gebhart/patrepeval huggingface dataset:
A founder synonymous COL7A1 mutation in three Danish families with dominant dystrophic epidermolysis bullosa pruriginosa identifies exonic regulatory sequences required for exon 87 splicing (2011)

Dystrophic epidermolysis bullosa pruriginosa (DEB-Pr) (OMIM 604129) represents a distinct variant within the DEB clinical spectrum.1 It is characterized by intense pruritus and distinctive nodular prurigo-like and/or hypertrophic lichenoid lesions mainly localized on the arms, legs and upper shoulders.2 DEB-Pr is caused by either dominant (DDEB-Pr) or recessive mutations in the COL7A1 gene encoding type VII collagen (COLVII).3-5 The full spectrum of COL7A1 mutations in DEB-Pr remains elusive and the genotype–phenotype correlation is largely incomplete. Here, we report and functionally characterize a previously unrecognized translationally silent exonic COL7A1 mutation that results in skipping of exon 87 and is associated with DDEB-Pr phenotypes in several members of three apparently unrelated Danish families. A haplotype segregation study suggested a common ancestor in these kindred. Functional splicing analysis of the mutant exon by a COL7A1 minigene construct and computational prediction for splicing regulatory cis-sequences prove that the mutation alters the activity of an exonic splicing enhancer (ESE) critical for exon inclusion.6, 7 These findings substantiate for the first time the involvement of an ESE mutation in the pathogenesis of DEB and have implications for genetic counselling of Danish families with DDEB.
Johne's Disease Update (2019)

Mycobacterium paratuberculosis causes a granulomatous enteritis in cattle that is commonly known as Johne's Disease or paratuberculosis. Young calves acquire the infection in utero or within the first few months of age by the fecal-oral route. Available diagnostic tests have limitations, but when used with appropriate management practices, a control/eradication program can be designed to meet the needs of the producer. Control programs are aimed at minimizing or eliminating exposure to the organism. There is no curative treatment for paratuberculosis.
The role of apoptosis, proliferation, and the Bcl-2–related proteins in the myelodysplastic syndromes and acute myeloid leukemia secondary to MDS (2000)

Bone marrow CD34(+) cell apoptosis (annexin V), proliferation (Ki-67), and Bcl-2-related protein expression was evaluated by flow cytometry in 102 patients with myelodysplastic syndrome (MDS) and acute myeloid leukemia secondary to MDS (MDS-AML) and in 30 normal donors (NBM). Apoptosis was significantly increased in refractory anemia (RA)/RA with ringed sideroblasts (RARS) (56.9% [20.4%-93.6%]) and refractory anemia with excess blasts (RAEB) (51.2% [25.2%-76. 6%]) compared with NBM (16.7% [3.4%-35.3%], P <.0001). In RA/RARS, apoptosis always exceeded proliferation (Ki-67-positivity, 26.1% [9.5%-47.8%]; apoptosis:proliferation ratio 2.08 [1.15-3.63]); whereas in RAEB, this ratio equalized (1.14 [0.93-2.08]) due to increased proliferation (40.4% [22%-69.5%]). Progression to RAEB in transformation (RAEB-t)/MDS-AML was associated with a significant reduction in apoptosis (22.3% [2.1%-53.2%]; P <.0001) and proliferation (16.8% [1.9%-75.8%); P =.04; ratio 1.69 [0.16-12.21]). Pro-apoptotic (Bax/Bad) versus anti-apoptotic (Bcl-2/Bcl-X) Bcl-2-related protein ratios were increased in RA/RARS compared with NBM (2.57 [1.93-9.42] versus 1.89 [0.65-4.1]; P =.06), whereas disease progression was associated with significantly reduced ratios (1.16 [0.06-3.32]; P <.0001) due primarily to increased Bcl-2 expression. Apoptosis and Bax/Bad:Bcl-2/Bcl-X ratio were inversely correlated with both International Prognostic Scoring System score and cytogenetic risk group; highest levels observed in patients with low score and/or good risk cytogenetics. There was a trend toward an association between Bcl-2-related protein expression and apoptosis (P =.07). This study indicates that MDS progression arises through multiple hits that alter levels of CD34(+) cell apoptosis and proliferation. Early disease is associated with excessive apoptosis and elevated ratio of apoptosis to proliferation. Increased proliferative rates are observed in RAEB, whereas leukemic transformation arises through inhibition of apoptosis rather than excessive cell growth. Although disease progression is accompanied by a fall in pro-apoptotic versus anti-apoptotic Bcl-2-related protein ratios, heterogeneity in patterns of protein expression indicates that factors additional to Bcl-2 family members play a role in the deregulated apoptosis in MDS. (Blood. 2000;96:3932-3938)
TOPSPOT-a new method for the fabrication of microarrays (2002)

