gebhart/patrepeval huggingface dataset:
Mutations in GDP-Mannose Pyrophosphorylase B Cause Congenital and Limb-Girdle Muscular Dystrophies Associated with Hypoglycosylation of α-Dystroglycan (2013)

Congenital muscular dystrophies with hypoglycosylation of α-dystroglycan (α-DG) are a heterogeneous group of disorders often associated with brain and eye defects in addition to muscular dystrophy. Causative variants in 14 genes thought to be involved in the glycosylation of α-DG have been identified thus far. Allelic mutations in these genes might also cause milder limb-girdle muscular dystrophy phenotypes. Using a combination of exome and Sanger sequencing in eight unrelated individuals, we present evidence that mutations in guanosine diphosphate mannose (GDP-mannose) pyrophosphorylase B (GMPPB) can result in muscular dystrophy variants with hypoglycosylated α-DG. GMPPB catalyzes the formation of GDP-mannose from GTP and mannose-1-phosphate. GDP-mannose is required for O-mannosylation of proteins, including α-DG, and it is the substrate of cytosolic mannosyltransferases. We found reduced α-DG glycosylation in the muscle biopsies of affected individuals and in available fibroblasts. Overexpression of wild-type GMPPB in fibroblasts from an affected individual partially restored glycosylation of α-DG. Whereas wild-type GMPPB localized to the cytoplasm, five of the identified missense mutations caused formation of aggregates in the cytoplasm or near membrane protrusions. Additionally, knockdown of the GMPPB ortholog in zebrafish caused structural muscle defects with decreased motility, eye abnormalities, and reduced glycosylation of α-DG. Together, these data indicate that GMPPB mutations are responsible for congenital and limb-girdle muscular dystrophies with hypoglycosylation of α-DG.
Preparation and Reactions of Derivatives of Squaric Acid. Alkoxy-, Hydroxy-, and Aminocyclobutenediones<sup>1</sup> (1966)

ADVERTISEMENT RETURN TO ISSUEPREVArticleNEXTPreparation and Reactions of Derivatives of Squaric Acid. Alkoxy-, Hydroxy-, and Aminocyclobutenediones1Sidney Cohen and Saul G. CohenCite this: J. Am. Chem. Soc. 1966, 88, 7, 1533–1536Publication Date (Print):April 1, 1966Publication History Published online1 May 2002Published inissue 1 April 1966https://pubs.acs.org/doi/10.1021/ja00959a040https://doi.org/10.1021/ja00959a040research-articleACS PublicationsRequest reuse permissionsArticle Views1797Altmetric-Citations118LEARN 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
Synthesis, Characterization, and Growth Mechanism of Tellurium Nanotubes (2004)

A simple hydrothermal reduction method, employing sodium tellurate (Na2TeO4·2H2O) as tellurium source and formamide (HCONH2) as a reductant, was used to prepare and investigate tellurium nanotubes. The diameters of the nanotubes range from 200 to 600 nm and their lengths from 4 to 15 μm. Unlike studies reported previously,1,2 a series of electron microscopy characterization results suggests that the growth of tellurium nanotubes under the present experimental conditions is governed by a nucleation−dissolution−recrystallization growth mechanism: sphere-like tellurium nanoparticles initially formed in the hydrothermal system; the sphere-like nanoparticles were gradually dissolved to generate free tellurium atoms in the solution; these tellurium atoms were renewedly transferred onto the surfaces of the sphere-like nanoparticles and evolved into groove-like nanorods; the groove-like nanorods could be grown into tellurium nanotubes eventually.
Carbocations and Electrophilic Reactions (1973)

Abstract The general concept of carbocations encompasses all cations of carbon containing compounds which can be differentiated into two distinct classes: trivalent (“classical”) carbenium ions and pentacoordinated (“nonclassical”) carbonium ions. In this paper the preparation and structural study (by NMR, IR, Raman and ESCA spectroscopy) of stable carbenium ions and carbonium ions are discussed. As is well known, trivalent carbenium ions play an important role in electrophilic reactions of π‐ and n‐donor systems. Similarly, pentacoordinated carbonium ions are the key to electrophilic reactions of σ‐donor systems (single bonds). The ability of single bonds to act as σ‐donor lies in their ability to form carbonium ions via triangular two electron, three‐center bond formation. Consequently there seems to be no principle difference between the electrophilic reactions of π‐ and σ‐bonds except that the former react more easily, even with weak electrophiles, whereas the latter necessitate more severe conditions. The role of carbocations in electrophilic reactions of π‐ and σ‐donor systems is discussed.
An approach to multilayer microfluidic systems with integrated electrical, optical, and mechanical functionality (2005)

