gebhart/patrepeval huggingface dataset:
AAV-Mediated Gene Delivery in a Feline Model of Sandhoff Disease Corrects Lysosomal Storage in the Central Nervous System (2015)

Sandhoff disease (SD) is an autosomal recessive neurodegenerative disease caused by a mutation in the gene for the β-subunit of β-N-acetylhexosaminidase (Hex), resulting in the inability to catabolize ganglioside GM2 within the lysosomes. SD presents with an accumulation of GM2 and its asialo derivative GA2, primarily in the central nervous system. Myelin-enriched glycolipids, cerebrosides and sulfatides, are also decreased in SD corresponding with dysmyelination. At present, no treatment exists for SD. Previous studies have shown the therapeutic benefit of adeno-associated virus (AAV) vector-mediated gene therapy in the treatment of SD in murine and feline models. In this study, we treated presymptomatic SD cats with AAVrh8 vectors expressing feline Hex in the thalamus combined with intracerebroventricular (Thal/ICV) injections. Treated animals showed clearly improved neurologic function and quality of life, manifested in part by prevention or attenuation of whole-body tremors characteristic of untreated animals. Hex activity was significantly elevated, whereas storage of GM2 and GA2 was significantly decreased in tissue samples taken from the cortex, cerebellum, thalamus, and cervical spinal cord. Treatment also increased levels of myelin-enriched cerebrosides and sulfatides in the cortex and thalamus. This study demonstrates the therapeutic potential of AAV for feline SD and suggests a similar potential for human SD patients.
The Firegoose: two-way integration of diverse data from different bioinformatics web resources with desktop applications (2007)

The Firegoose incorporates Mozilla Firefox into the Gaggle environment and enables interactive sharing of data between diverse web resources and desktop software tools without maintaining local copies. Additional web sites can be incorporated easily into the framework using the scripting platform of the Firefox browser. Performing data integration in the browser allows the excellent search and navigation capabilities of the browser to be used in combination with powerful desktop tools.
Real-Time Control of Focused Ultrasound Heating Based on Rapid MR Thermometry (1999)

Automatic control of FUS power level was demonstrated ex vivo at three target temperature levels (increase of 5 degrees C, 10 degrees C, and 30 degrees C above room temperature) during 30-minute hyperthermic periods. Preliminary in vivo results on rat leg muscle confirm that necrosis estimate, calculated on-line during FUS sonication, allows prediction of tissue damage. CONCLUSIONS. The feasibility of fully automatic FUS control based on MRI thermometry has been demonstrated.
Criteria for Early Identification of Aceruloplasminemia (2011)

A 52-year-old Japanese woman being treated for type 1 diabetes showed forgetfulness and microcytic anemia with a high serum ferritin concentration. Serum and brain radiological examinations revealed aceruloplasminemia, which was confirmed by genetic testing. Aceruloplasminemia is characterized by the triad of retinal degeneration, diabetes mellitus, and adult-onset disorder of the extrapyramidal system. Though physicians should treat such patients earlier, it is difficult to diagnose the disease before the presentation of neurological symptoms. Despite the presence of microcytic anemia, aceruloplasminemia patients usually have a high serum ferritin concentration due to the complete absence of ceruloplasmin ferroxidase activity. Thus, physicians should consider aceruloplasminemia when diabetic patients present with microcytic anemia and a high serum ferritin concentration.
Magnetic Resonance Imaging of Human Embryonic Stem Cells (2009)

Magnetic resonance imaging (MRI) may emerge as an ideal non-invasive imaging modality to monitor stem cell therapy in the failing heart. This imaging modality generates any arbitrary tomographic view at high spatial and temporal resolution with exquisite intrinsic tissue contrast. This capability enables robust evaluation of both the cardiac anatomy and function. Traditionally, superparamagnetic iron oxide nanoparticle (SPIO) has been widely used for cellular MRI due to SPIO's ability to enhance sensitivity of MRI by inducing remarkable hypointense, negative signal, "blooming effect" on T2*-weighted MRI acquisition. Recently, manganese chloride (MnCl(2)) has been reported by our laboratory for its ability as a contrast agent to track biological activity of viable cells. Hyperintense, positive signals can be achieved from the Mn(2+)-labeled stem cells on T1-weighted MRI acquisition. Cytotoxicity is a potential drawback of Mn(2+) labeling of the cells. However, in our laboratory the labeling method has been optimized to minimize cytotoxic effects. This article describes two different magnetic labeling methods of human embryonic stem cells (hESC) using SPIO and MnCl(2).
Densely Packed Arrays of Ultra‐High‐Aspect‐Ratio Silicon Nanowires Fabricated using Block‐Copolymer Lithography and Metal‐Assisted Etching (2009)

