gebhart/patrepeval huggingface dataset:
The molecular basis of cystathionine ?-synthase deficiency in Australian patients: Genotype-phenotype correlations and response to treatment (2002)

Cystathionine beta-synthase (CBS) deficiency is the most common cause of homocystinuria. It is inherited as an autosomal recessive trait and common clinical features are: dislocation of the optic lens, osteoporosis, mental retardation, and thromboembolism. We determined the molecular basis of CBS deficiency in 36 Australian patients from 28 unrelated families, using direct sequencing of the entire coding region of the CBS gene. The G307S and I278T mutations were the most common mutations. They were present in 19% and 18% of independent alleles, respectively. In total, seven novel and 20 known mutations were detected. Of those, the two novel missense mutations (C109R and G347S), as well as two known missense mutations (L101P and N228K), were expressed in E. Coli. All mutant proteins completely lacked catalytic activity. Furthermore, we studied the correlation between genotype and the biochemical response to pyridoxine treatment in the patients of whom 13 were pyridoxine responsive, 21 were non-responsive, and two were partially responsive. The G307S mutation always resulted in a severe non-responsive phenotype, whereas I278T resulted in a milder B6 responsive phenotype. From our results, we were also able to establish three other mild mutations: P49L, R369C, and V371M.
White electrophosphorescence from semiconducting polymer blends (2004)

White emission from polymer light-emitting diodes (PLEDs) is demonstrated by spin-casting polymer blends from solution containing poly(9,9-dioctylfluorenyl-2.7-diyl) (PFO) and tris (2,5-bis-2'-(9',9'-dihexylfluorene) pyridine) iridium (III), Ir(HFP)<sub>3</sub>. The white electrophosphorescence PLEDs exhibit luminance of 1.2 x 104 cd/m<sup>2</sup> at 17 volts and luminous efficiency of 4.3 cd/A at current density of 5.2 mA/cm<sup>2</sup>. Because a single semiconducting polymer, PFO, was used as the common host for red, green and blue emission, the color coordinates, the color temperatures and the color rendering indices of the white emission are insensitive to the brightness, applied voltage and applied current density.
Aminoalkylation of 10‐Hydroxycamptothecin Using Methylene Chloride under Solid—Liquid Phase Transfer Catalysis: A New Approach for the Preparation of Topotecan. (2004)

Abstract For Abstract see ChemInform Abstract in Full Text.
Direct current glow discharges in atmospheric air (2002)

Summary form only given, as follows. Research on atmospheric pressure glow discharges in air is motivated by applications such as instantly activated reflectors and absorbers for electromagnetic radiation, remediation and detoxification of gaseous pollution, and surface treatment. One of the major obstacles in obtaining stable glow discharges at high electron densities (> 10/sup 11/ cm/sup -3/) is the glow-to-arc-transition, which develops generally in the cathode fall region. The use of a microhollow cathode discharge as independent electron emitter, has been shown to reduce the cathode fall drastically, and has therefore allowed us to generate direct current, atmospheric-pressure air glow discharges. The millimeter size plasmas, studied in earlier experiments, have been scaled to centimeter dimensions by operating MHCD sustained air glows in parallel and extending the electrode distance up to 2 cm. The electric field in these discharge plasmas, varies between 1.2 kV/cm, for a discharge current of 13 mA, to 2 kV/cm at 5 mA. The current density varies between 50 mA/cm/sup 2/ for high currents (13 mA) and 500 mA/cm/sup 2/ for low currents (5 mA), corresponding to electron densities between 10/sup 11/ cm/sup -3/ and 10/sup 12/ cm/sup -3/. The MHCD supported air glow discharge has a negative differential resistance. Parallel operation of the individual discharges therefore requires individual ballast. Two parallel discharges were generated, with their axes 0.4 cm apart. It was found that for this configuration the positive columns merged for currents exceeding 10 mA, creating a homogeneous plasma. The required electrical power density for electron densities of 1.5 /spl times/ 10/sup 11/ cm/sup -3/ was measured as 100 W/cm/sup 3/. By pulsing the plasma this power dissipation can be reduced considerably without sacrificing on the time-average value of the electron density.
PM double-clad fibers for high-power lasers and amplifiers (2003)

