gebhart/patrepeval huggingface dataset:
Compound Heterozygous Protein C Deficiency Caused by Two Mutations, Arg-178 to Gin and Cys-331 to Arg, Leading to Impaired Secretion of Mutant Protein C (1994)

The protein C gene in a patient apparently homozygous for protein C deficiency was analyzed. Two different point mutations, each located in a different allele, were detected to reveal that the patient is a compound heterozygote. Mutation of Arg-178 (CGG) to Gln (CAG) [mutation I] was detected in exon VII, in the vicinity of activation peptide cleavage site by thrombin. Mutation of Cys-331 (TGC) to Arg (CGC) [mutation II] was found in exon IX, at one of the sites involved in disulfide bond formation in the catalytic domain of the heavy chain. The alteration of Cys-331 to Arg disables the formation of the disulfide bond and would alter the protein conformation. Transient expression assays using COS-7 cells transfected with protein C expression vectors containing each one of these two mutations suggested that each of the two mutations would lead to the protein C deficiency by an impairment of secretion of the respective mutant proteins.
The Proteasome in Health and Disease (2012)

The giant proteolytic factory called the proteasome came a long way from a biochemical curio to a major regulator of cellular physiology and a renowned drug target within the ubiquitin proteasome pathway (UPP). Thanks to availability of highly specific inhibitors of the proteasome, in less than twenty years it was possible to identify major transcription factors, cyclins, and products of oncogenes as crucial substrates for the UPP. Nine years passed since the FDA speedily approved bortezomib, the inhibitor of proteasome, for treatment of multiple myeloma. One year after its approval, the field was honored by awarding the Nobel Prize to Hershko, Ciechanover and Rose for introducing the concept of controlled proteolysis of ubiquitin-tagged substrates, with proteasome as the intracellular recycling facility. Taking into consideration the universal involvement of the proteasome in the life of all cells in human body, it comes to no surprise that the enzyme is deeply implicated in etiology, progression, diagnosis or cure of multiple diseases. Below we discuss some aspects of the involvement: from direct causative links to changes in proteasome properties that correlate with pathological conditions. We start with diseases collectively known as cancer, and with immune system-related pathologies. Here, the proteasome inhibitors are either already used in clinics, or undergo advanced preclinical screening. Then, we will continue with cardiovascular disorders, followed by aging. Changes of the proteasome make-up during aging may be a priming factor for neurodegenerative diseases, described last. We discuss the potential for proteasome regulation: inhibition, activation or specificity modulation, to successfully enter the clinical setting.
Cytochrome c oxidase contains an extra charged amino acid cluster in a new type of respiratory chain in the amino-acid-producing Gram-positive bacterium Corynebacterium glutamicum The GenBank/EMBL/DDBJ accession numbers for the sequences reported in this paper are AB052748 and AB052749. (2001)

The membranes from Corynebacterium glutamicum cells contain a hydrophobic di-haem C protein as the cytochrome c subunit of the new type of cytochrome bc complex (complex III in the respiratory chain) encoded by the qcrCAB operon [Sone, N., Nagata, K., Kojima, H., Tajima, J., Kodera, Y., Kanamaru, T., Noguchi, S. & Sakamoto, J. (2001). Biochim Biophys Acta 1503, 279–290]. To characterize complex IV, cytochrome c oxidase and its structural genes were isolated. The oxidase is of the cytochrome aa 3 type, but mass spectrometry indicated that the haem is haem As, which contains a geranylgeranyl side-chain instead of a farnesyl group. The enzyme is a SoxM-type haem–copper oxidase composed of three subunits. Edman degradation and mass spectrometry suggested that the N-terminal signal sequence of subunit II is cleaved and that the new N-terminal cysteine residue is diacylglycerated, while neither subunit I nor subunit III is significantly modified. The genes for subunits II (ctaC) and III (ctaE) are located upstream of the qcrCAB operon, while that for subunit I (ctaD) is located separately. The oxidase showed low enzyme activity with extrinsic substrates such as cytochromes c from horse heart or yeast, and has the CuA-binding motif in its subunit II. A prominent structural feature is the insertion of an extra charged amino acid cluster between the β2 and β4 strands in the substrate-binding domain of subunit II. The β2–β4 loop of this oxidase is about 30 residues longer than that of major cytochrome c oxidases from mitochondria and proteobacteria, and is rich in both acidic and basic residues. These findings suggest that the extra charged cluster may play a role in the interaction of the oxidase with the cytochrome c subunit of the new type of bc complex.
Enhancement by exogenous and locally generated angiotensin II of purinergic neurotransmission via angiotensin type 1 receptor in the guinea‐pig isolated mesenteric artery (1997)

