gebhart/patrepeval huggingface dataset:
X-linked acrogigantism syndrome: clinical profile and therapeutic responses (2015)

X-linked acrogigantism (X-LAG) is a new syndrome of pituitary gigantism, caused by microduplications on chromosome Xq26.3, encompassing the gene GPR101, which is highly upregulated in pituitary tumors. We conducted this study to explore the clinical, radiological, and hormonal phenotype and responses to therapy in patients with X-LAG syndrome. The study included 18 patients (13 sporadic) with X-LAG and microduplication of chromosome Xq26.3. All sporadic cases had unique duplications and the inheritance pattern in two families was dominant, with all Xq26.3 duplication carriers being affected. Patients began to grow rapidly as early as 2-3 months of age (median 12 months). At diagnosis (median delay 27 months), patients had a median height and weight standard deviation scores (SDS) of >+3.9 SDS. Apart from the increased overall body size, the children had acromegalic symptoms including acral enlargement and facial coarsening. More than a third of cases had increased appetite. Patients had marked hypersecretion of GH/IGF1 and usually prolactin, due to a pituitary macroadenoma or hyperplasia. Primary neurosurgical control was achieved with extensive anterior pituitary resection, but postoperative hypopituitarism was frequent. Control with somatostatin analogs was not readily achieved despite moderate to high levels of expression of somatostatin receptor subtype-2 in tumor tissue. Postoperative use of adjuvant pegvisomant resulted in control of IGF1 in all five cases where it was employed. X-LAG is a new infant-onset gigantism syndrome that has a severe clinical phenotype leading to challenging disease management.
PDTB-20. PRECLINICAL EFFICACY OF ALK2 INHIBITORS IN ACVR1 MUTANT DIPG (2016)

We recently reported somatic mutations in ACVR1, encoding the serine/threonine kinase ALK2 receptor, in diffuse intrinsic pontine glioma (DIPG) and have explored its utility as a novel drug target in these incurable childhood brainstem tumours. 50/206 (24%) DIPG patients harboured ACVR1 mutations, with the extended survival times (p=0.0028) and HIST1H3B K27M associations (p=0.046) of this genotype restricted to G328E/V/W substitutions; the common germline variant in fibrodysplasia ossificans progressiva (FOP), R206H, instead frequently co-segregated with H3F3A K27M and a shorter overall survival. This mutation is reported to confer abnormal responsiveness to activin A in FOP, a phenomenon recapitulated in vitro in both R206H and G328V primary patient-derived DIPG models assessed by Western blot for p-Smad1/5/8 and ID1. Unlike classical BMP ligands, the spatiotemporal expression of activin A in neurodevelopment correlates with the suggested origins of DIPG. Screening mutant and wild-type (wt) DIPG cultures with a range of quinazolinone- and pyrazolo [1,5-a] pyrimidine-based ALK2 inhibitors, both published and newly-derived in-house, demonstrated differential effects on cell viability down to sub-micromolar concentrations, recapitulating genetic knockdown with shRNA, and corresponding to sustained time- and concentration-dependent reduction in signaling. Preclinical studies showed LDN-193189 and LDN-214117 to be orally bioavailable and tolerated, with penetration in the CNS at potentially efficacious doses. Treatment of mice bearing orthotopic xenografts of H3F3A K27M, ACVR1 R206H mutant HSJD-DIPG-007 cells with 25mg/kg of LDN-193189 for 28 days significantly reduced tumour burden and extended survival compared to vehicle control (p=0.0023), an effect not observed in an H3F3A/ACVR1 wild-type orthotopic model. Continued development of novel ALK2 inhibitors with increased potency, selectivity and pharmacokinetic properties may play an important role in future trials of DIPG patients.
Is Weight Gain an Unavoidable Consequence of Insulin Therapy? (2007)