This article outlines the new non-contact TOPSPOT method for printing microarrays in a highly parallelized fashion. It is based on a micromachined print head incorporating a central microarray of presently up to 96 vertical nozzles on the output side. Droplets featuring volumes down to 1nl aligned in a 500 /spl mu/m grid are simultaneously ejected by applying a steep air pressure ramp to the open upper side of the liquid. Each of these nozzles is connected to a distinct fluidic reservoir constituting the interface to the macro world. To allow an automated replenishing of the chip, the alignment of the reservoirs is amenable for liquid handling via standard pipetting robots. Depending on the design of the print head, a maximum of 20 /spl mu/l can be stored thus allowing one to dispend about 20.000 droplets of equal quality in a row. Besides its suitability for a robust biochip manufacturing facility in the laboratory, the print head is also well-integrable in a high-throughput production plant. These versions are scheduled to become commercially available in 2000.
Structures of an Apo and a Binary Complex of an Evolved Archeal B Family DNA Polymerase Capable of Synthesising Highly Cy-Dye Labelled DNA (2013)

Thermophilic DNA polymerases of the polB family are of great importance in biotechnological applications including high-fidelity PCR. Of particular interest is the relative promiscuity of engineered versions of the exo- form of polymerases from the Thermo- and Pyrococcales families towards non-canonical substrates, which enables key advances in Next-generation sequencing. Despite this there is a paucity of structural information to guide further engineering of this group of polymerases. Here we report two structures, of the apo form and of a binary complex of a previously described variant (E10) of Pyrococcus furiosus (Pfu) polymerase with an ability to fully replace dCTP with Cyanine dye-labeled dCTP (Cy3-dCTP or Cy5-dCTP) in PCR and synthesise highly fluorescent "CyDNA" densely decorated with cyanine dye heterocycles. The apo form of Pfu-E10 closely matches reported apo form structures of wild-type Pfu. In contrast, the binary complex (in the replicative state with a duplex DNA oligonucleotide) reveals a closing movement of the thumb domain, increasing the contact surface with the nascent DNA duplex strand. Modelling based on the binary complex suggests how bulky fluorophores may be accommodated during processive synthesis and has aided the identification of residues important for the synthesis of unnatural nucleic acid polymers.
Electron Amplification in Diamond (2006)

We report on recent progress toward development of secondary emission “amplifiers” for photocathodes. Secondary emission gain of over 300 has been achieved in transmission mode and emission mode for a variety of diamond samples. Techniques of sample preparation, including hydrogenation to achieve negative electron affinity (NEA), have been adapted to this application.
Improvement of Pointing Time by Predicting Targets in Pointing with a PC Mouse (1998)

A method was proposed for the prediction of a target to which a user is to point with a mouse on the basis of the trajectory of the mouse cursor. An empirical study was carried out in order to evaluate the validity of the proposed prediction algorithm to reduce the pointing time with the prediction accuracy remaining high. The effects of the distance between the edges of two adjacent targets and the position of the indicated target on the prediction accuracy were investigated. Pointing with no prediction mode was also conducted. In the prediction method, the angle between the cursor movement vector and the vector that connected the current cursor position and the center of each target was calculated respectively at various sampling frequencies and intervals, and the minimum cumulative value was determined as the prediction target. The pointing time of the prediction method was shown to be less than that of the control condition for various combinations of the sampling interval and frequency. The trade-off...
Electrochemical energy conversion in fuel cells (1988)

