gebhart/patrepeval huggingface dataset:
Bethlem myopathy: a slowly progressive congenital muscular dystrophy with contractures (1999)

Bethlem myopathy is an early-onset benign autosomal dominant myopathy with contractures caused by mutations in collagen type VI genes. It has been reported that onset occurs in early childhood. We investigated the natural course of Bethlem myopathy in five previously published kindreds and two novel pedigrees, with particular attention to the mode of onset in 23 children and the progression of weakness in 36 adult patients. Our analysis shows that nearly all children exhibit weakness or contractures during the first 2 years of life. Early features include diminished foetal movements, neonatal hypotonia and congenital contractures which are of a dynamic nature during childhood. The course of Bethlem myopathy in adult patients is less benign than previously thought. Due to slow but ongoing progression, more than two-thirds of patients over 50 years of age use a wheelchair.
Application of Fractal Dimension of Terrestrial Laser Point Cloud in Classification of Independent Trees (2021)

Tree precise classification and identification of forest species is a core issue of forestry resource monitoring and ecological effect assessment. In this paper, an independent tree species classification method based on fractal features of terrestrial laser point cloud is proposed. Firstly, the terrestrial laser point cloud data of an independent tree is preprocessed to obtain terrestrial point clouds of independent tree canopy. Secondly, the multi-scale box-counting dimension calculation algorithm of independent tree canopy dense terrestrial laser point cloud is proposed. Furthermore, a robust box-counting algorithm is proposed to improve the stability and accuracy of fractal dimension expression of independent tree point cloud, which implementing gross error elimination based on Random Sample Consensus. Finally, the fractal dimension of a dense terrestrial laser point cloud of independent trees is used to classify different types of independent tree species. Experiments on nine independent trees of three types show that the fractal dimension can be stabilized under large density variations, proving that the fractal features of terrestrial laser point cloud can stably express tree species characteristics, and can be used for accurate classification and recognition of forest species.
Multiple Transcripts Encode Full-Length Human Cytomegalovirus IE1 and IE2 Proteins during Lytic Infection (2016)

The HCMV IE1 and IE2 proteins are critical regulators of HCMV replication, both during primary infection and reactivation from viral latency. This study expands our understanding of the sequences controlling IE1 and IE2 expression by defining novel transcriptional units controlling the expression of full-length IE1 and IE2 proteins. Our results suggest that alternative promoters may allow for IE1 and IE2 expression when MIEP activity is limiting, as occurs in latently infected cells.
Multiple Morphologies of "Crew-Cut" Aggregates of Polystyrene- <i>b</i> -poly(acrylic acid) Block Copolymers (1995)

The observation by transmission electron microscopy of six different stable aggregate morphologies is reported for the same family of highly asymmetric polystyrene-poly-(acrylic acid) block copolymers prepared in a low molecular weight solvent system. Four of the morphologies consist of spheres, rods, lamellae, and vesicles in aqueous solution, whereas the fifth consists of simple reverse micelle-like aggregates. The sixth consists of up to micrometer-size spheres in aqueous solution that have hydrophilic surfaces and are filled with the reverse micelle-like aggregates. In addition, a needle-like solid, which is highly birefringent, is obtained on drying of aqueous solutions of the spherical micelles. This range of morphologies is believed to be unprecedented for a block copolymer system.
Magnetoresistive response of small permalloy features (1980)

We have systematically studied the character and variations of Barkhausen noise in a variety of micron size magnetoresistive sensors. From domain observations, we have revealed the basic micromagnetic process involved and demonstrated the central role played by Neel wall structures and their instabilities in Barkhausen noise. A small amount of easy axis field is effective in producing rotational response and hence quiet magnetoresistive outputs.
An optimization-based approach to dynamic data content selection in intelligent multimedia interfaces (2004)