Weight gain is often a significant concern among patients with type 2 diabetes. Patients using long-acting insulin analogs, particularly insulin detemir, often have decreased weight gain and sometimes even weight loss following treatment, as well as a reduced incidence of hypoglycemia. These benefits may be due in part to the more predictable action profiles associated with long-acting insulin analogs, as compared with the older basal formulations that are based on human insulin. In this review, the properties of basal insulin analogs will be considered as they relate to weight gain, and several strategies to more effectively manage the problem of insulin-associated weight gain will be discussed. CONFLICT OF INTEREST Bruce W. Bode receives grant/research support, participates in the speaker's bureau program, and is a consultant to Eli Lilly, Novo Nordisk, and Sanofi-Aventis. Dr. Bode would like to thank Novo Nordisk for funding that helped support the preparation of this manuscript.
Glossar zu Begriffen mit Bezug zu Kinetik, Thermodynamik und Mechanismen von Polymerisationen (2009)

Abstract Im Folgenden werden empfohlene Definitionen von grundlegenden Begriffen mit Bezug zu Polymerisationsprozessen vorgestellt. Neuere Entwicklungen im Hinblick auf die Kinetik, die Thermodynamik und die Mechanismen von Polymerisationen machen die Einführung neuer Begriffe und einige Revisionen oder Erweiterungen von Begriffen notwendig, die zuvor im “Compendium of Chemical Terminology” oder im “Glossary of Basic Terms in Polymer Science” definiert wurden.
Collision detection using band designed Disturbance Observer (2012)

In this work, we design frequency band filters in Disturbance Observer (DOB) for collision detection between the industrial robot manipulator and human. We realize that the frequency characteristics of the inverse dynamics of the manipulator is similar to that of high pass filter and the sensor noise filter is in the form of low pass filter whereas the collision between the robot and human is relatively high frequency. By investigating the frequency characteristics of each part of the manipulator and possible disturbances, we proposes a set of band-width filters using intrinsic data and detects the human-robot collision while the robot is in motion for performing some tasks. We verify the effectiveness of the method using two different types of robotic manipulators; electric motor driven manipulator and manipulator with hydraulic rotary actuators. In both cases, experimental data show that the robot-human collision can be detected reliably and faster than simply using a joint torque sensor while the manipulator load is not constant.
Study of the Partial Charge Transport Properties in the Molten Alumina via Molecular Dynamics (2019)

Knowing the charge-transport properties of molten oxides is essential for industrial applications, particularly when attempting to control the energy required to separate a metal from its ore concentrate. Nowadays, in the context of a drastic increase of computational resources, research in industrial process simulation and their optimization is gaining popularity. Such simulations require accurate data as input for properties in a wide range of compositions, temperatures, and mechanical stresses. Unfortunately, due to their high melting points, we observe a severe lack of (reproducible) experimental data for many of the molten oxides. An alternative consists in using molecular dynamic simulations employing nonempirical force fields to predict the charge-transport properties of molten oxides and thus alleviate the lack of experimental data. Here, we study molten alumina using two polarizable force fields, with different levels of sophistication, parameterized on electronic structure calculations only. After validating the models against the experimental sets of density and electrical conductivity, we are able to determine the various ionic contributions to the overall charge transport in a wide range of temperatures.
Green Fluorescent Protein Tag for Studies of Drug-Induced Translocation of Nucleolar Protein RH-II/Gu (1998)

We have constructed a human osteogenic sarcoma cell line, U-2 OS/GFP-Gu, that expresses nucleolar RNA helicase RH-II/Gu tagged with green fluorescent protein (GFP). The presence of a GFP tag does not inhibit RNA helicase, RNA folding and ATPase activities of RH-II/Gu protein. The derived cell line responds to cytotoxic agents like the parental cell line U-2 OS. In the presence of either actinomycin D or toyocamycin, the GFP-RH-II/Gu fusion protein translocates from the nucleolus to the nucleoplasm in the same way as the translocation of endogenous RH-II/Gu. The drug-induced translocation of GFP-RH-II/Gu is easily monitored by direct observation of live cells in vivo. This cell line can be used to screen cytotoxic drugs and to study the mechanisms of drug-induced translocation of RH-II/Gu. The cellular localization of RH-II/Gu during the cell cycle-dependent formation of the nucleolus is readily monitored. Real-time results are obtained more quickly without the disadvantages associated with cell fixation and immunofluorescence-based staining.
THE GENETIC ANALYSIS OF MAMMALIAN CELL-CYCLE MUTANTS (1985)

