gebhart/patrepeval huggingface dataset:
Lipofuscinosis ceroide neuronal tipo juvenil. Enfermedad Spielmeyer - vogt - sjögren - batten. Presentacion de una familia. Juvenile Neuronal Ceroid-lipofuscinoses (Spielmeyer - vogt - sjögren – Batten's Disease). A case report in a family (2002)

Se describen por primera vez en Pinar del Rio (Cuba), 2 pacientes (varon y hembra), provenientes de una familia sin aparente consanguinidad, que presentaron una enfermedad degenerativa del Sistema Nervioso Central del tipo de la Lipofuscinosis Ceroide Neuronal, forma juvenil, o Enfermedad de Spielmeyer - Vogt - Sjogren - Batten. El modo de aparicion en dos hermanos de diferente sexo con padres normales, lo hace compatible con una herencia autosomica recesiva. El diagnostico se baso en los hallazgos clinicos, electrofisiologicos y ultraestructurales. Se hace enfasis en el estudio morfologico ultraestructural, como criterio confirmatorio y para la clasificacion de estas entidades. DeCS: LIPOFUSCINOSIS CEROIDE NEURONAL/ diagnostico - genetica - ultraestructura, INFORME DE CASO, PINAR DEL RIO. ABSTRACT For the first time it's described in Pinar del Rio (Cuba) two patients (male and female), hailing from a family without apparent consanguinity, they presented a degenerative disease of the Central Nervous System type Neuronal Ceroid Lipofuscinosis, juvenile or Spielmeyer - Vogt - Sjogren - Batten Disease. The onset of the identity in two siblings of different sex with normal parents is compatible with a recessive autosomal inheritance. Diagnosis was based on clinical, electrophysiological and ultraestructural findings. It is emphasized that ultraestructural morphologic study is a comfirmative criterium and for the classification of these entities. DeCS: NEURONAL CEROID LIPOFUSCINOSIS/diagnosis, genetic, ultraestructure, CASE REPORT, PINAR DEL RIO.
Sodium-Calcium Exchange: A Molecular Perspective (2000)

Plasma membrane Na(+)-Ca2+ exchange is an essential component of Ca2+ signaling pathways in several tissues. Activity is especially high in the heart where the exchanger is an important regulator of contractility. An expanding exchanger superfamily includes three mammalian Na(+)-Ca2+ exchanger genes and a number of alternative splicing products. New information indicates that the exchanger protein has nine transmembrane segments. The exchanger, which transports Na+ and Ca2+, is also regulated by these substrates. Some molecular information is available on regulation by Na+ and Ca2+ and by PIP2 and phosphorylation. Altered expression of the exchanger in pathophysiological states may contribute to various cardiac phenotypes. Use of transgenic approaches is beginning to improve our knowledge of exchanger function.
Small-footprint Keyword Spotting Using Deep Neural Network and Connectionist Temporal Classifier (2017)

Mainly for the sake of solving the lack of keyword-specific data, we propose one Keyword Spotting (KWS) system using Deep Neural Network (DNN) and Connectionist Temporal Classifier (CTC) on power-constrained small-footprint mobile devices, taking full advantage of general corpus from continuous speech recognition which is of great amount. DNN is to directly predict the posterior of phoneme units of any personally customized key-phrase, and CTC to produce a confidence score of the given phoneme sequence as responsive decision-making mechanism. The CTC-KWS has competitive performance in comparison with purely DNN based keyword specific KWS, but not increasing any computational complexity.
Cascade Coupling/Cyclization Process to N-Substituted 1,3-Dihydrobenzimidazol-2-ones (2007)

Assembly of N-substituted 1,3-dihydrobenzimidazol-2-ones is achieved starting from methyl o-haloarylcarbamates via a CuI/amino acid catalyzed coupling with amines and subsequent condensative cyclization. A number of functional groups are tolerated by these reaction conditions, including vinyl, nitro, carboxylate, amide, ester, ketone, and silyl ether groups.
Dyno Test Investigations of Gasoline Engine Fueled with Butanol-Gasoline Blends (2009)

