gebhart/patrepeval huggingface dataset:
Glycogenosis type II (acid maltase deficiency) (1995)

Glycogen storage disease type II (GSD II/glycogenosis type II/Pompe's disease/acid maltase deficiency) is caused by the deficiency of lysosomal alpha-glucosidase resulting in lysosomal accumulation of glycogen. The disease is inherited as an autosomal recessive trait and is clinically heterogeneous. Early and late onset phenotypes are distinguished. Insight in the molecular nature of the lysosomal alpha-glucosidase deficiency and the underlying genetic defect has increased significantly during the past decade. This minireview on GSD II was written at the occasion of The International Symposium on Glycolytic and Mitochondrial Defects in Muscle and Nerve, held in Osaka, Japan, July 1994. It is an update of current literature, but also includes original data from the collaborating authors on mutations occurring in the lysosomal alpha-glucosidase gene and on prenatal diagnosis by chorionic villus sampling. The genotype-phenotype correlation and the prospects for therapy are addressed.
Development and Optimization of a Real-Time Quantitative PCR-Based Method for the Titration of AAV-2 Vector Stocks (2002)

Despite the clinical application of adeno-associated virus (AAV) gene therapy, the titration of viral stocks has not yet been standardized. This complicates the comparison of viral stocks between laboratories. Functional titering of AAV is time-consuming, requires the manipulation of hazardous material, and often has a high degree of variability. We established an optimized real-time quantitative polymerase chain reaction (RQ-PCR) titration assay to determine viral titers and compared it with a functional green fluorescent protein (GFP)-based titration method. With a combination of improved lysis procedures and RQ-PCR protocols we could decrease the intraexperimental coefficient of variation (CV) from 0.24 +/- 0.03 to 0.042 +/- 0.004 and the interexperimental CV from 0.34 +/- 0.06 to 0.093 +/- 0.028 following functional and RQPCR-based titration, respectively. This low variability conforms to even the strictest quality standards required, for example, in clinical laboratories. The highly standardized titration by RQPCR described here will be especially advantageous for groups working on AAV-based gene therapy in a good manufacturing practice setting.
New economic perspectives for resource allocation in wireless networks (2005)

The economic approaches of potential game theory and bargaining theory are applied to the area of power control in CDMA wireless networks. These perspectives help identify suitable equilibrium points, and algorithms that can be shown to converge to them. The bargaining approach also suggests an iterative method for the simultaneous routing and resource allocation problem.
The chromosomal distribution of mouse odorant receptor genes. (1996)

Odorant receptors (ORs) on nasal olfactory sensory neurons are encoded by a large multigene family. Each member of the family is expressed in a small percentage of neurons that are confined to one of several spatial zones in the nose but are randomly distributed throughout that zone. This pattern of expression suggests that when the sensory neuron selects which OR gene to express it may be confined to a particular zonal gene set of several hundred OR genes but select from among the members of that set via a stochastic mechanism. Both locus-dependent and locus-independent models of OR gene choice have been proposed. To investigate the feasibility of these models, we determined the chromosomal locations of 21 OR genes expressed in four different spatial zones. We found that OR genes are clustered within multiple loci that are broadly distributed in the genome. These loci lie within paralogous chromosomal regions that appear to have arisen by duplications of large chromosomal domains followed by extensive gene duplication and divergence. Our studies show that OR genes expressed in the same zone map to numerous loci; moreover, a single locus can contain genes expressed in different zones. These findings raise the possibility that OR gene choice may be locus-independent or involve consecutive stochastic choices.
Early growth and abdominal fatness in adult life. (1992)

The tendency to store fat abdominally, which is known to increase the risk of cardiovascular disease and diabetes independently of obesity, may be a persisting response to adverse conditions and growth failure in fetal life and infancy.
DoesMycobacterium paratuberculosisSurvive Current Pasteurization Conditions? (1998)

