gebhart/patrepeval huggingface dataset:
Spectrum of Mutations in the Gene for Autosomal Recessive Polycystic Kidney Disease (ARPKD/PKHD1) (2003)

ABSTRACT. Autosomal recessive polycystic kidney disease (ARPKD/PKHD1) is an important cause of renal-related and liver-related morbidity and mortality in childhood. Recently mutations in the PKHD1 gene on chromosome 6p21.1-p12 have been identified as the molecular cause of ARPKD. The longest continuous open reading frame (ORF) is encoded by a 67-exon transcript and predicted to yield a 4074–amino acid protein (“polyductin”) of thus far unknown function. By now, a total of 29 different PKHD1 mutations have been described. This study reports mutation screening in 90 ARPKD patients and identifies mutations in 110 alleles making up a detection rate of 61%. Thirty-four of the detected mutations have not been reported previously. Two underlying mutations in 40 patients and one mutation in 30 cases are disclosed, and no mutation was detected on the remaining chromosomes. Mutations were found to be scattered throughout the gene without evidence of clustering at specific sites. About 45% of the changes were predicted to truncate the protein. All missense mutations were nonconservative, with the affected amino acid residues found to be conserved in the murine polyductin orthologue. One recurrent missense mutation (T36M) likely represents a mutational hotspot and occurs in a variety of populations. Two founder mutations (R496X and V3471G) make up about 60% of PKHD1 mutations in the Finnish population. Preliminary genotype-phenotype correlations could be established for the type of mutation rather than for the site of the individual mutation. All patients carrying two truncating mutations displayed a severe phenotype with perinatal or neonatal demise. PKHD1 mutation analysis is a powerful tool to establish the molecular cause of ARPKD in a given family. Direct identification of mutations allows an unequivocal diagnosis and accurate genetic counseling even in families displaying diagnostic challenges. E-mail: [email protected]
Dual mode injection locking of a Fabry-P&#x00E9;rot laser for tunable broadband gain switched comb generation (2015)

We propose a simple technique to generate free-spectral range and wavelength tunable broadband combs by gain-switching and simultaneously injection locking two Fabry-Pérot longitudinal modes. The highly coherent comb source exhibits a 6dB bandwidth of 325GHz and wavelength tunability over 40nm.
Optical median filtering using threshold decomposition (1987)

A hybrid optical/electronic system performs median filtering and related ranked-order operations using threshold decomposition to encode the image. Threshold decomposition transforms the nonlinear neighborhood ranking operation into a linear space-invariant filtering step followed by a point-to-point threshold comparison step. Spatial multiplexing allows parallel processing of all the threshold components as well as recombination by a second linear space-invariant filtering step. An incoherent optical correlation system performs the linear filtering, using a magnetooptic spatial light modulator as the input device and a computergenerated hologram in the filter plane. Thresholding is done electronically. By adjusting the value of the threshold, the same architecture is used to perform median, minimum, and maximum filtering of images.
Effect of Nb and Ti Addition on Microstructure and Hardness of Dual Two-Phase Intermetallic Alloys Based on Ni<SUB>3</SUB>Al-Ni<SUB>3</SUB>V Pseudo-Binary Alloy System (2010)

The microstructures and hardness property of dual two-phase intermetallic alloys that are composed of various kind of volume fractions of geometrically closed packed (GCP) Ni3Al(L12) and Ni3V(D022) phases was studied. Higher volume fraction of primary Ni3Al precipitates was observed in the Ti and Nb added alloys when keeping Al content the same. Also, the microstructures in the eutectoid (channel) region consisting of Ni3Al+Ni3V were sensitive to alloying addition. The hardness of dual two-phase intermetallic alloys was basically explained by mixture rule in hardness between primary Ni3Al precipitates and eutectoid region. Nb and Ti addition raised hardness of dual two-phase intermetallic alloys by solid solution hardening in the constituent phases. This hardening was more significant in Nb addition than in Ti addition. In addition to hardness owing to the mixture rule, additional hardening arising from interfacial area between primary Ni3Al precipitates and eutectoid region was found. With increasing Ni3Al/channel (eutectoid) interfacial area, the additional hardening increased. As temperature increases, the additional hardening monotonously decreased for the base and Nb added alloys but little decreased for the Ti added alloys.
Scheduling with soft constraints (2009)