<div class="htmlview paragraph">As the issue of oil shortage and air pollution caused by automotive engine emissions become more and more serious day by day, researchers and engineers from all over the world are seeking for alternative fuels of lower pollution and renewable nature. This paper discusses in detail the feasibility of fueling gasoline engines with Butanol-gasoline blends. Besides the production, transportation, storage, physical and chemical properties of Butanol-gasoline blends, the combustion characteristics were analyzed as well. As the result Butanol was considered an excellent alternative fuel for gasoline engines, with many unique advantages superior to Natural Gas, LPG, Carbinol and Ethanol, the latter are widely studied at present time. In order to validate the above conclusions in engine application, engine dyno tests were conducted for a gasoline engine fueled with different concentrations of Butanol blend ranging from 10% to up to 35%. Test results showed that the engine power level maintained, without any necessary modifications to the engine, when the Butanol concentration is below 20% by volume. The maximum engine power drops and BSFC goes up slightly, however, when the concentration of Butanol approaches 30%, but those could be recovered by optimizing engine operational parameters, such as advancing the spark timing. Significant improvement to the overall fuel economy is observed in terms of reducing the total fuel energy consumption to produce unit crankshaft energy output. In all the cases investigated engine raw HC and CO emissions are significantly reduced but NOx emissions go up. Finally, approaches to reduce NOx emissions are discussed, with test investigations planed as future work.</div>
GRAPHITE-EXPANDED POLYTETRAFLUOROETHYLENE (1977)

The Biomedical Engineering Research and Education Program at Arizona State University, Tempe, Arizona, and St. Joseph's Hospital, Phoenix, Arizona.
Hindgut Fermentation in Three Species of Marine Herbivorous Fish (2002)

Symbioses with gut microorganisms provides a means by which terrestrial herbivores are able to obtain energy. These microorganisms ferment cell wall materials of plants to short-chain fatty acids (SCFA), which are then absorbed and used by the host animal. Many marine herbivorous fishes contain SCFA (predominantly acetate) in their hindgut, indicative of gut microbial activity, but rates of SCFA production have not been measured. Such information is an important prerequisite to understanding the contribution that gut microorganisms make in satisfying the energy needs of the fish. We have estimated the rates of acetate production in the gut of three species of temperate marine herbivorous fish from northeastern New Zealand: Kyphosus sydneyanus (family Kyphosidae), Odax pullus (family Odacidae), and Aplodactylus arctidens (family Aplodactylidae). Ex vivo preparations of freshly caught fish were maintained with their respiratory and circulatory systems intact, radiolabeled acetate was injected into ligated hindgut sections, and gut fluid was sampled at 20-min intervals for 2 h. Ranges for acetate turnover in the hindguts of the studied species were determined from the slope of plots as the log of the specific radioactivity of acetate versus time and pool size, expressed on a nanomole per milliliter per minute basis. Values were 450 to 570 (K. sydneyanus), 373 to 551 (O. pullus), and 130 to 312 (A. arctidens). These rates are comparable to those found in the guts of herbivorous reptiles and mammals. To determine the contribution of metabolic pathways to the fate of acetate, rates of sulfate reduction and methanogenesis were measured in the fore-, mid-, and hindgut sections of the three fish species. Both rates increased from the distal to proximal end of the hindgut, where sulfate reduction accounted for only a small proportion (<5%) of acetate methyl group transformed to CO(2), and exceeded methanogenesis from acetate by >50-fold. When gut size was taken into account, acetate uptake from the hindgut of the fish species, determined on a millimole per day per kilogram of body weight basis, was 70 (K. sydneyanus), 18 (O. pullus), and 10 (A. arctidens).
Glomerular injury and proteinuria in rats after intrarenal injection of cobra venom factor. Evidence for the role of neutrophil-derived oxygen free radicals. (1986)