We have made rapid progress in recent years in identifying the genetic causes of many human diseases. However, despite this recent progress, our mechanistic understanding of these diseases is often incomplete. This is a problem because it limits our ...Read More
MAGNETIC SATURATION AND ANISOTROPY OF La2C07, PrZCo7, AND Nd2C07 (1977)

Room-temperature lattice parameters, density, saturation magnetization and magneto-crystalline anisotropy data are reported for the intermetallic compounds La2C07, Pr2C07, and Ndfo7. Powder x-ray techniques were used to determlne lattice constants and the easy direction of magnetization. Saturation values and anisotropy constants were derived from c-axis and basal-plane moment vs. field measurements on small single-crystal fragments of arc-melted and annealed alloy buttons.
Quantitative evaluation of nanoparticle classification by size-exclusion chromatography (2018)

Although few attempts for classification of nanoparticles (NPs) in labscale do exist, the transfer to industrial applications is still challenging. One promising separation method, which is already established for biological molecules, is chromatography. Herein, we study the classification of differently sized gold NPs (AuNPs) by size-exclusion chromatography (SEC). First, we investigated the interactions of AuNPs with potential stationary phases in order to identify a suitable material for the chromatographic process where irreversible NP adhesion is excluded. Then, we demonstrate the high reproducibility of our SEC experiments by multiple sample injections that lead to constant peak areas. In particular, we show the size-dependent elution behavior of AuNP mixtures resulting in bimodal elution peaks, where size separation was confirmed by inline measured UV/Vis spectra. Finally, NP classification results by using a fraction collector are characterized by retention time, mass balances and size-dependent separation efficiencies. The adjustment of the particle size distributions (PSDs) is demonstrated by changing the switching time of the fraction collector. Our study evidences the high potential of SEC for preparative and continuous separation of NPs.
Systems-Level Performance Estimation of a Pulse Detonation Based Hybrid Engine (2006)

A key application for a Pulse Detonation Engine concept is envisioned as a hybrid engine, which replaces the combustor in a conventional gas turbine with a Pulse Detonation Combustor (PDC). A limit cycle model, based on quasi 1-D, unsteady Computational Fluid Dynamics (CFD) simulations, was developed to estimate the performance of a pressure-rise PDC in a hybrid engine to power a subsonic engine core. The parametric space considered for simulations of the PDC operation includes the mechanical compression or the flight conditions that determine the inlet pressure and the inlet temperature conditions, fill fraction and purge fraction. The PDC cycle process time scales including overall operating frequency were determined via limit-cycle simulations. The methodology for estimation of performance of the PDC considers the unsteady effects of PDC operation. These metrics include a ratio of time-averaged exit total pressure to inlet total pressure and a ratio of mass-averaged exit total enthalpy to inlet total enthalpy. This information can be presented as a performance map for the PDC, which was then integrated into a systems-level cycle analysis model, using Gate-Cycle, to estimate the propulsive performance of the hybrid engine. Three different analyses were performed. The first was a validation of the model against published data for specific impulse. The second examined the performance of a PDC versus a traditional Brayton cycle for a fixed combustor exit temperature; the results show an increased efficiency of the PDC relative to the Brayton cycle. The third analysis performed was a detailed parametric study varying engine conditions to examine the performance of the hybrid engine. The analysis has shown that increasing the purge fraction, which can reduce the overall PDC exit temperature, can simultaneously provide small increases in overall system efficiency.
Synthesis of LaPO4:Eu Nanostructures Using the Sol−Gel Template Method (2008)

The sol−gel template synthesis method was used to investigate size-induced changes in luminescence properties of Eu3+-doped LaPO4 materials. Both untemplated bulk powders as well as templated nanotubes ranging in size from 20 to 200 nm were synthesized. These materials were dried at 70 °C or dried and sintered at 650 °C. Powder X-ray diffraction data indicate that the untemplated dried bulk powder is a mix of hexagonal and monazite phases, whereas the dried and sintered bulk powder is a pure monazite phase. When the LaPO4:Eu material is templated, nanotubes are formed that reflect the dimensions of the template membrane. Luminescence data indicate the Eu3+ ion substitutes for the La3+ ion in the LaPO4 host lattice for both the bulk powders and the nanotubes. Notably, as the diameter of the tube decreases, the spectra broaden and the background signal increases significantly. This is likely the result of increased defects in smaller particles that have a larger surface-to-volume ratio.
FANCI is a negative regulator of Akt activation (2016)

