gebhart/patrepeval huggingface dataset:
A clinical and molecular characterisation of CRB1-associated maculopathy (2018)

To date, over 150 disease-associated variants in CRB1 have been described, resulting in a range of retinal disease phenotypes including Leber congenital amaurosis and retinitis pigmentosa. Despite this, no genotype-phenotype correlations are currently recognised. We performed a retrospective review of electronic patient records to identify patients with macular dystrophy due to bi-allelic variants in CRB1. In total, seven unrelated individuals were identified. The median age at presentation was 21 years, with a median acuity of 0.55 decimalised Snellen units (IQR = 0.43). The follow-up period ranged from 0 to 19 years (median = 2.0 years), with a median final decimalised Snellen acuity of 0.65 (IQR = 0.70). Fundoscopy revealed only a subtly altered foveal reflex, which evolved into a bull's-eye pattern of outer retinal atrophy. Optical coherence tomography identified structural changes-intraretinal cysts in the early stages of disease, and later outer retinal atrophy. Genetic testing revealed that one rare allele (c.498_506del, p.(Ile167_Gly169del)) was present in all patients, with one patient being homozygous for the variant and six being heterozygous. In trans with this, one variant recurred twice (p.(Cys896Ter)), while the four remaining alleles were each observed once (p.(Pro1381Thr), p.(Ser478ProfsTer24), p.(Cys195Phe) and p.(Arg764Cys)). These findings show that the rare CRB1 variant, c.498_506del, is strongly associated with localised retinal dysfunction. The clinical findings are much milder than those observed with bi-allelic, loss-of-function variants in CRB1, suggesting this in-frame deletion acts as a hypomorphic allele. This is the most prevalent disease-causing CRB1 variant identified in the non-Asian population to date.
A quad low voltage rail-to-rail operational amplifier (2003)

A low-voltage rail-to-rail amplifier that has a NPN PNP switchable differential input stage allowing rail-to-rail input voltage swings is presented. The inputs can be overdriven without causing phase reversal in the output signal. The amplifier provides rail-to-rail output voltage swings which extend to within 50 mV of either rail. The output stage is current boosted to provide up to 50 mA of drive current.< <ETX xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink">></ETX>
CCXXII.—The alkaline condensations of nitrohydrazo–compounds. Part I (1911)

A. G. Green and E. A. Bearder, J. Chem. Soc., Trans., 1911, 99, 1960 DOI: 10.1039/CT9119901960
Image management in a virtualized data center (2008)

Industrial research firms such as Gartner and IDC are predicting an explosion in the number of online services in the coming years. Virtualization technologies could play an important role in such a world, as they create an opportunity to provide services in a cost-effective manner. However, to achieve ideal savings, more dynamic environments must be created, with Virtual Machines (VMs) being provisioned and altered on-the-fly. Management issues arise when using these elastic resources at scale. In this study, we provide an initial investigation of performance and scalability issues for image management in a virtualized data center. Results provided show that the choice of storage solution and access protocol matters. For example, our tests show the time to start a VM from a local hard drive under I/O intensive workload increases by a factor of 15 and for certain shared storage options, this factor increases to 30 times.
Astronomical image coding using an edge and texture model (2002)

Presents an image coding model that separates edge information from texture information. Edges are detected by means of a wavelet transform modulus. An image approximation is reconstructed from edges selected by a threshold operation. A texture image is obtained by subtracting the reconstructed image from the original one. The texture information is decomposed using a scheme based on an orthonormal-basis wavelet decomposition followed by a threshold operation. The authors reconstruct the original image by obtaining edge image approximation and texture image approximation from an inverse process. They show the results of applying this method to astronomical images.< <ETX xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink">></ETX>
Phytophthora infestans Cholinephosphotransferase with Substrate Specificity for Very-Long-Chain Polyunsaturated Fatty Acids (2012)