The purpose of these studies was to determine how intravascular complement activation could lead to glomerular injury. Cobra venom factor (CVF) infused into the renal artery of rats resulted in increased excretion of protein in urine, which was maximal over the first 24 hours (51.2 +/- 6.0 mg/24 hours in CVF versus 14.1 +/- 0.9 mg/24 hours in saline-treated animals; P less than 0.001). Depletion of circulating neutrophils with anti-neutrophil serum significantly reduced the CVF-induced proteinuria in the first 24 hours (neutrophil depleted rats 22.7 +/- 2.8 mg/24 hours versus 63.4 +/- 9.9 mg/24 hours in neutrophil intact rats; P less than 0.005). Morphologic abnormalities (which were quantitated morphometrically) included accumulation of neutrophils in glomerular capillary loops, blebbing of endothelial cells, and epithelial cell foot process fusion. The increased protein excretion was reduced by 70% by simultaneous administration of catalase (23 +/- 4.3 mg/24 hours in CVF plus catalase versus 52.1 +/- 10 mg/24 hours in CVF alone; P less than 0.05). Catalase reduced glomerular endothelial cell blebbing and epithelial cell foot process fusion but not neutrophil accumulation in glomeruli as assessed by morphometry. In similar experiments superoxide dismutase, dimethyl sulfoxide, and deferoxamine did not prevent CVF-induced proteinuria. These studies, therefore, suggest that intravascular activation of complement in the rat causes glomerular injury and proteinuria which is dependent on neutrophils and upon the generation of hydrogen peroxide and/or its metabolites.
Manual In-line Stabilization Increases Pressures Applied by the Laryngoscope Blade during Direct Laryngoscopy and Orotracheal Intubation (2008)

Pressures applied to airway tissues by the laryngoscope blade are secondarily transmitted to the cervical spine and result in cranio-cervical motion. In the presence of cervical instability, impaired glottic visualization and secondary increases in pressure application with MILS have the potential to increase pathologic cranio-cervical motion.
Femtosecond laser-induced periodic surface structure on diamond film (2003)

We report the laser-induced periodic surface structure (LIPSS) with periodicity about a quarter of the laser wavelength on unpolished diamond film treated by a P-polarized femtosecond laser. The short period LIPSS is parallel to the laser polarization and independent on the incidence angle. The LIPSS perpendicular to the laser polarization with periodicity shorter than a third of the laser wavelength slightly dependent on the incidence angle is also observed as well as the LIPSS perpendicular to the laser polarization with periodicity dependent on the incidence angle. The results are explained by interference of the incident laser and surface scattered wave related to the excited electrons during laser interactions with diamond, being in excellent agreement with a previously developed theory.
Serologic follow-up of a repopulated swine herd after an outbreak of proliferative hemorrhagic enteropathy. (2002)

Lawsonia intracellularis is an obligate intracellular organism that causes porcine proliferative enteropathy, a widespread infectious disease. Very little is known about the immune response and the epidemiologic features of the disease in the field. The aims of this study were to evaluate the duration and titers of antibody specific for L. intracellularis in gilts after an outbreak of proliferative hemorrhagic enteropathy (PHE), to evaluate maternal antibodies in piglets, and to evaluate seroconversion and fecal shedding in growing-finishing pigs. Thirty-six gilts in a herd that had recently experienced an outbreak of PHE, including 13 that had recovered, were bled 3 wk after the beginning of the outbreak and then every 3 wk until they became seronegative in 2 consecutive tests. Fourteen piglets from 5 gilts seropositive at farrowing and 5 piglets from 2 sows that remained seronegative were bled once or twice at the farrowing house and then every 3 wk until they reached market age. Fecal samples from these pigs were tested by polymerase chain reaction at 7 wk of age and then on the days of blood collection. After the PHE outbreak, the gilts had high serum antibody levels; the levels decreased over time, but antibody was still detectable for up to 3 mo in some animals. Four piglets from sows that were seropositive at farrowing had detectable passive antibodies up to 5 wk of age. Some nursery pigs started shedding L. intracellularis around 7 wk of age; peak shedding was observed between 13 and 16 wk. Antibody was not detected until 16 wk of age and was more often detected between 19 and 22 wk.
Timing, energy, and thermal performance of three-dimensional integrated circuits (2004)

We examine the performance of custom circuits in an emerging technology known as three-dimensional integration. By combining multiple device layers with a high-density inter-layer interconnect, 3D integration of a given circuit is expected to provide better timing and energy performance relative to a single-wafer implementation of the same circuit. In this paper, we show that by using our performance-driven design tool for 3D ICs, the interconnect energy dissipation of standard-cell circuits can be reduced by 24% to 42% using two to five device layers respectively. Similarly, the interconnect energy-delay product can be reduced by 30% to 50%.At the same time, thermal performance in 3D ICs is expected to be a critical issue. By incorporating thermal management and analysis into our placement tool, we may investigate the thermal scalability of 3D integration. We find that the thermal performance actually can be improved with the use of a modest number of additional device layers. Also, we show that the absolute die temperature can be controlled through the use of extra silicon.
Neurolipidomics: challenges and developments (2006)