We are building a multimedia conversation system to facilitate information seeking in large and complex data spaces. To provide tailored responses to diverse user queries introduced during a conversation, we automate the generation of a system response. Here we focus on the problem of determining the data content of a response. Specifically, we develop an optimization-based approach to content selection. Compared to existing rule-based or plan-based approaches, our work offers three unique contributions. First, our approach provides a general framework that effectively addresses content selection for various interaction situations by balancing a comprehensive set of constraints (e.g., content quality and quantity constraints). Second, our method is easily extensible, since it uses feature-based metrics to systematically model selection constraints. Third, our method improves selection results by incorporating content organization and media allocation effects, which otherwise are treated separately. Preliminary studies show that our method can handle most of the user situations identified in a Wizard-of-Oz study, and achieves results similar to those produced by human designers.
Rekindling network protocol innovation with user-level stacks (2014)

Recent studies show that more than 86% of Internet paths allow well-designed TCP extensions, meaning that it is still possible to deploy transport layer improvements despite the existence of middleboxes in the network. Hence, the blame for the slow evolution of protocols (with extensions taking many years to nbecome widely used) should be placed on end systems. In this paper, we revisit the case for moving protocols stacks up into user space in order to ease the deployment of new protocols, extensions, or performance optimizations. We present MultiStack, operating system support for user-level protocol stacks. MultiStack runs within commodity operating systems, can concurrently host a large number of isolated stacks, has a fall-back path to the legacy host stack, and is able to process packets at rates of 10Gb/s. We validate our design by showing that our mux/demux layer can validate and switch packets at line rate (up to 14.88 Mpps) on a 10 Gbit port using 1-2 cores, and that a proof-of-concept HTTP server running over a basic userspace TCP outperforms by 18-90% both the same server and nginx running over the kernel's stack.
A murine model of hereditary hemorrhagic telangiectasia (1999)

Endoglin (CD105), an accessory protein of the TGF-beta receptor superfamily, is highly expressed on endothelial cells. Hereditary hemorrhagic telangiectasia type 1 (HHT1) is associated with mutations in the Endoglin gene, leading to haploinsufficiency. To generate a disease model and ascertain the role of endoglin in development, we generated mice lacking 1 or both copies of the gene. Endoglin null embryos die at gestational day 10.0-10.5 due to defects in vessel and heart development. Vessel formation appears normal until hemorrhage occurs in yolk sacs and embryos. The primitive vascular plexus of the yolk sac fails to mature into defined vessels, and vascular channels dilate and rupture. Internal bleeding is seen in the peritoneal cavity, implying fragile vessels. Heart development is arrested at day 9.0, and the atrioventricular canal endocardium fails to undergo mesenchymal transformation and cushion-tissue formation. These data suggest that endoglin is critical for both angiogenesis and heart valve formation. Some heterozygotes, either with an inbred 129/Ola or mixed C57BL/6-129/Ola background, show signs of HHT, such as telangiectases or recurrent nosebleeds. In this murine model of HHT, it appears that epigenetic factors and modifier genes, some of which are present in 129/Ola, contribute to disease heterogeneity.
2-Pyrones. XIX. 3-Hydroxy-2-pyrone<sup>1</sup> and 4-Arylhydrazono-2,3-pyranones (1956)

ADVERTISEMENT RETURN TO ISSUEPREVArticleNEXT2-Pyrones. XIX. 3-Hydroxy-2-pyrone1 and 4-Arylhydrazono-2,3-pyranonesRichard H. Wiley and C. H. JarboeCite this: J. Am. Chem. Soc. 1956, 78, 11, 2398–2401Publication Date (Print):June 1, 1956Publication History Published online1 May 2002Published inissue 1 June 1956https://pubs.acs.org/doi/10.1021/ja01592a015https://doi.org/10.1021/ja01592a015research-articleACS PublicationsRequest reuse permissionsArticle Views446Altmetric-Citations20LEARN ABOUT THESE METRICSArticle Views are the COUNTER-compliant sum of full text article downloads since November 2008 (both PDF and HTML) across all institutions and individuals. These metrics are regularly updated to reflect usage leading up to the last few days.Citations are the number of other articles citing this article, calculated by Crossref and updated daily. Find more information about Crossref citation counts.The Altmetric Attention Score is a quantitative measure of the attention that a research article has received online. Clicking on the donut icon will load a page at altmetric.com with additional details about the score and the social media presence for the given article. Find more information on the Altmetric Attention Score and how the score is calculated. Share Add toView InAdd Full Text with ReferenceAdd Description ExportRISCitationCitation and abstractCitation and referencesMore Options Share onFacebookTwitterWechatLinked InRedditEmail Other access optionsGet e-Alertsclose Get e-Alerts
A Systematic Approach to Flexible Specifications, Composition, and Restructuring of Workflow Activities (2004)