In a behavioral synthesis system, a typical approach used to guide the scheduler is to impose hard constraints on the relative timing between operations considering performance, area, power, etc., so that the resulting RTL design is favorable in these aspects. The mechanism is often flawed in practice because many such constraints are actually soft constraints which are not necessary, and the constraint system may become inconsistent when many hard constraints are added for different purposes. This paper describes a scheduler that distinguishes soft constraints from hard constraints when exploring the design space. We propose a special class of soft constraints called integer-difference soft constraints, which lead to a totally unimodular constraint matrix in an integer linear programming formulation. By exploiting the total unimodularity, the problem can be solved optimally and efficiently using a linear programming relaxation without expensive branch and bound procedures. We also show how the proposed method can be used to support a variety of design considerations. As an example application, we apply the method to the problem of low-power synthesis with operation gating. In a set of experiments on real-world designs, our method achieves an average of 33.9% reduction in total power; it outperforms a previous method by 17.1% on average and gives close-to-optimal solutions on several designs.
FETRAM. An Organic Ferroelectric Material Based Novel Random Access Memory Cell (2011)

Science and technology in the electronics area have always been driven by the development of materials with unique properties and their integration into novel device concepts with the ultimate goal to enable new functionalities in innovative circuit architectures. In particular, a shift in paradigm requires a synergistic approach that combines materials, devices and circuit aspects simultaneously. Here we report the experimental implementation of a novel nonvolatile memory cell that combines silicon nanowires with an organic ferroelectric polymer—PVDF-TrFE—into a new ferroelectric transistor architecture. Our new cell, the ferroelectric transistor random access memory (FeTRAM) exhibits similarities with state-of-the-art ferroelectric random access memories (FeRAMs) in that it utilizes a ferroelectric material to store information in a nonvolatile (NV) fashion but with the added advantage of allowing for nondestructive readout. This nondestructive readout is a result of information being stored in our cell using a ferroelectric transistor instead of a capacitor—the scheme commonly employed in conventional FeRAMs.
Fluoride-ion solvation in non-aqueous electrolyte solutions (2019)

Non-aqueous electrolytes comprising the fluoride ion are characterized using a variety of experimental and computational methods.
P2P streaming capacity for heterogeneous users with degree bounds (2008)

Peer-to-peer live streaming is a promising way for service providers to offer high-quality IPTV to consumers at reasonable cost. The fundamental problem in peer-to-peer streaming is its capacity problem, or the maximum supported stream rate for a given network with a given peer selection constraint. This problem has been widely studied for small networks where the number of downloading peers is unlimited. For a large networks, however, the number of downloading peers must be limited, which adds an outgoing degree bound for each overlay multicast tree. The streaming capacity problem under uniform degree bound has been first studied in, which solves the problem for homogeneous users, and derives an upper bound for streaming capacity for heterogeneous users. In this paper, we first design an algorithm to construct trees that actually achieve the upper bound for streaming capacity under the case of heterogeneous users with uniform degree bound, and next solve the streaming capacity problem for nonuniform degree bound case also. From our study of the nonuniform degree bound case, we see that the degree bounds do not affect the streaming capacity if the degree bound of each peer is proportional to its uplink capacity.
Control of the STAT6–BCL6 Antagonism by SWAP-70 Determines IgE Production (2013)

