gebhart/patrepeval huggingface dataset:
The new frontier in muscular dystrophy research: booster genes (2003)

More than 30 different forms of muscular dystrophy (MD) have been molecularly characterized and can be diagnosed, but progress toward treatment has been slow. Gene replacement therapy has met with great difficulty because of the large size of the defective genes and because of difficulties in delivering a gene to all muscle groups. Cell replacement therapy has also been difficult to realize. Will it even be possible to design specific therapy protocols for all MDs? Or is a more realistic goal to treat some of the secondary manifestations that are common to several forms of MD, such as membrane instability, necrosis, and inflammation, and to promote regeneration? As reviewed here, enhanced expression of a range of proteins provides a boost for degenerating dystrophic muscle in mouse models. Expression of a mini-agrin promotes basement membrane formation instead of laminin alpha2; integrin alpha7, GalNac transferase, and ADAM12 promote cell adhesion and muscle stability in the absence of dystrophin; calpastatin prevents muscle necrosis; and nitric oxide synthase prevents inflammation. ADAM12, IGF-I, and myostatin blockade promote regeneration and reduce fibrosis. One can envision numerous other candidate booster genes which encode proteins that promote survival and/or regeneration of the compromised muscle or proteins that affect post-translational modifications of critical proteins. Finally, fibrosis, which is the curse of many human diseases, may also be attacked. Once the mechanisms of the boosters are better understood, drugs may be developed to provide the boost to muscle. Some of the experiences in models of muscular dystrophy may inspire new approaches in other genetic degenerative diseases as well.
Fatal familial insomnia: a new Austrian family (1999)

We present clinical, pathological and molecular features of the first Austrian family with fatal familial insomnia. Detailed clinical data are available in five patients and autopsy in four patients. Age at onset of disease ranged between 20 and 60 years, and disease duration between 8 and 20 months. Severe loss of weight was an early symptom in all five patients. Four patients developed insomnia and/or autonomic dysfunction, and all five patients developed motor abnormalities. Analysis of the prion protein (PrP) gene revealed the codon 178 point mutation and methionine homozygosity at position 129. In all brains, neuropathology showed widespread cortical astrogliosis, widespread brainstem nuclei and tract degeneration, and olivary 'pseudohypertrophy' with vacuolated neurons, in addition to neuropathological features described previously, such as thalamic and olivary degeneration. Western blotting of one brain and immunocytochemistry in four brains revealed quantitative and regional dissociation between PrP(res)(the protease resistant form of PrP) deposition and histopathology. In the cerebellar cortex of one patient, PrP(res) deposits were prominent in the molecular layer and displayed a peculiar patchy and strip-like pattern with perpendicular orientation to the surface. In another patient, a single vacuolated neuron in the inferior olivary nuclei contained prominent intravacuolar granular PrP(res) deposits, resembling changes of brainstem neurons in bovine spongiform encephalopathy.
Colocalization of eNOS and the Catalytic Subunit of PKA in Endothelial Cell Junctions: A Clue for Regulated NO Production (2004)

Localization and coordinate phosphorylation/dephosphorylation of endothelial nitric oxide synthase (eNOS) are critical determinants for the basal and stimulated production of nitric oxide. Several phosphorylation sites in eNOS have been identified as targets of the cAMP-dependent protein kinase A (PKA). Basal eNOS activity is also regulated by interaction with caveolin-1, the major coat protein of caveolae. In the present study we have examined in rat aorta endothelium the subcellular steady-state distribution of eNOS, the catalytic subunit of PKA (PKA-c), and caveolin-1. Basal eNOS expression was found in two distinct locations, the endothelial cell surface and the Golgi complex. Cell surface eNOS was equally distributed over caveolar and non-caveolar membranes but was 2.5-fold enriched on luminal lamellipodia located at endothelial cell contacts. PKA-c colocalized with eNOS in the lamellipodia, whereas caveolin-1 was absent from these membrane domains. PKA-c was also found associated with cell surface caveolae and with tubulovesicular membranes of Golgi complex and endosomes. The topological proximity of eNOS with the catalytic subunit of PKA in restricted intracellular locations may provide mechanisms for differential PKA-mediated eNOS regulation.
The Effect of N Acetyl D-Galactosamine and Other Sugars on the Mitogenic Activity and Attachment of PHA to Tonsil Cells (1968)