Abstract Metal‐assisted etching is used in conjunction with block‐copolymer lithography to create ordered and densely‐packed arrays of high‐aspect‐ratio single‐crystal silicon nanowires with uniform crystallographic orientations. Nanowires with diameters and spacings down to 19 nm and 10 nm, respectively, are created as either continuous carpets or as carpets within trenches. Wires with aspect ratios up to 220 are fabricated, and capillary‐induced clustering of wires is eliminated through post‐etching critical point drying. The wires are single crystals with 〈100〉 axis directions. The distribution of wire diameters is narrow and closely follows the size distribution of the block copolymer, with a standard deviation of 3.12 nm for wires of mean diameters 22.06 nm. Wire arrays formed in carpets and in channels have hexagonal order with good fidelity to the block copolymer pattern. Fabrication of wires in topographic features demonstrates the ability to accurately control wire placement. Wire arrays made using this new process will have applications in the creation of arrays of photonic and sensing devices.
Succinimidyl Carbamate Derivatives from N‐Protected α‐Amino Acids and Dipeptides―Synthesis of Ureidopeptides and Oligourea/Peptide Hybrids (2007)

Abstract The preparation of succinimidyl (1‐{[(alkyloxy)carbonyl]amino}‐1‐X‐methyl)carbamates ( 4 ) and succinimidyl [1‐(acylamino)‐1‐X‐methyl] carbamates ( 5 ) from a variety of N ‐Boc‐, ‐Z‐ or ‐Fmoc‐protected α‐amino acids and dipeptides as well as the carbamate structural characterization are described. We have previously shown that succinimidyl carbamates 5 , derived from N ‐Boc dipeptides, can serve as direct precursors of 1,3,5‐triazepan‐2,6‐diones, a novel class of conformationally constrained dipeptide mimetics. Herein, we have evaluated the use of these building blocks for the synthesis of ureido‐peptides (in solution and on solid support), peptidyl hydantoins, oligoureas and some oligo(urea/amide) hybrids. Conformational investigations by NMR of ureidotripeptide 6i and pentamer 10 , consisting of alternating amides and ureas, suggested that folded conformations (i.e. a urea turn) characterized by a cis , trans ( E , Z ) geometry of the urea linkage are populated in both [D 5 ]pyridine and [D 6 ]DMSO solutions. (© Wiley‐VCH Verlag GmbH &amp; Co. KGaA, 69451 Weinheim, Germany, 2007)
Breakdown in the pretext tokamak (1981)

Data are presented on the application of ion cyclotron resonance RF power to preionization in tokamaks. We applied 0.3-3 kW at 12 MHz to hydrogen and obtained a visible discharge, but found no scaling of breakdown voltage with any parameter we were able to vary. A possible explanation for this, which implies that higher RF power would have been much more effective, is discussed. Finally, we present our investigation of the dV/dt dependence of breakdown voltage in PRETEXT, a phenomenon also seen in JFT-2. The breakdown is discussed in terms of the physics of Townsend discharges.
Levulinic esters. Alcohol protecting group applicable to some nucleosides (1975)