In a recent article, Stabel, Steadham, and Bolin reported thatMycobacterium paratuberculosis did not survive high-temperature, short-time (HTST) pasteurization simulated with an Armfield HTST laboratory pasteurizer (7). The authors effectively dismissed the considerable data generated by previous studies (1-5) which indicated that current HTST pasteurization conditions may not be effective in killing M. paratuberculosis and chose to conclude that turbulent flow of milk during pasteurization was essential for complete killing of contaminating M. paratuberculosis. However, Hope et al. (4) also employed small-scale continuous-flow pasteurizing equipment and reported that viable M. paratuberculosis cells were isolated from HTST-pasteurized milk initially containing 104M. paratuberculosis CFU/ml. These findings would tend to invalidate the argument regarding turbulent versus static heating put forward by Stabel et al. to explain the results of their study. In my opinion there are a number of other factors which may explain their inability to isolate viable M. paratuberculosis during their recent study.
Underpotential deposition of silver on polycrystalline platinum studied by cyclic voltammetry and rotating ring-disc techniques (1997)

The underpotential deposition of Ag (UPD Ag) on polycrystalline Pt surfaces has been studied combining cyclic voltammetry and rotating ring-disc measurements. A new voltammetric methodology is proposed for the proper calculation of the anodic charge density in UPD Ag desorption measurements. It involves an evaluation of the surface oxidation contribution by measurement of the charge related to PtO reduction in the following scan of the voltammetric experiment. This methodology allows one to obtain the individual contributions to the total anodic charge density. Collecting experiments carried out with the rotating ring-disc system fully confirmed the validity of the procedure. In this way, a complete monolayer of Ag deposited on polycrystalline Pt from a 0.5 M H2SO4–5.0 × 10−6M AgNO3 solution showed an experimental maximum coverage of 90%. The remaining surface was covered by residual H atoms. This phenomenon was evident for either pure UPD Ag or for depositions involving also bulk Ag. Therefore, apart from the misfit between ad-atoms and substrate, UPD Ag occurs by a straightforward mechanism involving one-electron transfer and resulting in a complete monolayer of Agads. The reappearance of adsorbed hydrogen was observed for depositions beyond two monolayers. This was associated with a surface rearrangement followed by three-dimensional nucleation of Ag that generates free-sites for H-adsorption. No evidence of Ag insertion in the Pt phase was detected in this work.
Sesquiterpene Progenitor, Germacrene A: An Alarm Pheromone in Aphids (1977)

Germacrene A, the elusive biogenetic "parent" of many sesquiterpenes, has been isolated from the spotted alfalfa aphid and identified as a new intrageneric aphid alarm pheromone.
Conformationally Constrained Phosphotyrosyl Mimetics Designed as Monomeric src Homology 2 Domain Inhibitors (1995)

Inhibitors of specific src homology 2 (SH2) domain binding interactions could potentially afford new therapeutic approaches toward a variety of diseases, including several cancers. To date SH2 domain inhibitors have been confined to small phosphotyrosyl (pTyr)-containing peptides that appear to bind along the surface of SH2 domains with specific recognition features protruding into the protein. Among these protrusions is the pTyr residue itself, which is inserted into a well-formed binding pocket. In the present study monomeric pTyr mimetics were prepared having key aspects of their structure constrained to conformations of the bound pTyr residue observed in the previously reported X-ray structure of a pTyr-peptide bound to the Lck SH2 domain. The resulting constrained pTyr mimetics were examined for inhibitory potency in six SH2 domain constructs: Lck, Src, Grb2, and the C-terminal SH2 domains of PLC gamma (PLC gamma-C) and the p85 subunit of PI-3 kinase (p85-C), as well as the N-terminal SH2 domain of SH PTP2. Although inhibition constants were in the millimolar range, it was observed that capping pTyr as its N alpha-acetyl carboxamide [(L)-1] provided a roughly 2-3-fold increase in potency relative to free pTyr. Diastereomeric indanylglycine-based analogues (+/-)-3a,b were essentially inactive. Of note was methanobenzazocine (+/-)-2. While being racemic and a partial pTyr structure, this analogue retained full binding potency of the enantiomerically pure N alpha-acetyl pTyr amide (L)-1. Modification and elaboration of 2 could potentially result in small molecule inhibitors having greater potency.
Construction and Evaluation of an Ultra Low Latency Frameless Renderer for VR (2016)