The field of lipidomics is one of the most rapidly expanding areas of systems biology research. Considering the uniqueness and complexity of the lipidome in the nervous system (i.e., neurolipidome), neurolipidomics remains quite challenging but exciting. With the recent development of mass spectrometry (MS)-based lipidomics, particularly the rapid improvement of multi-dimensional MS-based shotgun lipidomics, much progress has been made in neurolipidomics. As the accelerated development of future technologies enables lipidomics penetrance into lower and lower abundance regions of mass contents of individual lipid molecular species, it can be anticipated that many biochemical mechanisms underlying lipid metabolism critical to neuronal disease states will be increasingly uncovered. Through exploiting the information content inherent in the complexity of neuronal lipid composition and kinetic turnover which can be revealed by neurolipidomics, substantial insights into neuronal plasticity and gene function can be gathered. Through neurolipidomics, the markers for these neuronal diseases which identify pathological alterations and are diagnostic of disease onset, progression or severity may potentially be discovered. Accordingly, with neurolipidomics, our understanding of the complexities of the nervous system will be undoubtedly accelerated, as many mysteries are resolved.
Quantitative autoradiographic measurement of regional myocardial substrate uptake in hypertensive rats (1984)

Quantitative double tracer autoradiographic technique permits to study the distribution of two tracers in the same section of small animals with high spatial resolution. The authors have applied this technique to measure regional myocardial perfusion and substrate uptake in hypertensive rats. To compate regional myocardial uptake of fatty acid and glucose analogs with regional myocardial perfusion, three different double tracer experiments were conducted utilizing the difference of physical half-lives as follows: I) (C-14)3-methyl-heptadecanoic acid (BMHDA) and T1-201 (TL), II) (C-14)2-deoxy-D-glucose (DG) and TL, III) (F-18)2-fluoro-2-deoxy-D-glucose (FDG) and BMHDA. DG (or FDG), BMHDA and TL were administered to rats 45 min, 15 min and 5 min prior to sacrifice, respectively. The autoradiograms were digitized and quantitated using a videodensitometry film analysis system. Myocardial perfusion as indicated by TL distribution was homogeneous in both normotensive and hypertensive rats. However, hypertensive myocardium showed decreased uptake of BMHDA in the endocardial and septal regions, where DG showed markedly increased uptake. Double tracer experiment with BMHDA and FDG clearly demonstrated this complementary relation in the same section. These data indicate that hypertensive myocardium was abnormal substrate utilization although regional myocardial perfusion is not impaired. Moreover, (C-11)BMHDA and (F-18)FDG may be used to measure regional myocardialmore » substrate utilization in humans with positron emission tomography.« less
Self‐assembled pearling structure of long duplex DNA with histone H1 (2001)

We report that complexes of giant DNA molecules with histone H1 proteins form a pearl necklace-like structure when the complexes are prepared by natural dilution from a high-salt solution (2 M NaCl) to a low-salt solution (0.2 M and 50 mM NaCl). We performed real-time observations on the conformational changes of individual T4 phage DNA (166 kb) molecules in bulk solution by fluorescence microscopy. To identify H1-binding regions on individual DNA molecules, we also performed immunofluorescence microscopic observations on the DNA-H1 complex spread on a glass surface. It was found that histone H1 binds DNA in a highly co-operative manner and is accompanied by local folding of the DNA. On the basis of the experimental observations and a theoretical simulation, we propose a self-assembling mechanism for the pearling structure.
<title>Silicon nitride biaxial pointing mirrors with stiffening ribs</title> (2001)

Gold-coated silicon nitride mirrors designed for two orthogonal rotations were fabricated. The devices were patterned out of nitride using surface micromachining techniques, and then released by a sacrificial oxide etch and bulk etching the silicon substrate. Vertical nitride ribs were used to stiffen the members and reduce the curvature of the mirrored surfaces due to internal stress in the nitride and the metal layer. This was accomplished by initially etching the silicon substrate to form a mold that was filled with nitride to create a stiffening lattice-work to support the mirrored section. Mirror diameters ranging from 100 mm to 500 mm have been fabricated, with electrostatic actuation used to achieve over four degrees of tilt for each axis.
An apparatus for applying strong longitudinal magnetic fields to clinical photon and electron beams (2001)