With the exception of high temperature fuel cells, fuel cells burn hydrogen fuel exclusively at low temperature giving energy conversion efficiencies of 40 to 60%. Alkaline cells, phosphoric acid cells, and membrane cells operate at low temperatures, whereas molten carbonate cells and zirconium dioxide membrane cells are designed as high temperature cells. The alcaline fuel cell has so far been used only for special purposes, such as space missions and military applications. The phosphoric acid cell is suitable for modular heating plant between 0.5 and 5 MW which are fueled with natural gas. It has demonstrated its technical reliability in a large-scale field test in the USA and an economic breakthrough is expected in the next few years. The two high temperature cells face a longer development phase, yet hold promise of better economic performance thanks to their greater efficiency.
Sequential steps in Tat trans-activation of HIV-1 mediated through cellular DNA, RNA, and protein binding factors. (1996)

The regulation of HIV expression is controlled by the activity of the Long Terminal Repeat (LTR). Trans-activation by the virally encoded Tat protein is one of the main mechanisms of LTR activation. Tat binds to its target, TAR RNA, and cellular proteins that bind the LTR, Tat, or TAR RNA are important components of the trans-activation process. We will review the factors that have been characterized for a possible involvement in this mechanism. Whereas LTR binding proteins consist of Sp1 and TBP, a large number of factors that bind TAR RNA have been isolated. We have previously cloned two of them by RNA probe recognition: TRBP and La. We have shown that the in vitro and in vivo binding of TRBP to TAR RNA correlates with a constant expression of the protein during HIV-1 infection. Several proteins that interact with Tat have mainly positive, but some negative, effects on trans-activation. Genetic studies have defined that human chromosome 12 encodes a protein that will allow trans-activation in rodent cells. The binding and the functional data about these proteins suggest sequential steps for the Tat trans-activation mechanism. Each of these intracellular molecular events could be the target for molecular intervention against the virus.
Characteristics of the Vold-Kalman order tracking filter (2002)

The filter characteristics of the Vold-Kalman (1993, 1960, 1961) order tracking filter are presented. Both the frequency response as well as the time response and their time-frequency relationship have been investigated for different filter types and guidelines for optimum choice of filter parameters are presented. The Vold-Kalman filter allows for the high performance simultaneous tracking of orders in systems with multiple independent shafts. With this new filter and using multiple tacho references, waveforms, as well as amplitude and phase may be extracted without the beating interactions that are associated with conventional methods. Orders extracted as waveforms have no phase bias, and may hence be used for playback, synthesis and tailoring.
The origin and identity of embryonic stem cells (2010)

Embryonic stem (ES) cells are used extensively in biomedical research and as a model with which to study early mammalian development, but their exact origin has been subject to much debate. They are routinely derived from pre-implantation embryos, but it has been suggested that the cells that give rise to ES cells might arise from epiblast cells that are already predisposed to a primordial germ cell (PGC) fate, which then progress to ES cell status via the PGC lineage. Based on recent findings, we propose here that ES cells can be derived directly from early epiblast cells and that ES cells might arise via two different routes that are dictated by their culture conditions.
Amphiphilic hyperbranched polymers from the copolymerization of a vinyl and divinyl monomer: The potential of catalytic chain transfer polymerization (2013)

This feature article focuses on the synthesis of amphiphilic hyperbranched polymers. There is a growing interest in the properties of these nanostructured materials and the copolymerization of a vinyl and divinyl monomer in the presence of a chain transfer agent, provides a simple, versatile and scalable synthesis route. In particular the use of catalytic chain transfer (CCT) will be highlighted. CCT is a very efficient and versatile chemistry for molecular weight control and the synthesis of vinyl ω-end functionalized polymers (e.g. macromonomers) in free radical polymerization. This makes CCT an interesting option for the synthesis of hyperbranched polymers as the use of cobaloxime complexes allows for control over the polymer architecture as well as ω-endgroup functionality. An overview of the synthesis of hyperbranched polymers via the copolymerization of a vinyl and divinyl monomer is presented, followed with a number of examples of amphiphilic hyperbranched polymers and their applications.
Self-Timed Scheduling Analysis for Real-Time Applications (2007)

