 1.123–28.619) were independent factors for disease free survival. An aggressive surgical approach to achieve R0 resection was important to enhance survival. Most of the recurrence occurred within 1 year after surgery. Close follow‐up is necessary after surgical resection.
Smithian shoreline migrations and depositional settings in Timpoweap Canyon (Early Triassic, Utah, USA)
Authors: OLIVIER NICOLAS , BRAYARD ARNAUD , FARA EMMANUEL , BYLUND KEVIN G. , JENKS JAMES F. , VENNIN EMMANUELLE , STEPHEN DANIEL A. , ESCARGUEL GILLES 
Published: January 2014

AbstractIn Timpoweap Canyon near Hurricane (Utah, USA), spectacular outcrop conditions of Early Triassic rocks document the geometric relationships between a massive Smithian fenestral-microbial unit and underlying, lateral and overlying sedimentary units. This allows us to reconstruct the evolution of depositional environments and high-frequency relative sea-level fluctuations in the studied area. Depositional environments evolved from a coastal plain with continental deposits to peritidal settings with fenestral-microbial limestones, which are overlain by intertidal to shallow subtidal marine bioclastic limestones. This transgressive trend of a large-scale depositional sequence marks a long-term sea-level rise that is identified worldwide after the Permian–Triassic boundary. The fenestral-microbial sediments were deposited at the transition between continental settings (with terrigenous deposits) and shallow subtidal marine environments (with bioturbated and bioclastic limestones). Such a lateral zonation questions the interpretation of microbial deposits as anachronistic and disaster facies in the western USA basin. The depositional setting may have triggered the distribution of microbial deposits and contemporaneous marine biota. The fenestral-microbial unit is truncated by an erosional surface reflecting a drop in relative sea level at the scale of a medium depositional sequence. The local inherited topography allowed the recording of small-scale sequences characterized by clinoforms and short-distance lateral facies changes. Stratal stacking pattern and surface geometries allow the reconstruction of relative sea-level fluctuations and tracking of shoreline migrations. The stacking pattern of these small-scale sequences and the amplitude of corresponding high-frequency sea-level fluctuations are consistent with climatic control. Large- and medium-scale sequences suggest a regional tectonic control.
Putting theory to the test: Examining family context, caregiver motivation, and conflict in the Family Check-Up model
Authors: Fosco Gregory M. , Van Ryzin Mark , Stormshak Elizabeth A. , Dishion Thomas J. 
Published: January 2014

AbstractThis study examined contextual factors (caregiver depression, family resources, ethnicity, and initial levels of youth problem behavior) related to the effectiveness of the Family Check-Up (FCU) and evaluated family processes as a mediator of FCU intervention response and adolescent antisocial behavior. We followed a sample of 180 ethnically diverse youths of families who engaged in the FCU intervention. Family data were collected as part of the FCU assessment, and youth data were collected over 4 years, from sixth through ninth grade. Findings indicated that caregiver depression and minority status predicted greater caregiver motivation to change. In turn, caregiver motivation was the only direct predictor of FCU intervention response during a 1-year period. Growth in family conflict from sixth through eighth grade mediated the link between FCU response and ninth-grade antisocial behavior. This study explicitly tested core aspects of the FCU intervention model and demonstrated that caregiver motivation is a central factor that underlies family response to the FCU. The study also provided support for continued examination of family process mechanisms that account for enduring effects of the FCU and other family-centered interventions.