1. Angiotensin II is known to enhance sympathetic neurotransmission in the vasculature by increasing the release of noradrenaline, but little is known about the effect on the co-released transmitter, adenosine 5'-triphosphate (ATP). In the present study we have examined the effect of angiotensin II on the excitatory junction potential (e.j.p.) elicited by repetitive field stimulation in the guinea-pig isolated mesenteric artery, to establish the angiotensin II receptor subtype involved in modulating the release of ATP and the role of the endothelium in converting angiotensin I to angiotensin II. 2. Suramin (300 microM), a P2 purinoceptor antagonist, abolished both the e.j.p.s and depolarizing response to alpha,beta-methylene-ATP, a stable analogue of ATP, without affecting the resting membrane potential and noradrenaline-induced depolarization. 3. Angiotensin II (0.1 microM) affected neither the resting membrane potential nor the amplitude of the first e.j.p., but increased the amplitudes of the subsequent e.j.p.s. This enhancing effect of angiotensin II was abolished by CV-11974 (0.1 microM), an angiotensin II type 1 (AT1) receptor antagonist, but unaffected by PD 123319 (1 microM), an angiotensin II type 2 (AT2) receptor antagonist, or CGP 42112A (1 microM), AT2 receptor ligand. 4. Angiotensin I (0.1 microM) exerted a similar effect on e.j.p.s to that of angiotensin II. CV-11974 (0.1 microM) or temocaprilat (10 microM), an angiotensin converting enzyme (ACE) inhibitor, abolished the effect of angiotensin I. Removal of the endothelium did not alter the action of angiotensin I. 5. The results of the present study indicate that the release of ATP from sympathetic nerves innervating the guinea-pig isolated mesenteric artery, as determined from the magnitude of the e.j.p., can be enhanced by angiotensin II via activation of prejunctional AT1 receptors. Qualitatively similar effects were observed with angiotensin I, which appears to be converted into angiotensin II by a subendothelial process.
A review of NDE methods for porosity measurement in fibre-reinforced polymer composites (2004)

Low porosity levels are essential for ensuring the performance of carbon fibre-reinforced composite structures. Despite many attempts to measure absolute porosity levels as a process-control tool, the currently accepted methods often rely on relative measurements in comparison with a reference standard. However, newer low-cost manufacturing methods such as resin-transfer moulding (RTM) and resin film infusion (RFI) would benefit from a reliable absolute 'porosity meter.' This paper reviews the literature documenting NDE methods for porosity measurement in fibre-reinforced polymer composites. The majority of the papers have concentrated on ultrasonic methods to determine porosity levels, mainly for quality control purposes. A few workers have reported on the application of other NDE methods. The review concentrates on methods that have potential for use during composite manufacture. To date, no one NDE method has been able to provide a porosity measurement independent of both the pore morphology and the fibre and resin matrix materials.
Facile Preparation of Aromatic Fluorides by the Fluoro-Dediazoniation of Aromatic Diazonium Tetrafluoroborates Using HF-Pyridine Solution (1994)

Abstract The fluoro-dediazoniation of ArN2BF4 using HF-pyridine solution has been successfully carried out either thermally or photochemically to afford the corresponding ArF in good yields. Particularly, the photochemically induced reaction in HF-pyridine was a useful tool for the preparation of ArF having polar substituents such as halogens, OH, OMe, CF3, etc.
How Permeability Depends on Stress and Pore Pressure in Coalbeds: A New Model (1998)