A general solution is presented for the realization of microfluidic systems containing multiple compartments with sequential functions. The approach utilizes multilayer plastic/glass/silicon microfluidic system configurations with integrated electrical, optical, and fluid control functionalities. The approach allows for the integration of on-column, on-chip electrical conductivity/impedance detectors and driving electrodes, as well as for the integration of on-column, off-chip optical detection using resonance light scattering. A process-compatible latex microvalve is demonstrated and characterized. The procedure utilizes a novel packaging technique for realizing integrated fluid and electrical interfaces between the microsystem and conventional tubing/instrumentation. The microfluidic system demonstrated in this work contains some of the most commonly used components in bioanalysis systems and can be used as a platform to fabricate more sophisticated microscale bioanalysis systems for a variety of applications.
The effects of corporate social disclosure on firm performance: empirical evidence from Bangladesh (2017)

Purpose: This study examines the influence of corporate social disclosure on firm performance within the context of Bangladesh.\nDesign/methodology/approach: This study develops hypotheses with the light of legitimacy and signalling theory using top 200 firms listed on the Dhaka Stock Exchange, Bangladesh. Corporate social disclosure (CSD) data are based on the period from 2011 to 2013, and the firm performance (FP) is based on the respective following year 2012 to 2014. A corporate social disclosure index (CSDI) including three categories (long-term, short- term and general disclosure) is constructed to measure the extent of social disclosures in annual reports. Firm performance is measured based on three indicators including Return on Asset (ROA), Market Capitalization, and Tobin Q. Ordinary Least Square (OLS) and Two Stage Least Square (2SLS) are used in analysing the data.\nFindings: It is found that there is a significant relationship between corporate social disclosure and the following year's firm performance for every performance indicator. It is also found that long-term disclosure plays a key role in influencing the firm performance.\nOriginality/ value: CSD is country-specific and, hence, the effects of corporate social disclosure on firm performance can vary from country to country. This study enhances understating of such relationship as it considers within the context of a developing country. The finding of this study is robust as every indicator of firm performance is found to be related positively to firm performance.
Core-Log Integration For A Saudi Arabian Sandstone Reservoir (1995)

ABSTRACT For a detailed characterization of a reservoir, core-log integration is essential. In this paper, data integration from logs and cores of a Saudi Arabian sandstone reservoir is discussed with particular attention to effects of clay on resistivity logs and water saturation. There are four sources of data, namely, core resistivity measurement, clay study from cores (XRD, CEC), spectral core gamma ray, and well logs. In order to generate continuous cation exchange capacity (CEC) with depth, spectral gamma ray measurements (both from core and downhole log) and CEC from cores are correlated. Qv (CEC per unit pore volume) values are calculated utilizing only well logs by applying Waxman-Smits equation in water bearing zone. Log derived Qv values from water zone were then correlated with porosity to generate Qv values in the oil column and compared with core derived Qv. Finally, data from well logs (porosity, resistivity and qv) and cores (resistivity parameters m, n, and Qv) were integrated for more accurate water saturation calculation. The core-log correlation can be applied to other wells avoiding expensive core analysis, and the technique developed in this project can be used in other sandstone reservoirs.
Static determination of dynamic properties of generalized type unions (1977)

The classical programming languages such as PASCAL or ALGOL 68 do not provide full data type security. Run-time errors are not precluded on basic operations. Type safety necessitates a refinement of the data type notion which allows subtypes. The compiler must also be able to ensure that basic operations are applicable. This verification consists in determining a local subtype of globally declared variables or constants. This may be achieved by improved compiler capabilities to analyze the program properties or by language constructs which permit the expression of these properties. Both approaches are discussed and illustrated by the problems of access to records via pointers, access to variants of record structures, determination of disjoint collections of linked records, and determination of integer subrange. Both approaches are complementary and a balance must be found between what must be specified by the programmer and what must be discovered by the compiler.
Occurrence of Components of Fibrinolytic Pathways in Situ in Laryngeal Cancer (2003)