ADVERTISEMENT RETURN TO ISSUEPREVArticleNEXTLevulinic esters. Alcohol protecting group applicable to some nucleosidesAlfred Hassner, Glen Strand, Menachem Rubinstein, and Avram PatchornikCite this: J. Am. Chem. Soc. 1975, 97, 6, 1614–1615Publication Date (Print):March 1, 1975Publication History Published online1 May 2002Published inissue 1 March 1975https://pubs.acs.org/doi/10.1021/ja00839a077https://doi.org/10.1021/ja00839a077research-articleACS PublicationsRequest reuse permissionsArticle Views1663Altmetric-Citations70LEARN ABOUT THESE METRICSArticle Views are the COUNTER-compliant sum of full text article downloads since November 2008 (both PDF and HTML) across all institutions and individuals. These metrics are regularly updated to reflect usage leading up to the last few days.Citations are the number of other articles citing this article, calculated by Crossref and updated daily. Find more information about Crossref citation counts.The Altmetric Attention Score is a quantitative measure of the attention that a research article has received online. Clicking on the donut icon will load a page at altmetric.com with additional details about the score and the social media presence for the given article. Find more information on the Altmetric Attention Score and how the score is calculated. Share Add toView InAdd Full Text with ReferenceAdd Description ExportRISCitationCitation and abstractCitation and referencesMore Options Share onFacebookTwitterWechatLinked InRedditEmail Other access optionsGet e-Alertsclose Get e-Alerts
An Endophytic Nodulisporium sp. from Central America Producing Volatile Organic Compounds with Both Biological and Fuel Potential (2013)

A Nodulisporium sp. (Hypoxylon sp.) has been isolated as an endophyte of Thelypteris angustifolia (Broadleaf Leaf Maiden Fern) in a rainforest region of Central America. It has been identified both on the basis of its morphological characteristics and by scanning electron microscopy as well as ITS sequence analysis. The endophyte produces volatile organic compounds (VOCs) that have both fuel (mycodiesel) and use for biological control of plant disease. When grown on potato dextrose agar, the organism uniquely produces a series of ketones, including acetone; 2-pentanone; 3-hexanone, 4-methyl; 3-hexanone, 2,4- dimethyl; 2-hexanone, 4-methyl, and 5-hepten, 2-one and these account for about 25% of the total VOCs. The most abundant identified VOC was 1,8 cineole, which is commonly detected in this group of organisms. Other prominent VOCs produced by this endophyte include 1-butanol, 2- methyl, and phenylethanol alcohol. Moreover, of interest was the presence of cyclohexane, propyl, which is a common ingredient of diesel fuel. Furthermore, the VOCs of this isolate of Nodulisporium sp. were selectively active against a number of plant pathogens, and upon a 24 h exposure caused death to Phytophthora palmivora, Rhizoctonia solani, and Sclerotinia sclerotiorum and 100% inhibition to Phytophthora cinnamomi with only slight to no inhibition of the other pathogens that were tested. From this work, it is becoming increasingly apparent that each isolate of this endophytic Nodulisporium spp., including the Daldina sp. and Hypoxylon spp. teleomorphs, seems to produce its own unique set of VOCs.
AlGaN/GaN HEMTs-an overview of device operation and applications (2002)

Wide bandgap semiconductors are extremely attractive for the gamut of power electronics applications from power conditioning to microwave transmitters for communications and radar. Of the various materials and device technologies, the AlGaN/GaN high-electron mobility transistor seems the most promising. This paper attempts to present the status of the technology and the market with a view of highlighting both the progress and the remaining problems.
Pharmacological Inhibition of PKR in APPswePS1dE9 Mice Transiently Prevents Inflammation at 12 Months of Age but Increases A&beta;42 Levels in the Late Stages of the Alzheimer’s Disease (2012)

The double-stranded RNA-dependent protein kinase (PKR) is switched on by a wide range of stimuli, including the amyloid peptide. Then, PKR transmits signals to the translational machinery, apoptosis and inflammatory signaling pathways by interacting with some adapters. In virus-infected cells, PKR engages the nucleus factor κB (NF-κB) pathway. In many models of Alzheimer's disease (AD) and patients with AD, PKR was activated. Furthermore, there is strong evidence implicating the inflammatory process in the AD brain. However, the PKR involvement in inflammatory responses in AD is not elucidated. Based on our previous in vitro results, the aim of this study was to evaluate the effects of a pharmacological inhibition of PKR in inflammation in APPswePS1dE9 transgenic mice. Our results showed that PKR inhibition prevented the NF-κB activation and production of tumor necrosis factor alpha (TNFα) and interleukin (IL)-1β at 12 months of age without decrease of Aβ42 levels and memory deficits. Surprisingly, PKR inhibition failed to prevent IL-1β- mediated inflammation and induced a great increase in β-amyloid peptide (Aβ42) levels at 18 months of age. In this model, our findings highlight the lack of relationship between inflammation and Aβ42 levels. Moreover, the age-dependent inflammatory response must be carefully taken into account in the establishment of an anti-inflammatory therapy in AD.
A Novel 2-Aminomuconate Deaminase in the Nitrobenzene Degradation Pathway of Pseudomonas pseudoalcaligenes JS45 (1998)