Latency - the delay between a user's action and the response to this action - is known to be detrimental to virtual reality. Latency is typically considered to be a discrete value characterising a delay, constant in time and space - but this characterisation is incomplete. Latency changes across the display during scan-out, and how it does so is dependent on the rendering approach used. In this study, we present an ultra-low latency real-time ray-casting renderer for virtual reality, implemented on an FPGA. Our renderer has a latency of ~1 ms from `tracker to pixel'. Its frameless nature means that the region of the display with the lowest latency immediately follows the scan-beam. This is in contrast to frame-based systems such as those using typical GPUs, for which the latency increases as scan-out proceeds. Using a series of high and low speed videos of our system in use, we confirm its latency of ~1 ms. We examine how the renderer performs when driving a traditional sequential scan-out display on a readily available HMD, the Oculus Rift DK2. We contrast this with an equivalent apparatus built using a GPU. Using captured human head motion and a set of image quality measures, we assess the ability of these systems to faithfully recreate the stimuli of an ideal virtual reality system - one with a zero latency tracker, renderer and display running at 1 kHz. Finally, we examine the results of these quality measures, and how each rendering approach is affected by velocity of movement and display persistence. We find that our system, with a lower average latency, can more faithfully draw what the ideal virtual reality system would. Further, we find that with low display persistence, the sensitivity to velocity of both systems is lowered, but that it is much lower for ours.
Monitoring for drug side‐effects in inflammatory bowel disease (2002)

The side-effects suitable for monitoring in patients with inflammatory bowel disease being treated with the four main groups of drugs (5-aminosalicylic acid preparations, azathioprine and 6-mercaptopurine, methotrexate, and corticosteroids) are reviewed. On the basis of the reported frequency, severity and timing of side-effects, a practical scheme of monitoring is recommended. This includes a baseline measurement of full blood count, creatinine and liver function tests in all patients. In the absence of worrying symptoms, we recommend the following: (i) no monitoring for sulfasalazine; (ii) for other 5-aminosalicylic acid preparations, the measurement of creatinine at 6 and 12 months and then annually; (iii) for azathioprine/6-mercaptopurine, thiopurine methyltransferase genotype/phenotype determination has no role in treatment monitoring, but a full blood count at 2 weeks, 1 month, 3 months and then every 3 months should be performed; (iv) for methotrexate, a full blood count and liver function tests should be performed every 3 months; (v) for steroids, dual energy X-ray absorptiometry bone scanning should be performed at the start of therapy, every year in which steroids are used if the T score is < 0, and every 3-5 years if the T score is > 0.
A sandwich enzyme immunoassay for measuring AD7C-NTP as an Alzheimer's disease marker: AD7C test (1998)

A simple, fast, reliable, and specific immunoassay has been developed to detect and measure AD7C-NTP, a biochemical marker for Alzheimer's disease, in cerebrospinal fluid (CSF). This assay, called the AD7C Test, is an enzyme-linked sandwich immunoassay (ELSIA) using 96 well microtiter plates. The plate surface is coated with a monoclonal antibody (N3I4) which has a high affinity and specificity for AD7C-NTP, capturing it effectively from CSF samples. The detection was achieved using a polyclonal antibody (ADRI). Both N3I4 and ADRI were generated using recombinantly produced AD7C-NTP. The assay is highly sensitive (30-50 pg), linear to 2.0 ng (r2 > 0.99), and reproducible (C.V. < 10%). The utility of the assay has been demonstrated using CSF specimens from early Alzheimer's disease patients and age matched controls (sensitivity of 89% and specificity of 89%).
The Mechanism of Formation of Polyunsaturated Fatty Acids by Photosynthetic Tissue (1969)

Labelled oleic acid, which is used as a substrate in studying the conversion of oleate into linoleate by Chlorella chloroplasts, is rapidly incorporated into the phospholipids. The phosphatidyl choline fraction accounts for almost all of the phospholipid label. Desaturation to linoleate lags behind the incorporation into lipids, but the newly synthesized linoleate remains esterified to phosphatidyl choline. [1‐ 14 C]Oleoyl‐phosphatidyl choline, incubated with chloroplasts, is converted into [1‐ 14 C]‐linoleoyl‐phosphatidyl choline, but considerable difficulties arise in presenting the lipid to the enzyme in an acceptable form. Oleoyl‐phosphatidyl choline formed in situ , however, does not present these difficulties and is desaturated at a faster rate than the CoA derivative under conditions in which unesterified fatty acid cannot be “activated”, and hence cannot be desaturated. We conclude that the precursor fatty acid can be transferred directly from the lipid to the desaturase enzyme and the product immediately accepted by a lipid to form a “desaturation‐acylation” cycle. Our data neither prove, nor exclude the possibility that the fatty acid is transformed while still attached to the lipid in ester linkage.
Phase 1 Evaluation ofVibrio choleraeO1, Serotype Inaba, Polysaccharide-Cholera Toxin Conjugates in Adult Volunteers (1998)