We introduce the ActivityFlow specification language for flexible specification, composition, and coordination of workflow activities. The most interesting features of the ActivityFlow specification language include: (1) a collection of specification mechanisms, allowing workflow designers to use a uniform workflow specification interface to describe different types (i.e., ad-hoc, administrative, or production) of workflows involved in their organizational processes– this feature helps to increase the flexibility of workflow processes in accommodating various types of changes; (2) a set of activity modeling facilities, enabling workflow designers to describe the flow of work declaratively and incrementally, allowing to reason about correctness and security of complex workflow activities independently from their underlying implementation mechanisms; (3) an open architecture that supports user interaction as well as collaboration of workflow systems of different organizations, and a set of workflow activity restructuring operators to respond to dynamic changes of workflow activities. We end the paper with a series of simulation-based experiments that demonstrate the effectiveness of these restructuring operators and the implementation architecture of the ActivityFlow system.
Wilms’ Tumor 1 Gene Mutations Independently Predict Poor Outcome in Adults With Cytogenetically Normal Acute Myeloid Leukemia: A Cancer and Leukemia Group B Study (2008)

We report the first evidence that WT1 mutations independently predict extremely poor outcome in intensively treated, younger patients with CN-AML. Future trials should include testing for WT1 mutations as part of molecularly based risk assessment and risk-adapted treatment stratification of patients with CN-AML.
Key issues when using tax data for concentration analysis: an application to the Spanish wealth tax (2009)

Tax data offers the opportunity to estimate income or wealth shares, as recent studies on 
different countries show for the upper groups. In this study we focus on wealth concentration in 
Spain by using Wealth Tax data, in the line carried out by Alvaredo and Saez (2008). However, 
the particular aim of our work is to identify the main critical points that must be considered 
when using data from the Spanish WT to avoid misleading conclusions: interpolation, 
underassessment of real estates, owner-occupied housing exemption, tax unit and tax fraud. 
Taken these key issues into account we obtain that our results offer a higher concentration than 
Alvaredo and Saez's one (2008) (on average 19.11% vs. 17.31% for the period 1988-2003). In 
addition, we observe that the boom of the housing market has been regressive and, moreover, 
has not been translated into a higher level of housing reported wealth in the WT due to tax 
fraud.
Potassium−Lead-Switched G-Quadruplexes: A New Class of DNA Logic Gates (2009)

A cation-driven allosteric G-quadruplex DNAzyme (PW17) was utilized to devise a conceptually new class of DNA logic gate based on cation-tuned ligand binding and release. K(+) favors the binding of hemin to parallel-stranded PW17, thereby promoting the DNAzyme activity, whereas Pb(2+) induces PW17 to undergo a parallel-to-antiparallel conformation transition and thus drives hemin to release from the G-quadruplex, deactivating the DNAzyme. Such a K(+)-Pb(2+) switched G-quadruplex, in fact, functions as a two-input INHIBIT logic gate. With the introduction of another input EDTA, this G-quadruplex can be further utilized to construct a reversibly operated IMPLICATION gate.
천식 마우스모델에서 Human Cytotoxic T Lymphocyte-associated Antigen 4-immunoglobulin (Hu.CTLA4-Ig) 융합단백의 기도 염증과 기도과민성 억제 (2008)