Abstract The effect of several simple sugars on the mitogenic and binding properties of PHA has been investigated. N acetyl D-galactosamine in concentration of 3-10 mg./ml. inhibited the incorporation of uridine-2-C14 and thymidine-2-C14 into the acid precipitable fraction of human tonsil cells stimulated by phytohemagglutinin. No inhibition, or a significantly lesser degree of inhibition was observed with N acetyl D-glucosamine or glucose. The binding of I131 labeled PHA to cells was decreased by NAGAL or EDTA. The EDTA inhibition, unlike the NAGAL inhibition was overcome by magnesium.
A Portable Instrument for the Location of Subcutaneous Fish-Tags (1954)

A Portable Instrument for the Location of Subcutaneous Fish-Tags Get access W. H. Moore, W. H. Moore Freshwater Biological AssociationAmbleside, Westmorland Search for other works by this author on: Oxford Academic Google Scholar C. H. Mortimer C. H. Mortimer Freshwater Biological AssociationAmbleside, Westmorland Search for other works by this author on: Oxford Academic Google Scholar ICES Journal of Marine Science, Volume 20, Issue 1, July 1954, Pages 83–86, https://doi.org/10.1093/icesjms/20.1.83 Published: 01 July 1954
Energy interactions between multicast and content distribution within data communication networks (2015)

While commercial data center energy use has shown remarkable progress, other ways to reduce the energy used on behalf of Internet devices within the global telecommunications network have not yet been used to their full benefit; one pertinent example is exploiting the economies of scale that exist due to the remarkable overlap between what individuals are using the Internet to do, creating some definite patterns and commonalities among what individuals watch or download at any given moment, thereby giving a high degree of collective redundancy to Internet transmissions. Redundant transmissions are wasted bits; by some measures, fully capitalizing on this wasted redundancy could reduce the number of transmissions at individual network nodes by as much as two orders of magnitude. Missing from prior analyses is an assessment of the degree to which two current common methods of reducing network activity related to popular content, namely multicast transmissions and a set of mechanisms for wider content distribution such as in-network caching, proxy or edge servers, complement and interfere with one another when the goal is improving the energy efficiency of the ICT as a whole. It can be seen that both categories of methods reduce the number of transmissions in the network; yet dispersing network activity to a wider set of sources also has the unintentional effect of reducing the exploitable redundancy for multicast. This paper defines and characterizes the tradeoff of multicast energy gain for energy gain via shorter transmission paths and the conditions within which overall energy savings occurs.
Dynamics of vibrationally mediated photodissociation of CH3CFCl2 (2001)

The ∼235 nm photodissociation of CH3CFCl2 pre-excited to three, four, and five quanta of C–H methyl stretches was studied to investigate the effect of internal parent excitation on the dynamics of two- and three-body photofragmentation. The ∼235 nm photons also tagged spin-orbit ground Cl 2P3/2 [Cl] and excited Cl 2P1/2 [Cl*] state photofragments, via (2+1) resonantly enhanced multiphoton ionization in a time-of-flight mass spectrometer. Monitoring the shapes of Cl35 and Cl*35 time-of-arrival profiles revealed their energies and angular distributions and showed broad and unstructured fragment kinetic energy distributions. Although a significant amount (∼50%) of the available energy is transferred into internal energy of the CH3CFCl fragment, the spatial Cl distribution is characterized by a nonvanishing anisotropy parameter, β, which indicates at a fast dissociation of the parent molecule along the C–Cl dissociation coordinate. Moreover, β for Cl changes from a slightly positive value to a negative value, while that for Cl* increases when the pre-excitation is increased from three to five quanta of C–H methyl stretches. This is attributed to the promotion of one of the nonbonding electrons located on the Cl atoms to the σ* antibonding C–Cl orbital and involvement of several upper states with different symmetry properties.
Signal Recognition Particle (SRP)-mediated Targeting and Sec-dependent Translocation of an Extracellular Escherichia coli Protein (2003)