Asthma and allergies are major health concerns in which Ig isotype E plays a pivotal role. Ag-bound IgE drives mast cells and basophils into exocytosis, thereby promoting allergic and potentially anaphylactic reactions. The importance of tightly regulated IgE production is underscored by severe immunological conditions in humans with elevated IgE levels. Cytokines direct IgH class-switching to a particular isotype by initiation of germline transcription (GLT) from isotype-specific intronic (I) promoters. The switch to IgE depends on IL-4, which stimulates GLT of the Iε promoter, but is specifically and strongly impaired in Swap-70(-/-) mice. Although early events in IL-4 signal transduction (i.e., activation of the JAK/STAT6 pathway) do not require SWAP-70, SWAP-70 deficiency results in impaired Iε GLT. The affinity of STAT6 to chromatin is reduced in absence of SWAP-70. Chromatin immunoprecipitation revealed that SWAP-70 binds to Iε and is required for association of STAT6 with Iε. BCL6, known to antagonize STAT6 particularly at Iε, is increased on Iε in absence of SWAP-70. Other promoters bound by BCL6 and STAT6 were found unaffected. We conclude that SWAP-70 controls IgE production through regulation of the antagonistic STAT6 and BCL6 occupancy of Iε. The identification of this mechanism opens new avenues to inhibit allergic reactions triggered by IgE.
Highly Versatile β-C(sp<sup>3</sup>)–H Iodination of Ketones Using a Practical Auxiliary (2017)

The first example of palladium(II)-catalyzed β-C(sp<sup>3</sup>)-H iodination of a wide range of ketones using a commercially available aminooxyacetic acid auxiliary has been achieved. This L, X-type directing group overcomes the limitations of the transient directing group approach for C(sp<sup>3</sup>)-H functionalization of ketones. Practical advantages of this method include simple installation of the auxiliary without chromatography, exceptional tolerance of α-functional groups, as well as alkenes and alkynes, and rapid access to diverse sterically hindered quaternary centers.
Design of prognostic circuit for electromigration failure of integrated circuit (2013)

A prognostic circuit for electromigration failure of integrated circuit was proposed, and it was simulated on the base of the SMIC 0.18 um mixed-signal CMOS process model. The prognostic circuit is composed of stress and detection module, two-stage comparator, offset voltage cancellation module, non-overlapping clock generation module, and output module. When the increase amount of resistance for interconnect line exceeds a preset value due to electromigration, the output of the prognostic circuit designed to fail faster will jump from low voltage to high voltage. It indicates the impending failure of hosted circuit because of that the prognostic circuit experiences the same manufacturing process and operational environment as the hosted circuit. The research results are useful for the prediction of performance degradation and failure of integrated circuit.
Reduction of critical current density for domain wall motion in U-shaped magnetic patterns (2008)

The critical current density required for current-induced domain wall motion (Jc) was reduced by decreasing the magnetization (Ms) of the free layer film of a U-shaped pattern for a domain wall motion memory. The Ms was decreased by adding the nonmagnetic elements Ta and Cu to NiFe films. The Jc of the U-shaped patterns with NiFe–Ta and –Cu decreased as the proportion of Ta and Cu in the NiFe increased. The relations between Ms and Jc were examined and it was found that Jc decreased monotonically from 1.2×1012to0.8×1012A∕m2 as Ms decreased from 1.0to0.6T regardless of what other materials were used. It is considered that the decrease in Ms caused a decrease in the hard axis anisotropy of the pattern, and the Jc decreased as predicted by a one-dimensional model.
Autonomous and Autonomic Swarms (2005)

A watershed in systems engineering is represented by the advent of swarm-based systems that accomplish missions through cooperative action by a (large) group of autonomous individuals each having simple capabilities and no global knowledge of the group’s objective. Such systems, with individuals capable of surviving in hostile environments, pose unprecedented challenges to system developers. Design and testing and verification at much higher levels will be required, together with the corresponding tools, to bring such systems to fruition. Concepts for possible future NASA space exploration missions include autonomous, autonomic swarms. Engineering swarm-based missions begins with understanding autonomy and autonomicity and how to design, test, and verify systems that have those properties and, simultaneously, the capability to accomplish prescribed mission goals. Formal methods-based technologies, both projected and in development, are described in terms of their potential utility to swarm-based system developers. 1.
Loss of the circadian clock-associated protein 1 in <i>Arabidopsis</i> results in altered clock-regulated gene expression (1999)