The effective flux between phospholipids and neutral lipids is critical for a high level of biosynthesis and accumulation of very-long-chain polyunsaturated fatty acids (VLCPUFAs), such as arachidonic acid (ARA; 20:4n-6), eicosapentaenoic acid (EPA; 20:5n-3), and docosahexaenoic acid (DHA; 22:6n-3). Here we describe a cDNA (PiCPT1) from Phytophthora infestans, a VLCPUFA-producing oomycete, that may have a role in acyl trafficking between diacylglycerol (DAG) and phosphatidylcholine (PC) during the biosynthesis of VLCPUFAs. The cDNA encodes a polypeptide of 393 amino acids with a conserved CDP-alcohol phosphotransferase motif and approximately 27% amino acid identity to the Saccharomyces cerevisiae cholinephosphotransferase (ScCPT1). In vitro assays indicate that PiCPT1 has high cholinephosphotransferase (CPT) activity but no ethanolaminephosphotransferase (EPT) activity. Substrate specificity assays show that it prefers VLCPUFA-containing DAGs, such as ARA DAG and DHA DAG, as substrates. Real-time PCR analysis reveals that expression of PiCPT1 was upregulated in P. infestans organisms fed with exogenous VLCPUFAs. These results lead us to conclude that PiCPT1 is a VLCPUFA-specific CPT which may play an important role in shuffling VLCPUFAs from DAG to PC in the biosynthesis of VLCPUFAs in P. infestans.
Bone morphogenetic protein‐7 reduces the severity of colon tissue damage and accelerates the healing of inflammatory bowel disease in rats (2003)

Bone morphogenetic protein-7 (BMP-7) is a growth and differentiation factor and belongs to the TGF-beta superfamily of proteins. Previous studies have shown an abundant expression of BMP-7 in the developing intestine and an association with a perturbed BMP/SMAD downstream signaling leading to a malignant phenotype and inflammation in the gut. In the present study, we have evaluated the effect of systemically administered recombinant human BMP-7 against trinitrobenzenesulfonic (TNBS) acid induced inflammatory bowel disease (IBD) in rats. The TNBS administered rats treated with BMP-7 have developed much less severe form of colitis based on macroscopic and histological scoring when administered 1.5 h before or 24 h after colitis induction. Bioavailability studies in healthy rats have revealed that significant portion (3.6%) of i.v. administered BMP-7 is targeted for BMP-7 receptors in the stomach and ileum, respectively, suggesting its availability to target tissue upon administration. Immunohistochemical and RT-PCR analyses have shown elevated expression of pro-inflammatory (IL-6, TNF-beta, ICAM-1) and pro-fibrogenic (TGF-beta) cytokines, and BMP-7 treatment significantly reduced their expression in the intestine; among which the suppression of IL-6 appeared to be the most important. Taken together, the results of this study suggest that BMP-7 plays an important role in the regulation of anti-inflammatory response in the adult gut tissue.
Monitoring and Control of Genotoxic Impurity Acetamide in the Synthesis of Zaurategrast Sulfate (2010)

In this article we describe the strategy adopted to minimize the risk of acetamide presence in zaurategrast sulfate drug candidate. A risk of acetamide formation (a potential genotoxic impurity) was identified in the API formation step of the process during the early development phase. In order to keep the project development timelines unchanged and without having the appropriate analytical method ready developed, we chose to minimize the risk of acetamide impurity presence by applying an adequate chemical process design. The implementation of a workup sequence involving initially three aqueous washes was later proven to be successful when an appropriate analytical method to detect acetamide below ppm levels was available. Additionally the analytical tool gave us the opportunity to assess and fine-tune the designed process for acetamide elimination by spiking experiments. Data acquired during this evaluation showed that a single aqueous wash associated with two efficient crystallization steps were finally enough to deliver API with a content of acetamide below the level defined as the acceptance criterion.
Molecular Pathways Regulating Macrophage Polarization: Implications for Atherosclerosis (2012)