Malignancy is characterized by the occurrence of components of coagulation reaction pathways in situ within tumor tissues detectable immunohistochemically. However, tumors vary in the details of this coagulation-cancer interaction. We have previously described tumor cell-associated tissue factor (TF), factor (F) VII, and F X in laryngeal carcinoma tissues. Fibrinogen and F XIIIa were found in the tumor connective tissue. Tissue factor pathway inhibitor (TFPI) occurred in the tumor connective tissue and on microvascular endothelial cells and normal squamous epithelial cells but not in the tumor cells. Fibrin (thrombin-cleaved fibrinogen) existed at the host-tumor interface and the margins of tumor nodules consistent with an active tumor cell-associated clotting pathway in this tumor type. Studies were extended here to detect components of fibrinolytic pathways. Plasminogen and tissue plasminogen activator (t-PA) were detected on laryngeal tumor cells, particularly in more well-differentiated cases. Low-molecular-weight urokinase plasminogen activator (LMW u-PA) was primarily a feature of more undifferentiated laryngeal carcinoma cells. Staining to a lesser extent was found for high-molecular-weight u-PA (HMW u-PA) on tumor cells and various normal cell types in the tumor tissue. Relatively weak and variable tumor cell staining was found for plasminogen activator inhibitors (PAI) 1, 2, and 3. Trace staining was found for u-PA receptor (u-PAR) in differentiated tumor cells. The significance of coagulation and fibrinolytic pathways present in situ to the economy of laryngeal carcinoma remains to be determined.
Inter-Symbol / Inter-Frame Interference in Time-Hopping Ultra Wideband Impulse Radio System (2006)

A closed-form expression for the intersymbol/inter-frame interference variance is derived in the context of a time-hopping ultra wideband impulse radio based system. This enables us to theoretically analyze on the performance the influence of the time-hopping codes, of the rake receiver number of fingers, and of the guard-time size. Simulations sustain our claims.
A Gaussian Mixture Model Based Classification Scheme for Myoelectric Control of Powered Upper Limb Prostheses (2005)

This paper introduces and evaluates the use of Gaussian mixture models (GMMs) for multiple limb motion classification using continuous myoelectric signals. The focus of this work is to optimize the configuration of this classification scheme. To that end, a complete experimental evaluation of this system is conducted on a 12 subject database. The experiments examine the GMMs algorithmic issues including the model order selection and variance limiting, the segmentation of the data, and various feature sets including time-domain features and autoregressive features. The benefits of postprocessing the results using a majority vote rule are demonstrated. The performance of the GMM is compared to three commonly used classifiers: a linear discriminant analysis, a linear perceptron network, and a multilayer perceptron neural network. The GMM-based limb motion classification system demonstrates exceptional classification accuracy and results in a robust method of motion classification with low computational load.
A digital signal processor for low power (2003)

Low power in digital signal processor design has been a critical design constraint since portable battery operated devices prevailed. Most of the power dissipation of a processor is in the clock network and the on-chip memory. By optimizing the critical path of the processor we could reduce the power of clock network. We propose an energy-efficient instruction set architecture to reduce the power consumption of the program memory access. We applied it to a digital hearing aid, and reduced the program memory size by approximately 75%.
Isomeric five-membered ring phosphites and phosphates (1969)

ADVERTISEMENT RETURN TO ISSUEPREVArticleNEXTIsomeric five-membered ring phosphites and phosphatesDorothy Z. Denney, Gwendyline Y. Chen, and Donald B. DenneyCite this: J. Am. Chem. Soc. 1969, 91, 24, 6838–6841Publication Date (Print):November 1, 1969Publication History Published online1 May 2002Published inissue 1 November 1969https://pubs.acs.org/doi/10.1021/ja01052a050https://doi.org/10.1021/ja01052a050research-articleACS PublicationsRequest reuse permissionsArticle Views172Altmetric-Citations36LEARN 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
Sweeteners (1991)

This chapter reviews the history of sweetener discovery (often serendipitous) and outlines directed approaches to sweetener discovery. Mechanisms of sweet taste transduction are being investigated at the cellular and molecular level, and computer-aided molecular design is being successfully applied to the discovery of new sweet entities.
Solid-phase synthesis of hentriacontanucleotide (1981)

ADVERTISEMENT RETURN TO ISSUEPREVArticleNEXTSolid-phase synthesis of hentriacontanucleotidePiotr Dembek, Kenichi Miyoshi, and Keiichi ItakuraCite this: J. Am. Chem. Soc. 1981, 103, 3, 706–708Publication Date (Print):February 1, 1981Publication History Published online1 May 2002Published inissue 1 February 1981https://pubs.acs.org/doi/10.1021/ja00393a053https://doi.org/10.1021/ja00393a053research-articleACS PublicationsRequest reuse permissionsArticle Views81Altmetric-Citations28LEARN 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
Temperature-Dependent Electric Characteristics in an E7/CNT Colloid (2007)