Hemoglobin protease (Hbp) is a hemoglobin-degrading protein that is secreted by a human pathogenic Escherichia coli strain via the autotransporter mechanism. Little is known about the earliest steps in autotransporter secretion, i.e. the targeting to and translocation across the inner membrane. Here, we present evidence that Hbp interacts with the signal recognition particle (SRP) and the Sec-translocon early during biogenesis. Furthermore, Hbp requires a functional SRP targeting pathway and Sec-translocon for optimal translocation across the inner membrane. SecB is not required for targeting of Hbp but can compensate to some extent for the lack of SRP. Hbp is synthesized with an unusually long signal peptide that is remarkably conserved among a subset of autotransporters. We propose that these autotransporters preferentially use the co-translational SRP/Sec route to avoid adverse effects of the exposure of their mature domains in the cytoplasm.
The capabilities of migration and differentiation of female primordial germ cells after transferring to male embryos (2001)

조류의 경우에는 포유류와 달리 수정란의 성별이 암컷에 의하여 결정된다. 수컷은 동일접합체로 ZZ 염색체를, 암컷의 경우에는 이형접합체로 ZW 염색체를 갖기 때문이다. 현재까지 조류에 있어서 염색체 분석 등에 의한 암ㆍ수의 세포 유전학적인 특성은 많은 연구가 되어 있으나, 배발달 초기의 원시생식세포 등에 대해서는 많은 연구가 진행되어 있지 않다. 따라서 본 연구는 암컷의 원시생식세포를 분리하여 숫컷의 초기 배자에 주입함으로써 수용체 배자의 원시생식기내로 이동이 가능한지를 검증하였으며, 또한 수컷의 원시생식기내로의 이동 후 정상적으로 분열 및 분화가 가능한지를 초기 배발달 과정에서 확인하였다. 본 연구 결과, 암컷의 원시생식세포는 수컷의 수용체 배자에 재주입시 정상적인 원시생식기내로의 이동 능력을 보여주었으며, 분열ㆍ분화함을 알 수있었다.
Absorption spectra of ketones. Part II. The configuration of some bromo-derivatives of 6-oxocholestanyl acetate. Absorption spectra of α-ketols (1955)

R. C. Cookson and S. H. Dandegaonker, J. Chem. Soc., 1955, 352 DOI: 10.1039/JR9550000352
Bio-Inspired Kinetic Curved-Line Folding for Architectural Applications (2016)

This paper discusses the development of a bio-inspired compliant mechanism for architectural applications and explains the methodology of investigating movements found in nature. This includes the investigation of biological compliant mechanisms, abstraction, and technical applications using computational tools such as finite element analysis (FEA). To demonstrate the possibilities for building envelopes of complex geometries, procedures are presented to translate and alter the disclosed principles to be applicable to complex architectural geometries. The development of the kinetic faÃ§ade shading device flectofold, based on the biological role-model Aldrovanda vesiculosa, is used to demonstrate the process. The following paper shows results of FEA simulations of kinetic curved-line folding mechanisms with pneumatic actuation and provides information about the relationship between varying geometric properties (e.g. curved-line fold radii) and multiple performance metrics, such as required actuation force and structural stability.
Fukuoka criteria accurately predict risk for adverse outcomes during follow-up of pancreatic cysts presumed to be intraductal papillary mucinous neoplasms (2016)

Fukuoka guidelines accurately stratify PCL-IPMNs for PC risk, with FN cysts having lowest and HR cysts having greatest risk. After 6-month follow-up, WF and FN cysts had a low 5-year PC risk. Surveillance strategies should be tailored appropriately.
A bipartite, differential I/sub DDQ/ testable static RAM design (2002)

I/sub DDQ/ (Defect Detection by Quiescent power supply current measurement), or current testing, has emerged in the last few years as an effective technique for detecting certain classes of faults in high-density ICs. In this paper, a testable design that enhances the I/sub DDQ/ testability of static random access memories (SRAMs) for off-line testing as proposed. To achieve high accuracy and a test speed approaching the system operational speed, the memory is partitioned for comparison of I/sub DDQ/ values. Parallel write/read operations are used to activate possible faults, while quiescent power supply currents from two blocks are compared.
Operational and performance report on ANIPET, a versatile PET scanner for laboratory animals (2002)