Fibers for high-power laser and amplifier applications require large claddings with high numerical apertures for efficiently coupling pump energy. In addition, such fibers should have high rare-earth dopant concentrations in relatively large cores, with low numerical apertures, to reduce non-linearities. Furthermore, polarization maintaining double-clad fibers (PM-DCF) are needed for coherently combining the outputs of several lasers/amplifiers to achieve output powers in excess of 100 kW for military and industrial laser applications. In this paper, we report the progress made towards fabricating PM double-clad fibers, with a variety of fiber characteristics, to facilitate development and production of high-power lasers and amplifiers. In particular, a Panda-type PM-DCF with a 0.06 NA, 30 micron diameter, Yb-doped core is reported. We also discuss various criteria that are critical for designing these PM double clad fibers.
Sensitive Detection of Viral Antigens with a New Method, “Laser Magnet Immunoassay” (1991)

A new method, "laser magnet immunoassay" (LMIA), has been developed for sensitive detection of viral antigens. Target viruses captured on microbeads were made to react with antibodies labeled with magnetite particles. In a magnetic field, magnetically labeled antigens dispersed in water were attracted to and concentrated at one point on the surface, resulting in the lifting up of a small surface area. A laser beam which was incident on the point reflected, making an interference fringe. The intensity of the fringe indicates the amount of the magnetite conjugated with antigen. A very low concentration of antigens, such as 5 particles of influenza virus and 0.1 pg/ml of human immunodeficiency virus (HIV) p24 antigen in human serum, could be detected by this method. Application of this method to diagnoses of viral diseases in early stages is discussed.
Broadband and gain-flattened Er <sup>3+</sup> -dopedtellurite fibreamplifier constructed using a gain equaliser (1998)

The authors have constructed a broadband and gain-flattened Er3+-doped tellurite fibre amplifier by using a Mach-Zehnder type optical filter as a gain equaliser. They obtained a 3 dB down bandwidth of 68 nm in the 1536 – 1604 nm wavelength region.
Translational development of BAFF-R-specific chimeric antigen receptor T-cell therapy targeting B-cell lymphoid malignancies. (2023)

e19501 Background: Relapsed and/or refractory (R/R) disease is observed in patients who receive CD19 targeting chimeric antigen receptor (CAR)-T cells to treat B-cell lymphoid leukemias and lymphomas. We generated a novel CAR, MC10029 that targets B-cell activating factor receptor (BAFF-R) to address this unmet medical need. Methods: We engineered a second-generation BAFF-R CAR (MC10029 CAR). The construct includes the single chain variable fragment (scFv) of our newly developed BAFF-R antibody with CD28 costimulatory and CD3ζ signaling domains. We confirmed MC10029 CAR-T cell antigen-specific activation with in vitro degranulation assays and a direct cytolytic assay. We used BAFF-R positive leukemia and lymphoma cell lines in both in vitro and in vivo models to further characterize MC10029 CAR-T cells, and finally, we enriched tumor primary cells from chronic lymphocytic leukemia (CLL) patients as a target for MC10029 CAR-T cells. In preparation for our Phase I clinical trial, we have manufactured three batches of CAR-T cells using a MC10029-encoding lentiviral vector produced under GMP conditions. The production batches of MC10029 CAR-T cells were evaluated for product quality with Viability, Identity and Potency as well as Safety from adventitious viral agents. Antigen specific cytotoxicity of the MC10029 CAR-T cells was confirmed using our standard degranulation assay. Results: MC10029 CAR-T cells elicited in vitro antigen-specific cytotoxicity against Nalm-6 cells, Z138 cells, MEC-1 cells, and a CD19 knock out Nalm-6 model designed to mimic CD19-negative R/R disease. We demonstrated in vivo antitumor effects of MC10029 CAR-T cells in NSG mice challenged with these same cell lines. We showed cytotoxicity of MC10029 CAR-T cells against CLL patients’ tumors. We also showed our quality control results to highlight our clinical trial trajectory. Conclusions: Novel MC10029 CAR-T cells showed broad utility against BAFF-R positive B-cell lymphoid malignancies in a series of in vitro, in vivo, and ex vivo models. With these robust data, we have moved forward to generate clinical grade MC10029 CAR-T cells using cGMP manufacturing and quality protocols in preparation of a MC10029 CAR-T cell Phase I clinical trial.
<title>Parameter estimation for process control with neural networks</title> (1991)