Summary In naturally fractured formations such as coal, permeability is sensitive to changes in stress or pore pressure (i.e., changes in effective stress). This paper presents a new theoretical model for calculating pore volume (PV) compressibility and permeability in coals as a function of effective stress and matrix shrinkage, by means of a single equation. The equation is appropriate for uniaxial strain conditions, as expected in a reservoir. The model predicts how permeability changes as pressure is decreased (i.e., drawdown). PV compressibility is derived in this theory from fundamental reservoir parameters. It is not constant, as often assumed. PV compressibility is high in coals because porosity is so small. A rebound in permeability can occur at lower drawdown pressures for the highest modulus and matrix shrinkage values. We have also history matched rates from a boomer well in the fairway of the San Juan basin by use of various stress-dependent permeability functions. The best fit stress/permeability function is then compared with the new theory.
Geolocation using video sensor measurements (2007)

Ground target tracking using electro-optical and infrared video sensors onboard unmanned aerial vehicles has drawn a great deal of interest in recent years due to the evolution of inexpensive video sensors and platforms. We present algorithms for geolocation using pixel location measurements which are based on the perspective transformation and includes radial and tangential lens distortions. The covariance of geolocation error takes into account the errors in pixel location, intrinsic and extrinsic camera parameters, and terrain height. Pixel coordinates of the optical center, focal distances along the X and Y axes, lens distortion parameters, and the skew parameter constitute the intrinsic camera parameters. The extrinsic camera parameters include the sensor position and sensor attitude relative a local east-north-up coordinate frame. Numerical results are presented using simulated data. Our results show that the errors in the Euler angles used to represent the sensor attitude is the dominant source of geolocation error.
LEKTI demonstrable by immunohistochemistry of the skin: a potential diagnostic skin test for Netherton syndrome (2004)

NS can be difficult to diagnose especially in the early stage, which can lead to inappropriate treatments particularly if it is misdiagnosed as atopic dermatitis. Immunohistochemistry of skin with an antibody against LEKTI is a potentially useful diagnostic test for NS.
Complexes between Herpes Simplex Virus Glycoproteins gD, gB, and gH Detected in Cells by Complementation of Split Enhanced Green Fluorescent Protein (2007)

The interactions between herpes simplex virus gD and its nectin1 receptor or between gD, gB, and gH were analyzed by complementation of the N and C portions of split enhanced green fluorescent protein (EGFP) fused to the glycoproteins. The gD(N)-Nect(C) complex was readily detected; the gD(N)-gC(C) complex was undetectable, highlighting the specificity of the assay. Split EGFP complementation was detected between proteins designated gD(N)+gH(C), gD(N)+gB(C), and gH(N)+gB(C)+wtgD (gB was deleted of endocytosis motifs), both in cells transfected with two-tree glycoproteins and in syncytia. The in situ assay provides evidence that gD interacts with gH and gB independently of each other and supports a model whereby gH and gB in complex exert their activities to gD.
Squaraine Dyes for Dye‐Sensitized Solar Cells: Recent Advances and Future Challenges (2013)

Abstract In the past few years, squaraine dyes have received increasing attention as a sensitizer for application in dye‐sensitized solar cells. This class of dyes not only leaves open a good opportunity to afford conventional high performance dyes but also holds great promise for applications in transparent solar cells due to its low absorption intensity in the eye‐sensitive region. This review provides a summary of the developments on squaraine dyes in the field of dye‐sensitized solar cells and the opportunities used to improve their overall energy conversion efficiency. In particular, the main factors responsible for the low values of open‐circuit voltage, short‐circuit photocurrent and fill factor are discussed in detail. Future directions in research and development of near‐infrared (NIR) organic materials and their applications are proposed from a personal perspective.
Pollution Prevention Handbook (1995)

Programs and Management Introduction and Purpose Government and Industry Programs for Reducing Pollution Setting Up a Successful Pollution Prevention Program Total Quality Environmental Management: Managing Corporate Changes Performing a Pollution Prevention Assessment Recycling Programs for Solid Waste Waste and Material Exchanges and Recyclers Treatment to Reduce Disposal Economic Evaluation in Pollution Prevention Programs Manufacturing Processes and Pollution Prevention Machining and Other Metalworking Operations Solvents Used for Cleaning Refrigeration, Firefighting, and Other Uses Metal Plating and Surface Finishing Painting and Coating Removal of Paint and Coatings Motor Oil and Antifreeze Industry Groups Aluminum Construction and Demolition Electric Utilities Food Processing Iron and Steel Petroleum Exploration and Refining Pharmaceuticals Pulp and Paper Industry
Temporal and spatial patterns of osteoblast activation following implantation of ?-TCP particles into bone (1998)