Monte Carlo studies have recently renewed interest in the use of the effect of strong transverse and longitudinal magnetic fields to manipulate the dose characteristics of clinical photon and electron beams. A 3.5 T superconducting solenoidal magnet was used to evaluate the effect of a longitudinal field on both photon and electron beams. This note describes the apparatus and demonstrates some of the effects on the beam trajectory and dose distributions for measurements in a homogeneous phantom. The effects were studied using film in air and in phantoms which fit in the magnet bore. The magnetic field focused and collimated the electron beams. The converging, non-uniform field confined the beam and caused it to converge with increasing depth in the phantom. Due to the field's collecting and focusing effect, the beam flux density increased, leading to increased dose deposition near the magnetic axis, especially near the surface of the phantom. This study illustrates some benefits and challenges associated with the use of non-uniform longitudinal magnetic fields in conjunction with clinical electron and photon beams.
Tunable diode laser absorption spectroscopy for oxygen detection (2012)

The evolution of diode laser sources for optical communications during the last years led to commercial availability of devices which are suitable for gas absorption spectroscopy in the near and mid infrared. In this work it is shown how the traditional limits of Tunable Diode Laser Absorption Spectroscopy are addressed with digital signal processing techniques and careful optical design towards the realization of gas sensing instruments with the stability, robustness and reliability that are required in an industrial environment. Being one of the most challenging gases to be sensed with this technique, oxygen was considered under many measurement aspects such as: • Non invasive monitoring; • Gas in scattering media sensing; • Sensing with back-scattering targets; • Pressure measurement techniques for weak absorption signals; • Time resolved, dynamic sensing; • Temperature measurement through absorption spectroscopy. Many of these aspects were considered together, leading to the developement of instruments tailored for real life industrial applications such as: • Oxygen sensing in partially transparent containers such as wine or soft drink bottles; • Monitoring of double glazing insulating glass gas filling machines; • Oxygen sensing in containers with backscattering targets such as food packagings. Other applications for the technique and experiments involving Gas in Scattering Media Absorption Spectroscopy were explored during a 6 months period at the Lunds Universitet - Lunds Tekniska Högskola - Atomfysik (Sweden) under the supervision of Prof. S. Svanberg: • Gas probing into porous fruit samples; • Gas sensing inside the human body as a medical diagnosis technique; • Oxygen measurement in fully scattering food containers; • Multi-line absorption spectroscopy as a temperature measurement.
Thyroid hormone regulation of prohormone convertase 1 (PC1): regional expression in rat brain and in vitro characterization of negative thyroid hormone response elements (2004)

Most pro-neuropeptides are processed by the prohormone convertases, PC1 and PC2. We previously reported that changes in thyroid status altered anterior pituitary PC1 mRNA and this regulation was due to triiodothyronine (T(3))-dependent interaction of thyroid hormone receptor (TR) with negative thyroid hormone response elements (nTREs) contained in a large region of the human PC1 promoter. In this study, we demonstrated that hypothyroidism stimulated, while hyperthyroidism suppressed, PC1 mRNA levels in rat hypothalamus and cerebral cortex, but not in hippocampus. In situ hybridization was used to confirm real-time PCR changes and localize the regulation within the hypothalamus and cortex. Using a human PC1 (hPC1) promoter construct (with and without deletions in two regions that each contain a negative TRE) transiently transfected into GH3 cells, we found that T(3) negatively regulated hPC1 promoter activity, and this regulation required both of these two regions. Electrophoretic mobility shift assays (EMSAs) using purified thyroid hormone receptor alpha1 (TRalpha1) and retinoid X receptor beta (RXRbeta) proteins demonstrated that RXR and TRalpha both bound the PC1 promoter. Addition of TRalpha1/RXRbeta to the wild-type PC1 probe demonstrated binding as both homodimers and a heterodimer. EMSAs with oligonucleotides containing deletion mutations of the putative nTREs demonstrated that the proximal nTRE binds more strongly to TR and RXR than the distal nTRE, but that both regions exhibit specific binding. We conclude that there are multiple novel TRE-like sequences in the hPC1 promoter and that these regions act in a unique manner to facilitate the negative effect of thyroid hormone on PC1.