An application of neural networks to the problem of parameter estimation for process systems is described. Neural network parameter estimators for a given parametrized model structure can be developed by supervised learning. Training examples can be dynamically generated using a process simulation, resulting in trained networks that are capable of high generalization. This approach can be used for a variety of parameter estimation applications. A proof-of-concept open-loop delay estimator is described, and extensive simulation results detailed. Some results of other parameter estimation networks are also given. Extensions to recursive and closed-loop identification and application to higher-order processes are discussed.
Multipolar corneal-shaping electrode (1981)

The disclosure relates to a multipolar probe using radiofrequency energy to reshape the cornea of an eye. The surface of the cornea is flushed continuously with a conductive coolant during operation.
An all-silicon single-wafer fabrication technology for precision microaccelerometers (2002)

This paper reports a novel all-silicon single-wafer fabrication technology for high precision capacitive accelerometers. This technology combines both surface and bulk micromachining to attain a large proofmass, controllable/small damping, and a small airgap for large capacitance variation. The microfabrication process provides large proofmass by using the whole wafer thickness, and a large sense capacitance by utilizing a thin sacrificial layer. The sense/feedback electrodes are formed by a deposited 2 /spl mu/m polysilicon film with embedded 20-30 /spl mu/m thick vertical stiffeners. These electrodes, while thin, are made very stiff by the thick embedded stiffeners so that force rebalancing of the proofmass becomes possible. The polysilicon electrodes are patterned to create damping holes. Several prototype microaccelerometers are fabricated successfully. Sensitivity of the devices with 2 mm/spl times/1 mm proofmass and full-bridge support are measured to be 2pF/g.
Proton spectroscopy without water suppression: The oversampled <i>J</i>‐resolved experiment (1998)

A method is introduced for obtaining proton spectra in vivo with all the advantages of a full water signal. The method, based on F1 oversampled J-resolved spectroscopy, makes it possible to separate metabolite signals from unwanted baseline artifacts. The dominant water resonance is used as a 2D reference signal for the phase-sensitive reconstruction of the 2D J-resolved metabolite spectra. The powerful specificity of this method is demonstrated with model compound spectra, phantoms, and in vivo examples.
Sound source separation by using matched beamforming and time-frequency masking (2010)

This paper proposes a two-stage algorithm to separate two sound sources by using matched beamforming and time-frequency masking techniques. At first, beamforming was used to separate the sound mixtures back to the original sources while preserving the original contents to the maximum extent. The residual interference was then suppressed by the time-frequency masking technique. A sequential least squares method was used in developing a matched beamformer to estimate the relative transfer function (RTF). From experimental results, it has been shown that the proposed method exhibits improved performance in sound source separation compared to conventional methods. Signal enhanced factor (SEF) was improved by an average of 8.39 dB over the baseline.
Narrowband emission line imaging spectrometry using Savart plates (2016)

Polarization spatial heterodyne interferometry (PSHI) allows for the development of compact, vibration insensitive, high spectral resolution sensors. Introducing the imaging qualities of a lenslet array extends the advantages of PSHI to imaging interferometers. The use of Savart plates enables a birefringent interferometer that obtains higher spectral resolution with fewer optical aberrations when compared to alternative designs. In this paper, we describe the design, construction, calibration and validation of a narrowband emission line imaging spectrometer (NELIS), based on Savart plates and liquid crystal polarization gratings, along with its associated theoretical model. This sensor is advantageous for spectral imaging in the areas of remote sensing, biomedical imaging and machine vision.
Design of a Fiber Optic Read/Write Head for a Military Optical Disk System (1987)