Temporal and spatial patterns of osteoblast activation around beta-TCP particles implanted into bone were analyzed by in situ hybridization with digoxygenin-labeled procollagen alpha 1(I) RNA probes. beta-TCP particles (150-300 microns in diameter) were implanted into rat tibiae, and specimens were collected 3, 5, 7, 14, and 28 days after operation. Activated osteoblasts displayed intense procollagen alpha 1(I) RNA specific labeling. At day 3, osteoblasts lining pre-existing trabeculae in places showed a specific signal. Additionally, scattered activated cells compatible with preosteoblasts also were observed in the vicinity of the trabeculae among red blood cells that filled the space between beta-TCP particles. Osteoblast activation on the surface of beta-TCP rarely was observed. At days 5 and 7, osteoblast activation and bone formation advanced centripetally. At the forefront of bone formation positive cells were scattered in the blood cell clots, and some of the positive cells colonized forming new bone matrix. Formation of new bone did not always begin at the surface of beta-TCP. At day 14, most of the beta-TCP particles were tightly associated with newly formed bone, and the number of positive osteoblasts was reduced. At day 28, absorption of the newly formed bone and the beta-TCP by multinuclear cells was sporadically demonstrated. Such cells often were accompanied by active osteoblasts, suggesting early bone remodeling. In conclusion, in situ hybridization with procollagen alpha 1(I) was employed to demonstrate precisely the mode of recruitment of bone cell precursors. beta-TCP does not positively guide collagen I expressing bone cells along its surface. It has no apparent effects on bone regeneration.
Analog dispersive time delayer for beam-scanning phased array without beam-squinting (2008)

This paper presented the application of the CRLH transmission line as a TD line applicable in squint-free beam-scanning phased-arrays. The CRLH TD exhibits attractive characteristics such as continuous and variable delay, no power consumption, no switching time, broadband matching, planar and cost-effective fabrication.
Degradation Phenomena of Reforming Catalyst in DIR-MCFC (2012)

Recently, energy consumption is increasing with the population growth of developed countries and developing countries, and an exhausting of petroleum resources has become a big problem worldwide. In addition, CO 2 , SO X and NO X which are toxic substances emitted when these resources are used, cause environmental polluting problem such as global warming and acid rain, etc. From these situations, high efficiency, energy-saving and environmental-saving power source is needed in recent years, and fuel cell can solve these problems. Therefore, researching, developing and commercializing on fuel cell are promoted in many countries. Fuel cell is categorized by the difference of its electrolyte and operating temperature. In this research, we use Molten Carbonate Fuel Cells (MCFC) which uses a molten carbonate as the liquid electrolyte. MCFC is expected as a distributed power station and the CO 2 concentrator .Generally, fuel cell performances are evaluated by I-V performance, cell resistance and gas chromatography, etc. for which fuel cell only generate by supplying fuel gas and oxidant gas to cell. Generally, although gas chromatography is utilized only to detect the gas crossover (it is gas leakage phenomenon between two poles through electrolyte) on fuel cell development, the performance of reforming catalyst in the anode channel in operation is evaluated by gas chromatography on R&D of Direct Internal Reforming-Molten Carbonate Fuel Cell (DIR-MCFC). Moreover, MCFC installed the segmented electrode is evaluated about the gas crossover and the utility of the segmented electrode by a gas chromatography and the image measurement technique. This chapter introduces the use method of a gas chromatograph through the degradation factor of reforming catalyst in DIR-MCFC.
Short Notices (1976)

Short Notices Get access K. THEODORE HOPPEN K. THEODORE HOPPEN University of Hull Search for other works by this author on: Oxford Academic Google Scholar The English Historical Review, Volume XCI, Issue CCCLVIII, January 1976, Pages 200-a–200, https://doi.org/10.1093/ehr/XCI.CCCLVIII.200-a Published: 01 January 1976
A Novel Method for the Simultaneous Enrichment, Identification, and Quantification of Phosphopeptides and Sialylated Glycopeptides Applied to a Temporal Profile of Mouse Brain Development (2012)