Recent years have seen a tremendous development of our insight into the biology of atherosclerosis and its acute thrombotic manifestations. Inflammation now takes center stage among traditional risk factors as a decisive factor in cardiovascular risk. Consequently, its assessment and modulation have become key to clinical care and fundamental research alike. Plaque macrophages orchestrate many of the inflammatory processes that occur throughout atherogenesis. These cells are characteristically heterogeneous and adopt diverse activation states in response to micro-environmental triggers. In this review, macrophage-mediated inflammation in atherosclerosis sets the scene for a discussion of the gene regulatory mechanisms that facilitate and shape polarized macrophage phenotypes. When applicable, we consider these factors within the context of atherosclerosis and reflect on opportunities for future application.
Systematic Interaction Management in a Workflow View Based Business-to-Business Process Engine (2005)

Workflow technology has recently been employed as a framework for implementing business-to-business (B2B) process enactment over the Internet. This typically requires collaborative enactment of workflows across multiple organizations. In this paper, we propose the use of workflow views as a fundamental support mechanism for B2B interactions. A workflow view is an external accessible subset of a private workflow. We present a methodology to show how B2B interactions can be systematically managed with the workflow view mechanism, by customizable orchestration of Web services mapping into internal existing workflows. We further present a deployment architecture for a B2B process engine and detail our implementation with contemporary Web services technologies. In addition, asynchronous events and exceptions as well as interaction monitoring can be facilitated in a unified framework.
Better than Burning It (2014)

Only one photo is necessary to conclude this section with Claire Zheng’s graduation dress, in which she finds a happy medium between architecture school and her interest in fashion by wearing her final project on her final day in the College of Design.
Synthesis of Large-Area Highly Crystalline Monolayer Molybdenum Disulfide with Tunable Grain Size in a H<sub>2</sub> Atmosphere (2015)

Large-area and highly crystalline monolayer molybdenum disulfide (MoS2) with a tunable grain size was synthesized in a H2 atmosphere. The influence of introduced H2 on MoS2 growth and grain size, as well as the corresponding mechanism, was tentatively explored by controlling the H2 flow rate. The as-grown monolayer MoS2 displays excellent uniformity and high crystallinity evidenced by Raman and high-resolution transmission electron microscopy. The Raman results also give an indication that the quality of the monolayer MoS2 synthesized in a H2 atmosphere is comparable to that synthesized by using seed or mechanical exfoliation. In addition, the electronic properties and dielectric inhomogeneity of MoS2 monolayers were also detected in situ via scanning microwave microscopy, with measurements on impedance and differential capacitance (dC/dV). Back-gated field-effect transistors based on highly crystalline monolayer MoS2 shows a field-effect mobility of ∼13.07 cm2 V(-1) s(-1) and an Ion/Ioff ratio of ∼1.1×10(7), indicating that the synthesis of large-area and high-quality monolayer MoS2 with H2 is a viable method for electronic and optoelectronic applications.
Text Augmentation: Inserting markup into natural language text with PPM Models (2006)

This thesis describes a new optimisation and new heuristics for automatically marking up XML documents. These are implemented in CEM, using PPMmodels. CEM is significantly more general than previous systems, marking up large numbers of hierarchical tags, using n-gram models for large n and a variety of escape methods.\n\nFour corpora are discussed, including the bibliography corpus of 14682 bibliographies laid out in seven standard styles using the BIBTEX system and markedup in XML with every field from the original BIBTEX. Other corpora include the ROCLING Chinese text segmentation corpus, the Computists’ Communique corpus and the Reuters’ corpus. A detailed examination is presented of the methods of evaluating mark up algorithms, including computation complexity measures and correctness measures from the fields of information retrieval, string processing, machine learning and information theory.\n\nA new taxonomy of markup complexities is established and the properties of each taxon are examined in relation to the complexity of marked-up documents. The performance of the new heuristics and optimisation is examined using the four corpora.
Basis pursuit (1994)