Little is known about plant circadian oscillators, in spite of how important they are to sessile plants, which require accurate timekeepers that enable the plants to respond to their environment. Previously, we identified a circadian clock-associated (CCA1) gene that encodes an Myb-related protein that is associated with phytochrome control and circadian regulation in plants. To understand the role CCA1 plays in phytochrome and circadian regulation, we have isolated an Arabidopsis line with a T DNA insertion that results in the loss of CCA1 RNA, of CCA1 protein, and of an Lhcb-promoter binding activity. This mutation affects the circadian expression of all four clock-controlled genes that we examined. The results show that, despite their similarity, CCA1 and LHY are only partially redundant. The lack of CCA1 also affects the phytochrome regulation of gene expression, suggesting that CCA1 has an additional role in a signal transduction pathway from light, possibly acting at the point of integration between phytochrome and the clock. Our results indicate that CCA1 is an important clock-associated protein involved in circadian regulation of gene expression.
An oxynitride, TaON, as an efficient water oxidation photocatalyst under visible light irradiation (λ ≤ 500 nm) (2002)

Under visible light irradiation (lambda = 420-500 nm), a tantalum oxynitride, TaON, functions as a stable and very efficient photocatalyst for oxidation of water into O2 with a sacrificial electron acceptor (Ag+).
Therapies of mucopolysaccharidosis IVA (Morquio A syndrome) (2013)

ERT, gene therapy and hematopietic stem therapy are clinically and/or experimentally conducted. However, there is no effective curative therapy for bone lesion to date. One of the limitations for Morquio A therapy is that targeting avascular cartilage tissues remains an unmet challenge. ERT or gene therapy with bone-targeting system will improve the bone pathology and skeletal manifestations more efficiently.
<title>Development of trilevel xerographic images</title> (1992)

One exposure, trilevel xerography provides a novel means to produce, single pass, highlight color prints. In this scheme, a ROS creates an imagewise three level, unipolar latent image in which the color information is encoded in the discharge level. The highest charge regions represent one color, and the lowest charge regions a second color. An intermediate charge level, approximately midway between the high and low charge, serves as the background reference and is not printed. The components of the composite two color latent image are developed sequentially using charged area and discharged area development and bipolar developers. Electrostatic transfer is enabled by converting the developed bipolar image to a unipolar image using a pretransfer charging step. Because of the nature of the latent image, a trilevel development system must satisfy demands substantially beyond those required in conventional xerography. This paper will discuss these requirements.© (1992) COPYRIGHT SPIE--The International Society for Optical Engineering. Downloading of the abstract is permitted for personal use only.
Liquid crystal-directed polymer nanostructure for vertically aligned nematic cells (2010)

We demonstrate a fast switching surface polymer-assisted vertically aligned (SPA-VA) liquid crystal (LC) cell. The deposition of the polymer nano spikes as a part of the alignment layer is achieved by polymerizing a small amount of a reactive monomer in vertically aligned liquid crystal in the absent of an applied voltage. The phase-separated polymer localized at the both substrate surfaces and formed nano-sized spikes. These polymer nano-spikes act as internal surface alignment layers and enhance the speed of field-induced reorientation of liquid crystal molecules.
Identification of characteristic oligonucleotides in the bacterial 16S ribosomal RNA sequence dataset (2002)

A computer-based algorithm that characterizes the extent to which any individual oligonucleotide sequence in 16S rRNA is characteristic of any particular bacterial grouping was developed. A measure of signature quality, Q(s), was formulated and subsequently calculated for every individual oligonucleotide sequence in the size range of 5-11 nucleotides and for 15mers with reference to each cluster and subcluster in a 929 organism representative phylogenetic tree. Subsequently, the perfect signature sequences were compared to the full set of 7322 sequences to see how common false positives were. The work completed here establishes beyond any doubt that highly characteristic oligonucleotides exist in the bacterial 16S rRNA sequence dataset in large numbers. Over 16,000 15mers were identified that might be useful as signatures. Signature oligonucleotides are available for over 80% of the nodes in the representative tree.
Diffusion Magnetic Resonance Imaging Detects Progression in <scp>Parkinson's</scp> Disease: A Placebo‐Controlled Trial of Rasagiline (2021)