This paper deals with the scheduling analysis of hard real-time streaming applications. These applications are mapped onto a heterogeneous multiprocessor system-on-chip (MPSoC), where we must jointly meet the timing requirements of several jobs. Each job is independently activated and processes streams at its own rate. The dynamic starting and stopping of jobs necessitates the usage of self-timed schedules (STSs). By modeling job implementations using multirate data flow (MRDF) graph semantics, real-time analysis can be performed. Traditionally, temporal analysis of STSs for MRDF graphs only aims at evaluating the average throughput. It does not cope well with latency, and it does not take into account the temporal behavior during the initial transient phase. In this paper, we establish an important property of STSs: the initiation times of actors in an STS are bounded by the initiation times of the same actors in any static periodic schedule of the same job; based on this property, we show how to guarantee strictly periodic behavior of a task within a self-timed implementation; then, we provide useful bounds on maximum latency for jobs with periodic, sporadic, and bursty sources, as well as a technique to check latency requirements. We present two case studies that exemplify the application of these techniques: a simplified channel equalizer and a wireless LAN receiver.
Instantaneous frequency estimation by the reassigned Stockwell spectrogram (2012)

The Stockwell transform, a mix of the short-time Fourier transform and the wavelet transform, has been widely used in practice in the last decade due to its easily interpretable and multi-resolution time-frequency characteristics. In this paper, we introduce the reassigned Stockwell spectrogram by re-mapping the surface of the spectrogram of the Stock-well transform with the aim to improve its readability. We first define and mathematically validate the channelized instantaneous frequency (CIF) and the local group delay (LGD) for the Stockwell transform. At any given point in the time-frequency domain, the associated CIF and LGD provide a re-estimation of the time arrival and instantaneous frequency of the signal component observed at that point. Thus, the reassigned Stockwell spectrogram has signal energy highly concentrated at the instantaneous frequency/group delay curves and greatly increases the resolution and readability of the time-frequency structure of the underlying signal. The instantaneous frequencies of signal components can then be extracted by detecting the local energy peaks in the reassigned Stockwell spectrogram. The difference between the reassigned and conventional Stockwell spectrograms is illustrated using both synthetic and real signals.
CARBON-13 NUCLEAR MAGNETIC RESONANCE SPECTRA OF CYCLODEXTRINS AND ITS PERACETATES (1973)

Abstract Carbon-13 chemical shifts for α-, β-, and γ-cyclodextrins and its peracetates, and also amylose, in several solvents, are reported with full assignment. It was found that the steric effects imposed by cyclic nature of the dextrins on the conformation of the glucopyranose residues in the dextrins are clearly reflected in their spectra.
Conversion of borate-based glass scaffold to hydroxyapatite in a dilute phosphate solution (2010)

Porous scaffolds of a borate-based glass (composition in mol%: 6Na2O, 8K2O, 8MgO, 22CaO, 36B2O3, 18SiO2, 2P2O5), with interconnected porosity of approximately 70% and pores of size 200-500 microm, were prepared by a polymer foam replication technique. The degradation of the scaffolds and conversion to a hydroxyapatite-type material in a 0.02 M K2HPO4 solution (starting pH = 7.0) at 37 degrees C were studied by measuring the weight loss of the scaffolds, as well as the pH and the boron concentration of the solution. X-ray diffraction, scanning electronic microscopy and energy dispersive x-ray analysis showed that a hydroxyapatite-type material was formed on the glass surface within 7 days of immersion in the phosphate solution. Cellular response to the scaffolds was assessed using murine MLO-A5 cells, an osteogenic cell line. Scanning electron microscopy showed that the scaffolds supported cell attachment and proliferation during the 6 day incubation. The results indicate that this borate-based glass could provide a promising degradable scaffold material for bone tissue engineering applications.
A Coil Design for Transcranial Magnetic Stimulation of Deep Brain Regions (2002)