The time-frequency and time-scale communities have recently developed an enormous number of over-complete signal dictionaries, wavelets, wavelet packets, cosine packets, Wilson bases, chirplets, warped bases, and hyperbolic cross bases being a few examples. Basis pursuit is a technique for decomposing a signal into an optimal superposition of dictionary elements. The optimization criterion is the l/sup 1/ norm of coefficients. The method has several advantages over matching pursuit and best ortho basis, including super-resolution and stability. >
Embryonic and Induced Pluripotent Stem Cells as a Model for Liver Disease (2009)

Induced pluripotent stem (iPS) cells are human somatic cells that have been reprogrammed to a pluripotent state. Through several elegant technologies, we are now able to generate human iPS cells with disease genotypes that could serve as invaluable tools for human disease modeling. This could lead to an understanding of the root causes of a disease and to the development of effective prophylactic and therapeutic strategies for it. However, we are still far from generating fully functional liver cells from stem cells, including iPS cells, on in vitro culture systems. Tissue-engineering techniques have opened the window to inducing a functional fate for differentiated cells by providing a microenvironment that allows the maintenance of signals similar to those found in the natural microenvironment. Here we review the current technology to establish iPS cells and discuss strategies to generate human liver disease modeling using iPS cell technology in concert with bioengineering approaches.
Identification of Glu340 as the active-site nucleophile in human glucocerebrosidase by use of electrospray tandem mass spectrometry (1994)

Gaucher disease is an inherited lysosomal storage disorder caused by a deficiency of human acid beta-glucosidase (glucocerebrosidase). This enzyme is inactivated by the specific, mechanism-based enzyme inactivator 2-deoxy-2-fluoro-beta-D-glucopyranosyl fluoride, which functions by forming a stable 2-deoxy-2-fluoro-alpha-D-gluco-pyranosyl-enzyme intermediate. The key nucleophilic amino acid residue involved in formation of this intermediate was conclusively identified by tandem mass spectrometry as Glu340, and not Asp443 as thought previously. This was confirmed by site-directed mutagenesis. Identification, and mass determination, of the labeled peptide in a proteolytic hydrolysate involved detection of a collision-induced fragmentation reaction specific to the sugar-peptide linkage. Confirmation of the identity of the labeled peptide was obtained both by tandem mass spectrometric sequencing and by chemical degradation of the purified peptide. This method allowed the rapid, sensitive, and non-isotopic determination of an essential amino acid residue in a clinically important enzyme.
Flecainide–metoprolol combination reduces atrial fibrillation clinical recurrences and improves tolerability at 1-year follow-up in persistent symptomatic atrial fibrillation (2016)

Flecainide-metoprolol combination therapy improves effectiveness of rhythm control in persistent symptomatic AF and increases tolerability, with a concomitant reduction of side effects and a better compliance.
Distance and orientation dependence of electron transfer and exciplex formation of naphthyl and p-dimethylanilino groups fixed on a helical polypeptide chain (1990)

ADVERTISEMENT RETURN TO ISSUEPREVArticleNEXTDistance and orientation dependence of electron transfer and exciplex formation of naphthyl and p-dimethylanilino groups fixed on a helical polypeptide chainYoshihito. Inai, Masahiko. Sisido, and Yukio. ImanishiCite this: J. Phys. Chem. 1990, 94, 16, 6237–6243Publication Date (Print):August 1, 1990Publication History Published online1 May 2002Published inissue 1 August 1990https://pubs.acs.org/doi/10.1021/j100379a017https://doi.org/10.1021/j100379a017research-articleACS PublicationsRequest reuse permissionsArticle Views96Altmetric-Citations24LEARN 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 options Get e-Alerts
Waking effectiveness of alarm (auditory, visual and tactile) for adults who are hard of hearing (2007)

The study's aim is to optimize performance requirements for alarm and signaling systems to meet the needs of high risk groups. Elements of the study included: a risk assessment to estimate the potential impact in lives saved of changes in notification effectiveness of smoke alarms for these groups.
Advanced supersonic technology propulsion system study (1974)