Presents the properties and the performance of the ANIPET scanner which has been designed for imaging positron-emitting tracers in small laboratory animals. It has two planar BGO-PS-PMT detectors with a useful FOV of 59/spl times/54 mm. These can be separated by 75-200 mm to optimize detection efficiency with different sized laboratory animals ANIPET has the capacity to scan in 3D mode for brain images or 2D mode for whole body scanning. The spatial resolution at the centre of the field of view is 2.8 mm FWHM for 2D mode scans and 3.2 mm in the 3D mode. The coincidence timing resolution is 10 ns FWHM. The system efficiency varies from 1% to 0.1% as the detector separation increased from 75 to 200 mm and the energy discriminator window is increased from 150 to 450 keV.
Microstructure, Texture and Mechanical Properties of AZ31 Mg Alloy Produced by Equal Channel Angular Extrusion (2008)

AbstractAbstractEqual channel angular pressing (ECAP) was carried out on samples machined from extruded AZ31 Mg alloy bars at 200, 250 and 300 °C using routes A and C for up to 8 passes. After one ECAP pass, a preferred orientation with the basal planes inclined at 45° to the ECAP axis is observed. For route A, as more passes are employed, the original fiber texture tends towards a rolling texture. By contrast, for route C, repeating the ECAP steps does not change the texture, which retains the 45° preferential orientation. Using a path with shearing in more directions, such as route C, resulted in the flow curve peak being less sharp (i.e. broader) in subsequent testing than when route A was employed. The strength of the extruded product increases with the ECAP temperature. This appears to be due to the coarser grain size of the 300 °C product and indicates that the effect of grain coarsening is greater than that of texture.Equal channel angular pressing (ECAP) was carried out on samples machined from extruded AZ31 Mg alloy bars at 200, 250 and 300 °C using routes A and C for up to 8 passes. After one ECAP pass, a preferred orientation with the basal planes inclined at 45° to the ECAP axis is observed. For route A, as more passes are employed, the original fiber texture tends towards a rolling texture. By contrast, for route C, repeating the ECAP steps does not change the texture, which retains the 45° preferential orientation. Using a path with shearing in more directions, such as route C, resulted in the flow curve peak being less sharp (i.e. broader) in subsequent testing than when route A was employed. The strength of the extruded product increases with the ECAP temperature. This appears to be due to the coarser grain size of the 300 °C product and indicates that the effect of grain coarsening is greater than that of texture.On a effectué le formage coudé à aires égales (ECAP) d'échantillons usinés à partir de barres extrudées d'alliage de Mg AZ31 à 200, 250 et 300 °C en utilisant les voies A et C, jusqu'à 8 passes. Après une passe d'ECAP, on observe une orientation préférée avec les plans de base inclinés à 45° par rapport à l'axe d'ECAP. Pour la route A, à mesure qu'on augmente les passes, la texture de la fibre originale tend vers une texture de laminage. En contraste, pour la route C, la répétition des étapes de l'ECAP ne change pas la texture, laquelle retient l'orientation préférentielle à 45°. L'utilisation d'une voie avec cisaillement à plusieurs directions, comme la route C, donnait un pic de la courbe d'écoulement moins défini (c'est-à-dire plus large) lors de l'évaluation subséquente que lorsque qu'on utilisait la route A. La résistance du produit extrudé augmente avec la température de l'ECAP. Ceci est attribuable au grain plus grossier du produit à 300 °C et indique que l'effet de grossissement du grain est plus important que celui de la texture.
Ecological and erosion protection functions of Chesapeake Bay living shorelines : Comprehensive Monitoring of Ecological and Erosion Protection Functions of Chesapeake Bay Living Shorelines (CMLS), Phase II (2019)

Armoring shorelines to prevent erosion, improve access, and accommodate individual landscaping interests can result in fragmentation or loss of habitats, reduction in capacity to moderate pollutant loads delivered to coastal waters, reduction in nekton and macrobenthic integrity (Bilkovic et al. 2005, King et al. 2005, Seitz et al. 2006, Bilkovic et al. 2006, Bilkovic & Roggero 2008), increases in invasive species, such as Phragmites australis (Chambers et al. 1999, King et al. 2007), and disturbance of sediment budgets sustaining adjacent properties. As an alternative to traditional armoring of shorelines, shoreline protection techniques incorporating natural elements from the system are increasingly promoted as not only less harmful to the system, but also beneficial due to their ability to provide or enhance coastal ecosystem services. Yet, there has been limited scientific investigation of ecological benefits and impacts associated with the implementation of natural shoreline protection designs (Burke et al. 2005, Davis et al. 2006).
Pushbroom-Frame Imagery Co-Registration (2012)