As the speed and capacity of advanced military computers increase, there is a growing need for fast high density memory systems. This report will detail work done at Sperry to develop a novel read/write head which will be used in a Write Once Read Many (WORM) optical disk memory system. The disk system will be used in a digital mapping unit for a tactical fighter aircraft which has extreme operating conditions.
New linear transforms for data on a Fourier 2-sphere with application to diffusion MRI (2012)

This paper describes a new family of linear transforms for data restricted to the surface of a 2-sphere in three-dimensional Fourier space. These transforms generalize the existing Funk-Radon Transform, which has previously been used with great success to extract microstructural tissue orientation information from high angular resolution magnetic resonance diffusion imaging data. Several properties of the new transforms are described, and computationally efficient implementations are derived using spherical harmonic basis functions. A special case from this family, called the FunkRadon and Cosine Transform, is introduced and evaluated. The method is illustrated with simulated and real diffusion weighted MRI data.
Enantioselective Recognition of Ammonium Carbamates in a Chiral Metal–Organic Framework (2017)

Chiral metal-organic frameworks have attracted interest for enantioselective separations and catalysis because of their high crystallinity and pores with tunable shapes, sizes, and chemical environments. Chiral frameworks of the type M<sub>2</sub>(dobpdc) (M = Mg, Mn, Fe, Co, Ni, Zn; dobpdc<sup>4-</sup> = 4,4'-dioxidobiphenyl-3,3'-dicarboxylate) seem particularly promising for potential applications because of their excellent stability, high internal surface areas, and strongly polarizing open metal coordination sites within the channels, but to date these materials have been isolated only in racemic form. Here, we demonstrate that when appended with the chiral diamine trans-1,2-diaminocyclohexane (dach), Mg<sub>2</sub>(dobpdc) adsorbs carbon dioxide cooperatively to form ammonium carbamate chains, and the thermodynamics of CO<sub>2</sub> capture are strongly influenced by enantioselective interactions within the chiral pores of the framework. We further show that it is possible to access both enantiomers of Mg<sub>2</sub>(dobpdc) with high enantiopurity (≥90%) via framework synthesis in the presence of varying quantities of d-panthenol, an inexpensive chiral induction agent. Investigation of dach-M<sub>2</sub>(dobpdc) samples following CO<sub>2</sub> adsorption-using single-crystal and powder X-ray diffraction, solid-state nuclear magnetic resonance spectroscopy, and density functional theory calculations-revealed that the ammonium carbamate chains interact extensively with each other and with the chiral M<sub>2</sub>(dobpdc) pore walls. Subtle differences in the non-covalent interactions accessible in each diastereomeric phase dramatically impact the thermodynamics of CO<sub>2</sub> adsorption.
Insights on the Mechanism for Ethylene Tetramerization (2022)

Selective tetramerization of ethylene to produce 1-octene may be viewed as an extension of the well-studied process for ethylene trimerization to form 1-hexene, which is commercially practiced by Chevron Phillips Chemical (CPChem) and others. The tetramerization reaction was first reported by researchers at SASOL in 2004, but CPChem and others have also reported proprietary systems. Tetramerization reactions are always coupled with some degree of 1-hexene formation, with 1-octene:1-hexene ratios typically varying from 1:2 to 4:1. These mixtures imply the intertwining of the tetramerization and trimerization catalytic cycles, and the primary products are always formed with two types of coproducts. These coproducts include cotrimer and cotetramer C10–C14 species resulting from product reincorporation, as well as other byproducts ranging from C2 species to waxes, and various cyclic hydrocarbons. Gas chromatography (GC) is used to deconvolute these overlapping catalytic cycles, and a model for using GC data to explain the ethylene efficiency in each cycle is presented.
Automatic detection of diseased regions in knee cartilage (2007)