Background: CD4+ T helper cells which differentiate into the Th2 cells are crucial for the initiation and progression of allergic asthma. Human Cytotoxic T Lymphocyte-associated Antigen 4-immunoglobulin (CTLA4-Ig) has been widely used in order to block T-cell costimulation in an array of experimental animal models of T-cell-mediated diseases. Objective: To evaluate whether human CTLA4-Ig fusion protein inhibits airway inflammation in a murine model of OVA-induced asthma. Method: Six-weeks-old BALB/c mice were used and airway inflammation was induced by OVA. Human CTLA4-Ig fusion protein was administered intravenously before and after sensitization with OVA. Airway responsiveness to methacholine was measured after antigen challenge. Total IgE, OVA-specific IgE, IgG1 and IgG2a in serum samples were assessed by ELISA. Bronchoalveolar lavage (BAL) and lung section were also performed. Result: In mice which were treated with the CTLA4-Ig fusion protein before and after sensitization OVA, airway hyperresponsiveness and eosinophil BAL were markedly decreased. In mice which were with human CTLA4-Ig before sensitization with serum total IgE level were more significantly reduced that of positive control mice. Peribronchial inflammatory infiltration was desreased by the treatment of CTLA4-Ig before and after sensitization with OVA. Conclusion: These results suggest that human CTLA4-Ig fusion protein may inhibit eosinophilic airway inflammation and airway hyperresponsivness in OVA-sensitized airway challenged mice. (Korean J Asthma Allergy Immunol 2008;28:205-213)
The Role of Global and Local Landmarks in Virtual Environment Navigation (2000)

In visual navigation, landmarks can be used in a number of different ways. In this paper, we investigate the role of global and local landmarks in virtual environment navigation. We performed an experiment in a virtual environment called “Hexatown”, consisting of a regular hexagonal grid of streets and junctions. Each junction was identified by the presence of distinct local landmarks (buildings, phone box, and so on). Additionally, compass information or a global frame of reference was provided by global landmarks (hilltop, television tower, and city skyline). According to participants' movement decisions, egomotion was simulated, and displayed on a 180 deg. projection screen. Participants learned the route back and forth between two local landmarks. In the test phase, individual junctions were approached and the participant's movement decision was recorded. We performed two experiments involving landmark changes after learning. In the first, we used conflicting cues by transposing landmarks. In the second experiment, we reduced either local or global landmark information. Results show that both local and global landmarks are used in wayfinding decisions. However, different participants rely on different strategies. In the first experiment (cue conflict) for example, some of the participants used only local landmarks while others relied exclusively on global landmarks. Other participants used local landmarks at one location and global landmarks at the other. When removing one landmark type in the second experiment, the other type could be used by almost all participants, indicating that information about the neglected landmark type was present in memory.
Microstructure of Spray-Dried and Freeze-Dried Microalgal Powders (1985)

Chiarella and Spirulina algae were spray-dried and freezedried under several different conditions, and the morphological changes induced were studied by scanning electron microscopy. The internal structure of the particles (granules) was revealed by cryofracturing. The cellular composition of the two algae, method of drying, cell concentration, feed conditions, temperature, and drying times all affected the external morphology of the resulting powders. In the case of spray-drying, at a temperature of 160 °C for 6 sec, the particles shrunk or collapsed; if the drying time was over 10 sec at 160 °C, similar results were observed. The cell concentration affected the thickness of the dried particle wall ; a high concentration produced thicker and heavier particles, lower concentrations produced thinner and lighter particles. The rate and pressure at which the liquid was fed into the drying chamber affected the proper atomization of the liquid . When freeze-dried , the algal powder structure was different from particles formed by spray-drying. Spray-dried particles were individual spheres with a void space in the center. Each particle was composed of a few thousand cells in the case of Chiarella and several trichomes in the case of Spirulina. In contrast freeze-dried algae formed sheets of cells that were no longer spherical and which adhered together in a linear fashion. The cryogenic process used in this study to fracture and study the structure of spray-dried microalgal powders should prove to be very helpful in selecting optimal conditions for the production of high quality health food products.
Small-Scale Characterization of Additively Manufactured Aluminum Alloys Through Depth-Sensing Identification (2018)

Selective laser melting (SLM) can be considered as a suitable additive manufacturing method for printing complex-in-shape aluminum components. However, to adopt the SLM for mass production of aluminum components for automotive and aerospace applications, one needs to fully understand the correlations between the SLM parameters, produced microstructure, and local mechanical properties of the printed parts. In this paper, along with microstructural assessments (optical and scanning electron microscopy), small-scale properties of two additively manufactured aluminum alloys, AlSi10Mg and A205.0, were examined using an instrumented (depth-sensing) indentation testing technique. An instrumented indentation testing approach is a semi-destructive, reliable and convenient method which enables us to study the variations and distributions of the mechanical properties (i.e. hardness) as a function of distance from the build plate. The variations in properties are then compared in AlSi10Mg and A205.0 alloys and are correlated to the generated microstructures in the printed alloys.
Sample rate conversion in software configurable radios (2002)