2-Aminomuconate, an intermediate in the metabolism of tryptophan in mammals, is also an intermediate in the biodegradation of nitrobenzene by Pseudomonas pseudoalcaligenes JS45. Strain JS45 hydrolyzes 2-aminomuconate to 4-oxalocrotonic acid, with the release of ammonia, which serves as the nitrogen source for growth of the microorganism. As an initial step in studying the novel deamination mechanism, we report here the purification and some properties of 2-aminomuconate deaminase. The purified enzyme migrates as a single band with a molecular mass of 16.6 kDa in 15% polyacrylamide gel electrophoresis under denaturing conditions. The estimated molecular mass of the native enzyme was 100 kDa by gel filtration and 4 to 20% gradient nondenaturing polyacrylamide gel electrophoresis, suggesting that the enzyme consists of six identical subunits. The enzyme was stable at room temperature and exhibited optimal activity at pH 6.6. The Km for 2-aminomuconate was approximately 67 microM, and the Vmax was 125 micromol x min(-1) x mg(-1). The N-terminal amino acid sequence of the enzyme did not show any significant similarity to any sequence in the databases. The purified enzyme converted 2-aminomuconate directly to 4-oxalocrotonate, rather than 2-hydroxymuconate, which suggests that the deamination was carried out via an imine intermediate.
Energy Integration of High and Low Temperature Solid Sorbents for CO2 Capture (2017)

It is crucial to reduce the energy penalties related to CO2 capture processes if CCS is to be implemented at industrial scale. In this context, gas-solid sorption has become a relevant technology. The absence of large amounts of water when using dry solid sorbents and their high heat capacity reduce the energy requirements in the gas-solid sorption CO2 capture process. Depending on the sorbent composition, the gas-solid sorption process carries out at high or low temperatures. High temperature sorbents allow the utilization of waste energy while energy requirements in low temperature processes will be less demanding. This study is focused on the assessment and comparison of the final energy penalty of low-temperature (amine impregnated alumina-based solid particles) and high-temperature solid sorbents capture process (calcium oxide).
Synthesis of Polymer–Lipid Nanoparticles for Image-Guided Delivery of Dual Modality Therapy (2013)

For advanced treatment of diseases such as cancer, multicomponent, multifunctional nanoparticles hold great promise. In the current study we report the synthesis of a complex nanoparticle (NP) system with dual drug loading as well as diagnostic properties. To that aim we present a methodology where chemically modified poly(lactic-co-glycolic) acid (PLGA) polymer is formulated into a polymer-lipid NP that contains a cytotoxic drug doxorubicin (DOX) in the polymeric core and an anti-angiogenic drug sorafenib (SRF) in the lipidic corona. The NP core also contains gold nanocrystals (AuNCs) for imaging purposes and cyclodextrin molecules to maximize the DOX encapsulation in the NP core. In addition, a near-infrared (NIR) Cy7 dye was incorporated in the coating. To fabricate the NP we used a microfluidics-based technique that offers unique NP synthesis conditions, which allowed for encapsulation and fine-tuning of optimal ratios of all the NP components. NP phantoms could be visualized with computed tomography (CT) and near-infrared (NIR) fluorescence imaging. We observed timed release of the encapsulated drugs, with fast release of the corona drug SRF and delayed release of a core drug DOX. In tumor bearing mice intravenously administered NPs were found to accumulate at the tumor site by fluorescence imaging.
Structural Characterization of Self-Assembled Lipid Monolayers by NπT Simulation (1998)