Osteoarthritis (OA) is a degenerative joint disease characterized by articular cartilage degradation. A central problem in clinical trials is quantification of progression and early detection of the disease. The accepted standard for evaluating OA progression is to measure the joint space width from radiographs however; there the cartilage is not visible. Recently cartilage volume and thickness measures from MRI are becoming popular, but these measures don't account for the biochemical changes undergoing in the cartilage before cartilage loss even occurs and therefore are not optimal for early detection of OA. As a first step, we quantify cartilage homogeneity (computed as the entropy of the MR intensities) from 114 automatically segmented medial compartments of tibial cartilage sheets from Turbo 3D T<sub>1 </sub>sequences, from subjects with no, mild or severe OA symptoms. We show that homogeneity is a more sensitive technique than volume quantification for detecting early OA and for separating healthy individuals from diseased. During OA certain areas of the cartilage are affected more and it is believed that these are the load-bearing regions located at the center of the cartilage. Based on the homogeneity framework we present an automatic technique that partitions the region on the cartilage that contributes to maximum homogeneity discrimination. These regions however, are more towards the noncentral regions of the cartilage. Our observation will provide valuable clues to OA research and may lead to improving treatment efficacy.
The Ion Conveyor. An Ion Focusing and Conveying Device (2004)

The control and transmission of ions or small charged droplets in the intermediate to high-pressure regime is of primary importance in areas such as atmospheric pressure ionisation. Where small apertures separate differentially pumped vacuum regions in the inlet systems to mass spectrometers, a large proportion of the available ion current is lost to the surrounding electrode structures. A new ion-optical device, named the ion conveyor, incorporating electrodynamic focusing and conveying of charged entities is described. Results from ion-optical simulations are presented demonstrating the performance of the device in various operating modes and electrode configurations.
Compact Apparatus For Growth Of Protein Crystals (1991)

Compact apparatus proposed specifically for growth of protein crystals in microgravity also used in terrestrial laboratories to initiate and terminate growth at prescribed times automatically. Has few moving parts. Also contains no syringes difficult to clean, load, and unload and introduces contaminant silicon grease into crystallization solution. After growth of crystals terminated, specimens retrieved and transported simply.
Serologic markers in inflammatory bowel disease (IBD). (2001)

Inflammatory bowel disease (IBD) is a generic term that refers to Crohn's disease and ulcerative colitis. Crohn's disease (CD) is a granulomatous enteritis which can involve the ileum, colon, and other parts of the intestinal tract. The serologic responses seen in Crohn's disease include antibodies to Saccharomyces cerevisiae, mycobacteria, bacteroides, listeria and E. coli. Many of these organisms may be involved in the pathogenesis of the Crohn's disease. Ulcerative colitis is characterized by inflammation of the mucosa and submucosa of the large intestine. The CD and UC are considered to be distinct forms of IBD; however, there is a subgroup of CD with a UC-like presentation. In recent years, several serologic markers have been found to be useful for the diagnosis and differentiation of CD and UC. These markers include the following antibodies (a) 2pANCA, (b) ASCA, (c) pancreatic antibody, and (d) OmpC antibody. The application of a panel of markers with the use of an algorithm can identify specific subtypes of IBD that have different clinical courses and progression of the diseases. The application of the serologic markers is useful for diagnosis and management of CD and UC patients.
A squaraine-linked metalloporphyrin two-dimensional polymer photocatalyst for hydrogen and oxygen evolution reactions (2019)

Efficient water splitting photocatalysts are an energetically demanding and cost-effective method for generating renewable energy. Significant research has been reported to advance this approach. However, the use of organic photocatalysts and the presence of residual catalysts trapped in the porous frameworks present major concerns about the efficiency of this strategy. Herein, we report the photocatalytic evolution of H2 and O2 by a multi-hydroxyl group-decorated metalloporphyrin-based two-dimensional catalyst developed via metal catalyst-free synthetic route. Though metalloporphyrins have long been used for catalytic functions, a heterogeneous photocatalyst delivering both H2 and O2 has not yet been realized. This polymer catalyst design enables the photocatalytic diatomic O2 release, a bottleneck in water splitting, in a facile way. Photocatalytic release of H2 as well as O2 occurs with long-term durability of 20 cycles in 300 days with negligible decrease in efficiency, thus demonstrating the excellent performance of this new catalyst.