Conjugate vaccines were prepared by binding hydrazine-treated lipopolysaccharide (DeALPS) from Vibrio cholerae O1, serotype Inaba, to cholera toxin (CT) variants CT-1 and CT-2. Volunteers (n = 75) were injected with either 25 microg of DeALPS, alone or as a conjugate, or the licensed cellular vaccine containing 4 x 10(9) organisms each of serotypes Inaba and Ogawa per ml. No serious adverse reactions were observed. DeALPS alone did not elicit serum LPS or vibriocidal antibodies in mice and only low levels of immunoglobulin M (IgM) anti-LPS in the volunteers. Recipients of the cellular vaccine had the highest IgM anti-LPS levels, but the difference was not statistically significant from that elicited by the conjugates. The conjugates elicited the highest levels of IgG anti-LPS (DeALPS-CT-2 > DeALPS-CT-1 > cellular vaccine). Both conjugates and the cellular vaccine elicited vibriocidal antibodies: after 8 months, recipients of cellular vaccine had the highest geometric mean titer (1,249), followed by DeALPS-CT-2 (588) and DeALPS-CT-1 (330). The correlation coefficient between IgG anti-LPS and 2-mercaptoethanol (2-ME)-resistant vibriocidal antibodies was 0. 81 (P = 0.0004). Convalescent sera from cholera patients had a mean vibriocidal titer of 2,525 that was removed by treatment with 2-ME. The vibriocidal activities of sera from all vaccine groups and from the patients were absorbed (>75%) by LPS but not by either CT-1 or CT-2. Conjugate-induced IgG vibriocidal antibodies persisted longer than those elicited by the whole-cell vaccine. Both conjugates, but not the cellular vaccine, elicited IgG anti-CT.
Reducing Economic Risk in Areally Anisotropic Formations with Multiple-Lateral Horizontal Wells (1995)

Abstract Well orientation is critical to horizontal well performance in areally anisotropic reservoirs. A horizontal well, drilled normal to the direction of maximum permeability, will have higher productivity than one drilled in any other arbitrary direction. Currently, horizontal permeability magnitudes and even indications of direction are rarely measured in the field. Based on well performance modeling and economic evaluation, this study attempts to determine the relative attractiveness of horizontal wells with multiple-laterals. The work exposes the economic risk in ignoring horizontal permeability magnitudes and directions and demonstrates the importance of adequate reservoir testing. A new rationalization for multiple-lateral horizontal wells is the reduction of the economic risk associated with poor reservoir characterization in areally anisotropic formations while increasing the incremental net present value (NPV) over single-horizontal wells.
Near-field power transfer and backscattering communication to miniature RFID tag in 65 nm CMOS technology (2016)

This work introduces an inductive wireless power transfer (IWPT) and backscattering communication to an extremely small CMOS RFID tag with a size of 200 μm by 200 μm. This is the smallest RFID tag to date, with the passive area taken into account. DC power of 0.1 mW can be generated on-chip with 21 dBm source power at 2 GHz. An amplitude-shift keying (ASK) back-scattering communication link is demonstrated. The on-chip matching varactor is switched to maximize the distance between the two reflection (backscattering) coefficients. A directional coupler is used to suppress the Tx-to-Rx leakage and to extract the backward scattering wave. An 8-bit, 5-GS/s ADC can demodulate the 625kb/s backscattering signal.
Generation of Synthetic Elastin-Mimetic Small Diameter Fibers and Fiber Networks (2000)

Elastin-mimetic peptide polymers have been synthesized, and the morphological properties of fabricated small diameter fibers and nonwoven fabrics have been characterized. An 81 kDa recombinant protein based upon the repeating elastomeric peptide sequence of elastin (Val-Pro-Gly-Val-Gly)4(Val-Pro-Gly-Lys-Gly) was obtained through bacterial expression of an oligomerized gene coding for tandem repeats of the monomer. The protein was processed into fibers by an electrospinning technique and morphology defined by SEM and TEM. The choice of processing parameters influenced both fiber diameter and morphology with diameters varying between 200 and 3000 nm and three morphological patterns noted: beaded fibers, thin filaments, and broad ribbonlike structures. Detailed image analysis of nonwoven textile fabrics produced from elastin-mimetic fibers revealed that the distribution of single fiber orientation was isotropic with an associated unimodal distribution of protein fiber diameter. In a dry state, the ultimate tensile strength of nonwoven fabrics generated from elastin-mimetic peptides was 35 MPa with a material modulus of 1.8 GPa.
Performance issues in public ABR service (1996)