Akt is a critical mediator of the oncogenic PI3K pathway, and its activation is regulated by kinases and phosphatases acting in opposition. We report here the existence of a novel protein complex that is composed minimally of Akt, PHLPP1, PHLPP2, FANCI, FANCD2, USP1 and UAF1. Our studies show that depletion of FANCI, but not FANCD2 or USP1, results in increased phosphorylation and activation of Akt. This activation is due to a reduction in the interaction between PHLPP1 and Akt in the absence of FANCI. In response to DNA damage or growth factor treatment, the interactions between Akt, PHLPP1 and FANCI are reduced consistent with the known phosphorylation of Akt in response to these stimuli. Furthermore, depletion of FANCI results in reduced apoptosis after DNA damage in accord with its role as a negative regular of Akt. Our findings describe an unexpected function for FANCI in the regulation of Akt and define a previously unrecognized intersection between the PI3K-Akt and FA pathways.
Imaging real-space flat band localization in kagome magnet FeSn (2023)

Abstract Kagome lattices host flat bands due to their frustrated lattice geometry, which leads to destructive quantum interference of electron wave functions. Here, we report imaging of the kagome flat band localization in real-space using scanning tunneling microscopy. We identify both the Fe 3 Sn kagome lattice layer and the Sn 2 honeycomb layer with atomic resolution in kagome antiferromagnet FeSn. On the Fe 3 Sn lattice, at the flat band energy determined by the angle resolved photoemission spectroscopy, tunneling spectroscopy detects an unusual state localized uniquely at the Fe kagome lattice network. We further show that the vectorial in-plane magnetic field manipulates the spatial anisotropy of the localization state within each kagome unit cell. Our results are consistent with the real-space flat band localization in the magnetic kagome lattice. We further discuss the magnetic tuning of flat band localization under the spin–orbit coupled magnetic kagome lattice model.
Does ethnicity have an effect on fetal behavior? A comparison of Asian and Caucasian populations (2015)

Our results suggest that ethnicity should be considered when evaluating fetal behavior, especially during assessment of fetal facial expressions. Although there was a difference in the total KANET score between Japanese and Croatian populations, all the scores in both groups were within normal range. Our results indicate that ethnical differences in fetal behaviour do not affect the total KANET score, but close follow-up should be continued in some borderline cases.
Mycobacterium tuberculosis WhiB3: A Novel Iron–Sulfur Cluster Protein That Regulates Redox Homeostasis and Virulence (2011)

How Mtb WhiB3 monitors its internal and external surroundings and modulates endogenous oxido-reductive pathways which in turn alter Mtb signal transduction, nucleic acid and protein synthesis, and enzymatic activation, is mostly unexplored. Modern expression, metabolomic and proteomic technologies should provide fresh insights into these yet unanswered questions.
Correlated nanoelectronics and the second quantum revolution (2022)

The growing field of correlated nanoelectronics exists at the intersection of two established fields: correlated oxide electronics and semiconductor nanoelectronics. The development of quantum technologies that exploit quantum coherence and entanglement for the purposes of computation, simulation, and sensing will require complex material properties to be controlled at nanoscale dimensions. Heterostructures and nanostructures formed at the interface between LaAlO3 and SrTiO3 exhibit striking behavior that arises from the ability to program the conductive behavior at extreme nanoscale dimensions. The active electronic layer, SrTiO3, exhibits a wide range of gate-tunable phenomena such as ferroelectricity, ferroelasticity, magnetism, superconductivity, and spin–orbit coupling, all of which can be controlled at the nanoscale using two reversible methods: conductive atomic force microscope lithography and ultra-low-voltage electron beam lithography. Mesoscopic devices such as single-electron transistors and quasi-one-dimensional electron waveguides can be “sketched” using these techniques, and the properties of these devices differ significantly from those created from traditional semiconductors, such as Si or GaAs. The strongly correlated nature of the SrTiO3 system is evident from superconducting behavior as well as a state in which electrons are paired outside the superconducting state. A highly exotic phase was discovered in which a degenerate quantum liquid is formed from bound states of n = 2, 3, 4, … electrons. Further development of correlated nanoelectronics based on the LaAlO3/SrTiO3 system can potentially lead to a general platform for quantum simulation as well as a pathway for the development of highly entangled states of multiple photons.
Protein Denaturation and Structural Damage During High‐Pressure‐Shift Freezing of Porcine and Bovine Muscle (2000)