Temperature effects on the electric characteristics were studied in a colloidal system made up of 0.05-wt.% multiwalled carbon nanotubes dispersed in the nematic liquid crystal E7. The electric properties were observed via measurement of transient current in a homogeneously aligned liquid-crystal cell induced by a polarity-reversed field. The cell filled with the suspension and that containing pristine liquid crystal exhibited similar voltage- and temperature-dependent behaviors in the experiments. However, it was interestingly found that the transient current originating from the transport of impurity ions was remarkably suppressed in the suspension. The enhancement of the ionic mobility was also observed in such a colloidal system.
Production of optically active epoxides by microbial oxidation of olefins. (1987)

Some of the microorganisms which assimilate hydrocarbons produce optically active epoxides from corresponding olefins. Although the substrates epoxidized by most of these microorganisms are restricted to aliphatic olefins, one of the gaseous olefin assimilating microorganisms, N.corallina, has been found to have a very wide substrate specificity in the epoxidation of olefins. This microorganism produces optically active epoxides from 1-alkenes (C3-C18), 2-alkenes, halogenated alkenes, styrene, phenyl allyl ether and their analogues. The rates of epoxidation of these substrates have been promoted by reducing the inhibitory effect of produced epoxides. In the epoxidation of moderate chain length 1-alkenes, the yield of (R) -1, 2-epoxides exceeded 80%. The optical purity of these 1, 2-epoxyalkanes ranges from 80 to 90% e.e.. These 1, 2-epoxyalkanes are now utilized as the intermediates in the syntheses of ferroelectric liquid crystals.
HIV global surveillance: Foundation for retroviral discovery and assay development (2006)

The high level of HIV genetic diversity has important implications for screening, diagnostic testing and patient monitoring. Continued diversification and global redistribution of HIV groups, subtypes and recombinants make it imperative that serological and molecular assays be designed and evaluated to ensure reliable performance on all HIV infections. Recognizing the importance of this issue, we initiated a comprehensive program to monitor global diversification of HIV, search for newly emerging variants, assemble large-volume panels of genetically and geographically diverse strains, and develop strategies to determine the impact of HIV diversity on assays used for detecting and monitoring HIV infection. Efforts to identify and characterize rare and emerging HIV strains have lead to the identification of HIV-1 group O, group N, and dual infections of groups M and O. A panel of plasma specimens was established that includes specimens collected from 12 countries in Africa, Asia, Europe, and South America; the panel comprises infections due to HIV-1 group M subtypes A, B, C, D, F, and G, as well as CRF01, CRF02, and unique recombinant forms, group N, and group O. Serological and molecular characterization of this unique panel has provided vital sequence data to support assay development and an invaluable source of well-defined specimens to evaluate and compare assay performance. The ability to address the challenge posed by ongoing evolution of HIV and the emergence of new variants requires continued surveillance of global HIV strain diversity, a sound scientific foundation for assay development, and suitable panels to evaluate and validate assay performance.
Gunn-Effect Amplifiers for Microwave Communication Systems in X, Ku, and Ka Bands (1975)

This paper describes the design and performance of small-signal stable multistage Gunn-effect reflection-type amplifiers for communication systems in X, Ku, and Ka bands. A single-stage design approach is developed, based on measured small-signal Z parameters of the Gunn diodes. This technique is then applied to a microstrip medium at lower frequencies (X and Ku bands) and to a coax/waveguide hybrid structure at Ku band. Performance of a two-stage amplifier is described in the bands 11.7 to 12.2 GHz and 14.0 to 14.5 GHz. In high Ka band, performance of both a two- and a four-stage amplifier is presented.
Utility Analysis: Current Trends and Future Directions (2001)

Utility analysis procedures offer organizational decision‐makers useful information regarding the relative values of different interventions. Years of research have resulted in a number of practically viable utility models and extensions. There is a continued need for research to examine the accuracy of utility estimates and to further compare the different models. A more recent research concern is that of low levels of acceptance of utility analysis results by practitioners. Many researchers are turning their attention to ways in which this acceptance may be increased. This article reviews different utility models as well as a number of important extensions. It then discusses current utility analysis issues, such as the aforementioned acceptance problem and the introduction of a multi‐attribute utility model. The article concludes with suggestions for future utility analysis research.