More and more littoral surveys are conducted with aerial platform sensor suites that include a hyperspectral pushbroom sensor and a frame camera. However, in some cases, data from auxiliary sensors may contain errors or are not available. For many research groups, a high-accuracy registration of the multi-sensor data relative to each other is essential, while absolute geo-location of each individual measurement is not. A co-registration procedure was developed for pseudo ortho-rectification of the hyperspectral imagery and to remove spatial distortions caused by the aircraft's trajectory during the survey based on a flat-earth assumption. This image-processing approach utilizes the aerial frame imagery as a reference. Each hyperspectral scan line is co-registered into the frame imagery coordinate system. The performance of the procedure was evaluated using hyperspectral imagery collected over northern New Hampshire and southern Maine. The evaluation results showed that the procedure is robust enough to pseudo ortho-rectify hyperspectral imagery over coastal areas and to remove significant spatial distortions.
ChemInform Abstract: A Synthesis of Protected Aminoalkyl Epoxides from α‐Amino Acids. (1987)

Abstract The reduction of the N‐protected amino esters (I) produces the amino aldehydes (II) which are converted to the amino alkenes (IV) by Wittig reaction.
Monte Carlo simulation for depth encoding multicrystal detector for PET (2002)

At the EEEE MIC 1999, we presented a Monte Carlo simulator made for designing a depth encoding multicrystal detector and reported some simulation results. While the basic performance of the simulator was validated from the comparison between the simulator outputs and experimental results using a prototype detector, many factors to be implemented were still remaining for simulating real phenomena inside the detector. This time, we improved the simulator by including Compton scattering, more accurate migration of optical photons at every interface of materials, and so on. Using this simulator, discrimination characteristics were investigated with respect to thickness of scintillator block, refractive index of inter-stage material, reflectance of optical reflector and surface roughness of scintillator. From these investigations, useful information for detector design was obtained.
Total synthesis of carboprostacyclin, a stable and biologically active analogue of prostacyclin (PGI2) (1978)

K. C. Nicolaou, W. J. Sipio, R. L. Magolda, S. Seitz and W. E. Barnette, J. Chem. Soc., Chem. Commun., 1978, 1067 DOI: 10.1039/C39780001067
ChemInform Abstract: KONFORMATIONSANALYSE VON AZETIDINEN, BEWEISE FUER EINEN NICHT‐PLANAREN RING (1972)

Abstract Die Konformationen der Azetidine (I) und (II) werden über NMR‐Spektren untersucht.
Anomalous temperature resistance characteristic of highly acceptor doped BaTiO<sub>3</sub>PTC resistors (1974)

Abstract The resistance increase above the Curie temperature in n-doped BaTiO3 ceramics is due to blocking layers. These layers are due to acceptor states at grain boundaries. When increasing the acceptor concentration an additional resistance maximum occurs below the Curie temperature, which finally prevails the original resistance increase. This maximum can be explained by the normal theory if the nonlinearity of the dielectric properties inside the blocking layers is taken into account.
Mechanism of magnetostructural transformation in multifunctional Mn3GaC (2017)

Mn3GaC undergoes a ferromagnetic to antiferromagnetic, volume discontinuous cubic-cubic phase transition as a function of temperature, pressure, and magnetic field. Through a series of temperature dependent x-ray absorption fine structure spectroscopy experiments at the Mn K and Ga K edge, it is shown that the first order magnetic transformation in Mn3GaC is entirely due to distortions in the Mn sub-lattice and with a very little role for Mn-C interactions. The distortion in the Mn sub-lattice results in long and short Mn-Mn bonds with the longer Mn-Mn bonds favoring ferromagnetic interactions and the shorter Mn-Mn bonds favoring antiferromagnetic interactions. At the first order transition, the shorter Mn-Mn bonds exhibit an abrupt decrease in their length resulting in an antiferromagnetic ground state and a strained lattice.