SRC in Software Radio. Basic Principles. A Concept for SRC. Systems for Integer-Factor SRC. Comb-Filters. Systems for Fractional SRC. Applications of SRC. Conclusions. Appendixes. Symbols and Abbreviations. Biography.
A Phase 2, Randomized, Dose-Finding Study of the Novel Once-Weekly Human GLP-1 Analog, Semaglutide, Compared With Placebo and Open-Label Liraglutide in Patients With Type 2 Diabetes (2015)

After 12 weeks, semaglutide dose-dependently reduced HbA1c level and weight in patients with type 2 diabetes. No unexpected safety or tolerability concerns were identified; gastrointestinal AEs typical of glucagon-like peptide 1 receptor agonists were mitigated by dose escalation. On this basis, weekly semaglutide doses of 0.5 and 1.0 mg with a 4-week dose escalation were selected for phase 3.
Marmaris Orman İşletme Müdürlüğü’nde yangın tehlike oranları (2000)

bal Marmaris Orman letm esi'nde kabilecek bir yangn ihtimalinin saptanmas (Yangn Tehlike Oran) am acyla yaplm tn Sz konusu iletme snrlar dahilinde 1987 -1996 yllar arasnda kan her bir yangnn kt gn ve saatlere ait mete orolojik veriler en yakn meteoroloji istasyonlarndan alnm daha sonra m a tematiksel yntem ler ve bilgi ilem yardm yla yangn
Leukocyte Recruitment in the Cerebrospinal Fluid of Mice with Experimental Meningitis Is Inhibited by an Antibody to Junctional Adhesion Molecule (Jam) (1999)

The mechanisms that govern leukocyte transmigration through the endothelium are not yet fully defined. Junctional adhesion molecule (JAM) is a newly cloned member of the immunoglobulin superfamily which is selectively concentrated at tight junctions of endothelial and epithelial cells. A blocking monoclonal antibody (BV11 mAb) directed to JAM was able to inhibit monocyte transmigration through endothelial cells in in vitro and in vivo chemotaxis assays. In this study, we report that BV11 administration was able to attenuate cytokine-induced meningitis in mice. The intravenous injection of BV11 mAb significantly inhibited leukocyte accumulation in the cerebrospinal fluid and infiltration in the brain parenchyma. Blood–brain barrier permeability was also reduced by the mAb. We conclude that JAM may be a new target in limiting the inflammatory response that accompanies meningitis.
Heck Reaction <i>versus</i> Free Radical Reaction for the Synthesis of Spiro[indoline-2,4′-piperidines] (1998)

Spiro[indoline-2,4′-piperidines] can be prepared from 2-bromoaniline by using a Heck reaction or a free radical reaction as the key step. The radical process produces better yields of spiro[indoline-2,4′-piperidines] than the Heck process.
Standardization of apolipoprotein B and A-I measurements. (1989)

Apolipoprotein B, the major protein of low-density lipoprotein, and apolipoprotein A-I, the major protein of high-density lipoprotein, can serve as important predictors of atherosclerotic cardiovascular disease. However, these apolipoprotein measurements have not realized their full potential because of inadequate standardization. Purified apolipoprotein A-I of known absolute mass, in lyophilized form, can serve as primary standard for apolipoprotein A-I, and freshly isolated low-density lipoprotein of narrow density range can serve as primary standard for apolipoprotein B. These primary standards can be used to assign target values to secondary reference material and calibrators by reference laboratories using standardized, validated immunoassay procedures. Lyophilized serum can serve as secondary reference material for apolipoprotein A-I. Freshly frozen serum pools should be used as reference material for apolipoprotein B until more practicable materials that do not exhibit matrix interactions are developed. Implementation of proper standardization procedures can lead to significant improvements in apolipoprotein measurements.