This study had the objectives of determining the most promising conventional and variable cycle engine types; the effect of design cruise Mach number (2.2, 2.7 and 3.2) on a commercial supersonic transport; effect of advanced engine technology on the choice of engine cycle; and effect of utilizing hydrogen as the engine fuel. The technology required for the engines was defined, and the levels of development to ensure availability of this technology in advanced aircraft propulsion systems were assessed. No clearcut best conventional or variable cycle engine was identified. The dry bypass turbojet and the duct burning turbofans were initially selected as the best conventional engines, but later results, utilizing augmentation at takeoff, added the mixed-flow augmented turbofan as a promising contender. The modulating air flow, three-rotor variable cycle engine identified the performance features desired from VCE concepts (elimination of inlet drag and reduction in afterbody drag), but was a very heavy and complex engine.
Plasma mechanism of terahertz photomixing in high-electron mobility transistor under interband photoexcitation (2002)

We show that modulated near-infrared radiation can generate terahertz plasma oscillations in the channel of a high-electron mobility transistor. This effect is associated with a temporarily periodic injection of the electrons photoexcited by modulated near-infrared radiation into the transistor channel. The excitation of the plasma oscillations has the resonant character. It results in the pertinent excitation of the electric current in the external circuit that can be used for generation of terahertz electromagnetic radiation.
Organic processes initiated by non‐classical energy sources (2000)

Non-classical energy sources such as microwave energy, ultrasound and mechanoenergy and their combination with UV–VIS radiation are new tools in synthetic chemistry and chemical processing. Here we describe the application of microwave treatment for selected organic reactions such as (i) enzymatic transesterification of optically active alcohols, (ii) mercury-sensitized gas-phase photolysis of hydrocarbons in the microwave field, (iii) environmentally benign oxidations of olefins and (iv) the application of mechanoenergy separately and in combination with microwave irradiation for special oxidation reactions. Copyright © 2000 John Wiley & Sons, Ltd.
Crowdsourced Databases: Query Processing with People (2011)

Amazon’s Mechanical Turk (“MTurk”) service allows users to post short tasks (“HITs”) that other users can receive a small amount of money for completing. Common tasks on the system include labelling a collection of images, combining two sets of images to identify people which appear in both, or extracting sentiment from a corpus of text snippets. Designing a workflow of various kinds of HITs for filtering, aggregating, sorting, and joining data sources together is common, and comes with a set of challenges in optimizing the cost per HIT, the overall time to task completion, and the accuracy of MTurk results. We propose Qurk, a novel query system for managing these workflows, allowing MTurk-style processing of relational databases. We describe a number of query execution and optimization challenges, and discuss some potential solutions.
Rip Currents (2011)

Rip Currents: Beach Safety, Physical Oceanography, and Wave Modeling is the culmination of research from over 100 coastal scientists, engineers, forecast meteorologists, lifeguard chiefs, and other practitioners from around the world who participated in the 1st International Rip Current Symposium. These experts identify advancements in research tha
Super-channel architectures for in-service capacity expansion of CWDM/DWDM systems (2004)

Super-channel architectures enable many narrow spaced wavelengths to be nested into one CWDM (coarse WDM) or DWDM channel at the rates up to 10 Gb/s. This gives a low-cost, growth on demand, capacity upgrades as an overlay to existing CWDM/DWDM systems.
Object-oriented Web application architectures and development strategies (2002)

The development of Web applications is moving quickly due to an explosive increase of Internet use. However, the architectures of current Web applications reveal several constraints and limitations in terms of performance, functionality, usability, etc. Several candidate technologies such as Java, Java RMI, Web-ORB, and Agents have been introduced. However, it has not been studied how these unit technologies can be put together to overcome the limitations of Web applications. As these newer language/technologies are based on the object-oriented paradigm, the object-oriented Web architectures based on these newcomers should be developed. Hence, we first identify the problems and limitations of current Web applications and then propose new architectures of and development strategies for next generation Web applications based on these newer unit technologies. Also three case studies based on the proposed architectures and strategies are presented.