ABSTRACT: Pork and beef muscles were subjected to 200 MPa and −20 °C with or without water freezing. Both tissues responded to the treatment with similar behavior. Protein denaturation was greater when freezing occurred. Pressure‐induced cold denaturation was complete for actin and very considerable for myosin and other muscle proteins. Connective proteins remained practically unaltered by pressurization and/or freezing. Structural changes in the muscle at sarcomere levels caused by pressurization were more severe when freezing occurred. Color, drip loss, and textural properties on the pressurized samples also revealed an additional deleterious influence of freezing. Pressurization alone and pressure‐shift freezing resulted unsuitable for muscle preservation.
A methodology for validation of microprocessors using equivalence checking (2004)

As embedded systems continue to face increasingly higher performance requirements, deeply pipelined processor architectures are being employed to meet desired system performance. Validation of such processor architectures is one of the most complex and expensive tasks in the current systems-on-chip design process. A significant bottleneck in the validation of such systems is the lack of a golden reference model. We present an architecture description language (ADL) driven methodology for generating golden reference model. We use EXPRESSION ADL to capture the structure and behavior of the processor. The synthesizable register transfer language (RTL) description of the architecture is generated from the ADL specification. The generated RTL description is used as a golden reference model for verifying the correctness of the implementation using equivalence checking. We applied our methodology on a RISC DLX architecture to demonstrate the usefulness of our approach.
Research on High-accuracy Clock Synchronization Based on IEEE 1588 Protocol (2019)

With the continuous development of mobile Internet3G/4G/5G, the requirement of time synchronization is becoming higher and higher. The IEEE1588 protocol has received extensive attention and research because it can provide sub-microsecond time synchronization to meet the actual needs. This research is based on Kalman Filter(KF) and Sliding Mode Controller(SMC) to achieve master-slave clock synchronization. Firstly, SMC is used to compensate the frequency drift caused by temperature, thereby reducing the influence of temperature on the frequency drift of crystal oscillator. Then, KF is used to suppress and optimize clock frequency jitter or random error. Through simulation, it is proved that this method can effectively improve the synchronization accuracy and meet the clock synchronization requirements of IEEE1588 protocol.
Time-lapse Q-factor tomography by reflected waves’ inversion (2020)

Hydrocarbon production modifies several lithological parameters of a reservoir, as P velocity, density and electrical conductivity, which may have a dominant effect. Anelastic absorption provides a weaker time-lapse signal, but its information may become critical when other parameters are not sensitive enough. We present a new method for estimating the spectral centroid of seismic signals, which is the input for the classical frequency-shift inversion. This approach is validated by a numerical simulation of direct, reflected and head waves that are jointly inverted in a time-lapse framework. Two models are compared that differ only for a local Q-factor anomaly, detected by head waves. This approach can contribute significantly to reservoir characterization and monitoring. Presentation Date: Monday, October 12, 2020 Session Start Time: 1:50 PM Presentation Time: 3:05 PM Location: Poster Station 9 Presentation Type: Poster
Enzymatic Adenylylation by Aminoglycoside 4′-Adenylyltransferase and 2′′-Adenylyltransferase as a Means of Determining Concentrations of Aminoglycoside Antibiotics in Serum (1976)

A fast and accurate enzymatic estimation of the concentration of aminoglycoside antiobiotics in serum is given by aminoglycoside 4′-adenylyltransferase (synonym: tobramycin adenylyltransferase), a new enzyme recently found in tobramycin-resistant staphylococci (P. Santanam and F. H. Kayser, J. Infect. Dis. [Suppl.], in press). This enzyme is useful in assaying the kanamycins, the butirosins, amikacin, ribostamycin and, if required, the neomycins, tobramycin, and paromomycin. In combination with the assay using aminoglycoside 2′′-adenylyltransferase (synonym: gentamicin adenylyltransferase) to determine the level of gentamicin, sisomicin, and tobramycin, a convenient method to estimate a wide range of aminoglycoside antibiotics in patients' sera is described.