Molecular dynamics simulations in which the number of molecules (N), the two-dimensional pressure (π), and the temperature (T) are held fixed (NπT) were performed on phospholipid monolayers in two-dimensional periodic boundary conditions. Four different phospholipids were simulated: dipalmitoylphosphatidylcholine (DPPC), disteroylphosphatidylethanolamine (DSPE), 1-palmitoyl-2-oleoylphosphatidylcholine (POPC), and 1-steroyl-2-arachidonylphosphatidylethanolamine (SAPE) in both low- and high-density states. The simulations reproduced pressure−area isotherms measured on a Langmuir−Blodgett trough. The alkyl chain alignment in the low-density simulations also compared well to results of H2 NMR investigations from the literature. Simulations in which the area of the lipid monolayer was fixed (NAT) were also performed. Simulations in the NπT ensemble were superior to the NAT simulations. Inaccurate equilibrium pressures and alkyl chain order resulted from the NAT simulations. Experimental results indicated the presence of a phase transition in the DPPC isotherm. The simulations indicate this phase transition results from a desolvation of the lipid headgroups as the monolayer is pressurized. However, these simulations have not completely equilibrated. This makes any conclusions drawn from these or the many similar simulations in the literature suspect until independent validation is obtained.
Cytochrome oxidase deficiency in Alzheimer's disease (1990)

We assayed cytochrome oxidase and other electron transport chain activities in platelet mitochondria isolated from patients with Alzheimer's disease (AD). Five of 6 patients had striking reductions of platelet cytochrome oxidase activity (patient mean, 83.72 +/- 82.99 nmol/min/mg; control mean, 167.14 +/- 36.21 nmol/min/mg; n = 8). Other electron transport chain catalytic activities were not significantly different than control values. AD may be a systemic illness, a primary defect in cytochrome oxidase may be pathogenically important in its production, and the mitochondrial genes encoding cytochrome oxidase subunits may be important in producing the defect.
Design of a navigation system for a household mobile robot using neural networks (2002)

A household use mobile robot is expected to perform daily duties such as cleaning and polishing the floor, delivering small items and security patrol. To accomplish these tasks, this robot must navigate in a continuously changing indoor environment and avoid obstacles. A self-navigation algorithm has been developed for such purposes by the use of neural networks. With the aid of self-navigation, this robot can be used to cover the entire floor area for cleaning purposes or move from one place to another for delivery. Through the abilities of learning and competing provided by the neural networks, the robot is capable of exploring unknown environments and searching for optimum paths so that it can fulfil its tasks with smallest overlapping spaces and minimum number of turns for energy conservation.
Development of a chemically amplified positive resist material for single-layer deep-UV lithography (1990)

The chemistry and preliminary lithographic characteristics of chemically amplified positive acting (CAMP) resist formulations that meet the deep-UV lithography requirements of low optical density and high sensitivity were described recently.11 This paper examines the lithographic performance of a specific resist comprised of poly (t-butoxycarbonyloxystyrene-sulfur dioxide) and the onium salt, triphenylsulfonium hexafluoroarsenate as the photoacid generator. The lithographic performance was evaluated as a function of resin molecular properties, sensitizer loading effects and various process parameters. Exposures of optimum formulations of this resist (CAMP) were conducted on a GCA prototype deep-UV exposure tool with a NA = 0.35 and 5X reduction optics. Line/space patterns down to 0.35p.m with vertical wall profiles and 0.4.tm windows in 1-1.2im thick films were obtained. The paper will summarize the resolution, depth of focus, exposure latitude and processing characteristics obtained from the evaluation of CAMP and will discuss the pattern transfer results obtained with different substrates. The performance of CAMP was also evaluated on substrates with topography and the results will be presented.
Characterization of adult human astrocytes derived from explant culture (1990)

Four different human astrocytic cell lines established from either epilepsy surgical specimens or cerebral white matter obtained during thalamotomy for tremor in a patient with multiple sclerosis were characterized using morphologic analysis, ultrastructural attributes, growth characteristics, and immunocytochemical analysis. Immunocytochemical characterization of cultures indicated a mean of 84% of cells contained cytoplasmic glial fibrillary acidic protein (GFAP): to confirm that GFAP(+) cells also proliferated, bromo-deoxyuridine (BrdU) uptake was measured in cell line. Our method of simplified explant culture allows establishment of astrocytic cell lines from a variety of pathologic substrates using limited amounts of human material.