Noninvasive magnetic stimulation of the human central nervous system has been used in research and the clinic for several years. However, the coils used previously stimulated mainly the cortical brain regions but could not stimulate deeper brain regions directly. The purpose of the current study was to develop a coil to stimulate deep brain regions. Stimulation of the nucleus accumbens and the nerve fibers connecting the prefrontal cortex with the nucleus accumbens was one major target of the authors' coil design. Numeric simulations of the electrical field induced by several types of coils were performed and accordingly an optimized coil for deep brain stimulation was designed. The electrical field induced by the new coil design was measured in a phantom brain and compared with the double-cone coil. The numeric simulations show that the electrical fields induced by various types of coils are always greater in cortical regions (closer to the coil placement); however, the decrease in electrical field within the brain (as a function of the distance from the coil) is markedly slower for the new coil design. The phantom brain measurements basically confirmed the numeric simulations. The suggested coil is likely to have the ability of deep brain stimulation without the need to increase the intensity to levels that stimulate cortical regions to a much higher extent and possibly cause undesirable side effects.
Applied Concepts in PBPK Modeling: How to Build a PBPK/PD Model (2016)

The aim of this tutorial is to introduce the fundamental concepts of physiologically based pharmacokinetic/pharmacodynamic (PBPK/PD) modeling with a special focus on their practical implementation in a typical PBPK model building workflow. To illustrate basic steps in PBPK model building, a PBPK model for ciprofloxacin will be constructed and coupled to a pharmacodynamic model to simulate the antibacterial activity of ciprofloxacin treatment.
조선후기 산림정책 및 산림황폐화: 시장주의적 고찰과 그에 대한 비판 (2008)

The Joseon Dynasty experienced the devastation of forests in the 18th and 19th centuries. This study insists that it resulted from the pre-modern socio-economic system of the dynasty. The social system of Joseon lacked the capacity to handle the structural problem. In the retarded social system, all the forests in the country became virtually common resources. People fought each other to get more private profits from the common resources. The people of the lower classes suffered from double and triple distress in the situation. We can consider the following three alternatives to resolve the problem: strict administrative regulation; agreement between interested parties; and authorization of ownership. Economics teaches that the last one is most effective. However, this study points out all the alternatives useless in those days because the social system itself of the country was totally corrupted, outmoded and incompetent.
Enhanced Emission Using Thin Li-Halide Cathodic Interlayers for Improved Injection into Poly(p-phenylene vinylene) Derivative PLEDs (2008)

In this study, the effects of thin Li-halide cathodic interlayers on electron injection were examined for electroluminescent layers of polymer light-emitting diodes (PLEDs). An order of magnitude increase in current density is observed as Li-halide salts are varied down the group VII column of the periodic table. When considering luminance, devices with a LiCl interlayer were 2.3× greater than those with LiF, whereas devices with LiBr were 2.8× greater, while concurrently lowering the turn-on voltage. This resulting enhanced current density and subsequent luminance could be due to a lowered work-function difference at the cathode created by either the Li-halides dissociation-induced doping of the polymer surface or an interfacial dipole of ionic Li-halide compound, leading to band bending.
ChemInform Abstract: Solid State NMR (1996)

Abstract ChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 100 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a “Full Text” option. The original article is trackable via the “References” option.
Position-Based Modeling for Wireless Channel on High-Speed Railway under a Viaduct at 2.35 GHz (2012)

This paper presents a novel and practical study on the position-based radio propagation channel for High-Speed Railway by performing extensive measurements at 2.35 GHz in China. The specification on the path loss model is developed. In particular, small scale fading properties such as K-factor, Doppler frequency feature and time delay spread are parameterized, which show dynamic variances depending on the train location and the transceiver separation. Finally, the statistical position-based channel models are firstly established to characterize the High-Speed Railway channel, which significantly promotes the evaluation and verification of wireless communications in relative scenarios.
最新Javaハンドブック : 図解・標準 : 言語構造から学ぶオブジェクト指向プログラミング入門 (2001)

始める前の基礎知識 変数／定数／データ型 演算子 処理のコントロール方法 繰り返し 配列 クラスの基本 クラスのプログラミングへの応用 クラスの継承 抽象クラスとインターフェイス パッケージ 例外処理 入出力処理 スレッド アプレット ウィンドウコンポーネント ＦｏｒｔｅによるＳｗｉｎｇコンポーネントの作成
A general constraint-centric scheduling framework for spatial architectures (2013)

Specialized execution using spatial architectures provides energy efficient computation, but requires effective algorithms for spatially scheduling the computation. Generally, this has been solved with architecture-specific heuristics, an approach which suffers from poor compiler/architect productivity, lack of insight on optimality, and inhibits migration of techniques between architectures.