The available bit rate (ABR) service attracted much attention during the negotiations leading to Traffic Management Specification Version 4.0, finalized by the ATM Forum. In thr ABR service, feedback flow control of the source rate is provided in response to the changing asynchronous transfer mode (ATM)-layer transfer characteristics. The reference behavior of the source end system, the destination end system, and the switch, as detailed in the specification, allows cooperative control among these systems. The performance of the public ABR service is discussed in connection with the evolution of ATM switches. Public networks with first-generation switches provide an ABR service with a limited peak cell rate (PCR), while those with second-generation switches can provide an ABR service with any PCR. In such networks, TCP-over-ABR works well. Point-to-multipoint ABR will be provided in advanced switches. A method is proposed for maintaining the throughput of point-to-multipoint ABR when the number of leaves is increased.
Production of glycerol from methanol by a mutant strain of Candida boidinii No. 2201. (1989)

We have studied the peculiar phenomenon of glycerol metabolism in methylotrophic yeasts1'2) and found the presence of a new enzyme, dihydroxyacetone reductase,
Prevalence of Pre-existing Antibodies to CRISPR-Associated Nuclease Cas9 in the USA Population (2018)

The repurposing of the CRISPR/Cas microbial adaptive immune system for gene editing has resulted in an exponential rise in new technologies and promising approaches for treating numerous human diseases. While some of the approaches being currently developed involve <i>ex vivo</i> editing by CRISPR/Cas9, many more potential applications will require <i>in vivo</i> editing. The <i>in vivo</i> use of this technology comes with challenges, one of which is the immune response to Cas9, a protein of microbial origin. Thus, the prevalence of pre-existing antibodies to Cas9 could also be a relevant parameter. There are many avenues for how CRISPR/Cas9 technologies will be applied <i>in vivo</i>, including the mode of delivery. These may be expected to invoke different immunological pathways. Nonetheless, as with all protein therapeutics, it may be desirable to monitor for anti-Cas9 antibodies during clinical development. This will require the development of robust and reliable assays. Here, we describe ELISA-based assays that are capable of detecting antibodies to Cas9 from <i>Staphylococcus aureus</i> (SaCas9) and <i>Streptococcs pyogenes</i> (SpCas9) in human sera. Furthermore, using these assays to screen for pre-existing antibodies in 200 human serum samples, we found the prevalence of anti-SaCas9 and anti-SpCas9 antibodies to be 10% and 2.5%, respectively.
Proteolytic Activation of the Spike Protein at a Novel RRRR/S Motif Is Implicated in Furin-Dependent Entry, Syncytium Formation, and Infectivity of Coronavirus Infectious Bronchitis Virus in Cultured Cells (2009)

The spike (S) protein of the coronavirus (CoV) infectious bronchitis virus (IBV) is cleaved into S1 and S2 subunits at the furin consensus motif RRFRR(537)/S in virus-infected cells. In this study, we observe that the S2 subunit of the IBV Beaudette strain is additionally cleaved at the second furin site (RRRR(690)/S) in cells expressing S constructs and in virus-infected cells. Detailed time course experiments showed that a peptide furin inhibitor, decanoyl-Arg-Val-Lys-Arg-chloromethylketone, blocked both viral entry and syncytium formation. Site-directed mutagenesis studies revealed that the S1/S2 cleavage by furin was not necessary for, but could promote, syncytium formation by and infectivity of IBV in Vero cells. In contrast, the second site is involved in the furin dependence of viral entry and syncytium formation. Mutations of the second site from furin-cleavable RRRR/S to non-furin-cleavable PRRRS and AAARS, respectively, abrogated the furin dependence of IBV entry. Instead, a yet-to-be-identified serine protease(s) was involved, as revealed by protease inhibitor studies. Furthermore, sequence analysis of CoV S proteins by multiple alignments showed conservation of an XXXR/S motif, cleavable by either furin or other trypsin-like proteases, at a position equivalent to the second IBV furin site. Taken together, these results suggest that proteolysis at a novel XXXR/S motif in the S2 subunit might be a common mechanism for the entry of CoV into cells.