We describe a method that combines an optimized titanium dioxide protocol and hydrophilic interaction liquid chromatography to simultaneously enrich, identify and quantify phosphopeptides and formerly N-linked sialylated glycopeptides to monitor changes associated with cell signaling during mouse brain development. We initially applied the method to enriched membrane fractions from HeLa cells, which allowed the identification of 4468 unique phosphopeptides and 1809 formerly N-linked sialylated glycopeptides. We subsequently combined the method with isobaric tagging for relative quantification to compare changes in phosphopeptide and formerly N-linked sialylated glycopeptide abundance in the developing mouse brain. A total of 7682 unique phosphopeptide sequences and 3246 unique formerly sialylated glycopeptides were identified. Moreover 669 phosphopeptides and 300 formerly N-sialylated glycopeptides differentially regulated during mouse brain development were detected. This strategy allowed us to reveal extensive changes in post-translational modifications from postnatal mice from day 0 until maturity at day 80. The results of this study confirm the role of sialylation in organ development and provide the first extensive global view of dynamic changes between N-linked sialylation and phosphorylation.
A Combination of α-Fetoprotein and Des-&#947;-Carboxy Prothrombin Is Superior in Detection of Hepatocellular Carcinoma (2013)

The data suggest that AFP alone is not sufficient for the serological diagnosis of HCC in European patients, while a combination of AFP and DCP can increase the sensitivity even in early-stage patients.
Reproducibility of quantitative indices of lung function and microstructure from <sup>129</sup>Xe chemical shift saturation recovery (CSSR) MR spectroscopy (2016)

<sup>129</sup> Xe CSSR presents a reproducible method for estimation of alveolar septal thickness. Magn Reson Med 77:2107-2113, 2017. © 2016 The Authors Magnetic Resonance in Medicine published by Wiley Periodicals, Inc. on behalf of International Society for Magnetic Resonance in Medicine. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Clinical and pathological findings in a HERDA‐affected foal for 1.5 years of life (2007)

A Quarter horse filly bred from two horses affected with HERDA (hereditary equine regional dermal asthenia) was observed clinically and its skin histologically for the 1.5 years of its life. Severe signs of the disease did not manifest until 1.5 years of age, and were not temporally related to saddling. Histological comparison to an age-, breed- and sex-matched control did not show any consistent diagnostic features. Monitoring of the proband substantiated previous reports of (i) the autosomal recessive nature of the disease, (ii) mares affected with HERDA being able to foal without damage to the skin or reproductive tract, (iii) HERDA foals appearing phenotypically normal throughout the first year of life, and (iv) demonstrated that histological interpretation of skin specimens from grossly normal skin may be insufficient to differentiate HERDA-affected horses from controls.
[Potential filaricides. Suramin analogs]. (1986)

A series of suramin analogues has been synthesized in which the methyl groups of suramin have been replaced by hydrogen, alkyl, phenyl, and fluoro substituents, or which contain more than two methyl groups. The substances have been screened against Dipetalonema viteae in Meriones unguiculatus, Litomosoides carinii in Sigmodon hispidus and L. carinii in Mastomys natalensis, respectively. Small structural modifications have a marked influence on the antifilarial activity. There are marked differences between antifilarial and trypanocidal activities. A symmetrical molecule structure seems not to be essential for the antifilarial activity.
C pk and P pk : One Capability Index Is Not Enough to Measure Photomask CD Performance (2002)

Many photomask customers require process capability reporting on a regular basis. One of their main purposes is to evaluate product quality based on process variability from period to period. The problem occurs when process improvement is based on a single reported index. There are a variety of methods used today to calculate process capability, and any one method has proven insufficient (see figure below). Process 'A' looks good with respect to the average-range divided by a constant (R-bar/d<sub>2</sub>), but the standard deviation looks too wide. Conversely, Process 'B' looks good with respect to standard deviation, but its R-bar/d<sub>2</sub> is skewed too far. An explanation for this phenomenon is that the use of the standard deviation method to calculate capability (actually the performance index) ignores within-sample variation, and using the R-bar/d<sub>2</sub> method omits sample-to-sample variation. Therefore, both methods should be employed to better evaluate process performance and capability. This paper presents a means by which to employ the merits of both sigma estimators to achieve a better representation of continual improvement. Utilizing a method of pooling within and between-sample variation (Cvk) indicates that the two processes below actually exhibit almost identical capability.