We found no evidence that 1 mg/day rasagiline has a disease-modifying effect in PD over 1 year. We found pSN free-water increased over 1 year, and baseline free-water relates to clinical motor progression, demonstrating the importance of diffusion imaging parameters for detecting and predicting PD progression. © 2021 International Parkinson and Movement Disorder Society.
Method for in-situ prevention of stable barium carbonate formation in high T[sub c] ceramic superconductor including the use of iodine or an iodine c (1992)

This patent describes a method of making a pre-ceramic resinous material capable of being converted into a ceramic semiconductor material substantially free from stable barium carbonate and having the general composition RR[prime][sub 2]Cu[sub 3]O[sub 7[minus]x] where R is a rare earth metal, R[prime] is an alkaline earth metal, and x is from 0 to 0.5. It comprises: refluxing stoichiometric amounts of a first solution comprising a rare earth isopropoxide and an alkaline earth isopropoxide in isopropanol; adding to the first solution a stoichiometric amount of a second solution comprising copper ethylhexanoate in isopropanol; refluxing the first and second solutions to obtain a precipitate; hydrolyzing the precipitate in a quantity of a first solvent comprising water and isopropanol sufficient to substantially dissolve the precipitate into a precipitate solution; adding a third solution comprising an iodine compound in an alcohol to the precipitate solution to form a precursor solution having at least about 1 mole of iodine per mole of rare earth metal; concentrating the precursor solution by removing a sufficient amount of solvents to produce the pre-ceramic resinous material; and adding a second solvent comprising a nonpolar solvent to the pre-ceramic resinous material to obtain a desired viscosity.
Dialysis without Membranes: How and Why? (2004)

Dialysis between two flowing, miscible fluids without an intervening membrane enhances both the transport rate and biocompatibility. Unfortunately, it also presents serious challenges, including the loss of pressure as a driving force for volume transport, the need for sterile dialysate in greater quantity than in conventional dialysis, the possibility of unacceptable protein loss, and even the possibility of blood cell loss. This paper quantifies these advantages and disadvantages, and evaluate the means by which the latter might be surmounted. Preliminary data are provided to show that stable flows of one fluid sheathing another, miscible fluid are achievable and that molecular exchange between the fluids is orderly and in qualitative agreement with the theory. Extension of the concept to other blood purification tasks, especially in the treatment of liver failure and various macromolecular separations, is also discussed. In conclusion, membraneless separations will require a secondary process and a recycle stream. Under these conditions, its advantages can be preserved and its disadvantages controlled.
Ferrite grain coarsening during transformation of thermomechanically processed C–Mn–Nb austenite (1992)

The ferrite grain size of low carbon steel is known to be refined by hot rolling in the austenite phase field at temperatures too low for recrystallisation to occur. The strain thus retained in the austenite increases ferrite nucleation density and in current models of grain refinement it is assumed that each nucleus becomes a grain in the fully transformed microstructure. In this paper it is shown that, in a heavily deformed C–Mn–Nb austenite, ferrite grains impinged, then coarsened during the initial stages of transformation during continuous cooling. The final ferrite grain size was not established until 35% of transformation had occurred. It is suggested, firstly, that ferrite grain refinement due to controlled rolling cannot be modelled simply from observed increases in nucleation density and, secondly, that deformation of austenite has considerably greater potential for grain refinement than is commonly observed, provided that coarsening of the ferrite during transformation can be limited.MST/1722
A Leaf Monitoring System for Continuous Measurement of Plant Water Status to Assist in Precision Irrigation in Grape and Almond crops (2016)

5th IFAC Conference on Sensing, Control and Automation Technologies for Agriculture (AGRICONTROL) -- AUG 14-17, 2016 -- Seattle, WA