gebhart/patrepeval huggingface dataset:
Acid ceramidase involved in pathogenic cascade leading to accumulation of α-synuclein in iPSC model ofGBA1-associated Parkinson’s disease (2023)

Bi-allelic mutations in GBA1, the gene that encodes β-glucocerebrosidase (GCase), cause Gaucher disease (GD), whereas mono-allelic mutations do not cause overt pathology. Yet mono- or bi-allelic GBA1 mutations are the highest known risk factor for Parkinson's disease (PD). GCase deficiency results in the accumulation of glucosylceramide (GluCer) and its deacylated metabolite glucosylsphingosine (GluSph). Brains from patients with neuronopathic GD have high levels of GluSph, and elevation of this lipid in GBA1-associated PD has been reported. To uncover the mechanisms involved in GBA1-associated PD, we used human induced pluripotent stem cell-derived dopaminergic (DA) neurons from patients harboring heterozygote mutations in GBA1 (GBA1/PD-DA neurons). We found that compared with gene-edited isogenic controls, GBA1/PD-DA neurons exhibit mammalian target of rapamycin complex 1 (mTORC1) hyperactivity, a block in autophagy, an increase in the levels of phosphorylated α-synuclein (129) and α-synuclein aggregation. These alterations were prevented by incubation with mTOR inhibitors. Inhibition of acid ceramidase, the lysosomal enzyme that deacylates GluCer to GluSph, prevented mTOR hyperactivity, restored autophagic flux and lowered α-synuclein levels, suggesting that GluSph was responsible for these alterations. Incubation of gene-edited wild type (WT) controls with exogenous GluSph recapitulated the mTOR/α-synuclein abnormalities of GBA1/PD neurons, and these phenotypic alterations were prevented when GluSph treatment was in the presence of mTOR inhibitors. We conclude that GluSph causes an aberrant activation of mTORC1, suppressing normal lysosomal functions, including the clearance of pathogenic α-synuclein species. Our results implicate acid ceramidase in the pathogenesis of GBA1-associated PD, suggesting that this enzyme is a potential therapeutic target for treating synucleinopathies caused by GCase deficiency.
Diversity-Oriented Synthesis of Functionalized Pyrrolo[3,2-d]pyrimidines with Variation of the Pyrimidine Ring Nitrogen Substituents (2003)

Nine 2,4-dioxo-2,3,4,5-tetrahydro-1H-pyrrolo[3,2-d]pyrimidine-6-carboxylic acid benzyl esters 12 were synthesized in four steps from 4-oxo-N-(PhF)proline benzyl ester 7 by a general method in which elements of molecular diversity were readily added onto the pyrimidine nitrogens. Conversion of 4-oxoproline 7 into the corresponding aminopyrrole 8 using benzyl-, allyl-, and isopropylamine followed by treatment with phenyl, allyl, and ethyl isocyanate gave nine different ureas 9. 4-Ureido-1H-pyrrole-2-carboxylic acid benzyl esters 9 were then converted into the respective pyrrolo[3,2-d]pyrimidines 12 using trichloroacetyl chloride in acetonitrile followed by treatment with Cs(2)CO(3). Crystallization from toluene gave the desired deazapurines in 37-55% overall yield from proline 7.
In Vivo Evaluation of Functionalized Biomimetic Hydroxyapatite for Local Delivery of Active Agents (2011)

This study was carried out to investigate the biological response in vivo to biomimetic hydroxyapatite implant coatings functionalized with bisphosphonates and bone morphogenetic proteins. The functionalization was carried out by a simple soaking procedure in the operating room immediately prior to surgery. Cylindrical titanium samples with and without coatings were implanted in the distal femoral epiphysis of sheep and retrieved after 6 weeks. The histological analysis proved that all samples were integrated well in the tissue with no signs of intolerance. Fewer osteoclasts were observed in the vicinity of bisphosphonate-functionalized samples and the bone was denser around these samples compared to the other samples. Samples functionalized with bone morphogenetic protein induced more bone/implant contact but showed a more inconsistent outcome with reduced bone density around the samples. This study demonstrates a simple method to functionalize implant coatings, which provides surgeons with an option of patient-specific functionalization of implants. The observed biological impact due to the delivery of active molecules from the coatings suggests that this strategy may also be employed to deliver antibiotics from similar coatings.
Dataset for Back-end-of-line a-SiOxCy:H dielectrics for resistive memory (2018)

Dataset of figures in the paper Fan, J., Kapur, O., Huang, R., De Groot, C., &amp; Jiang, L. (2018). Back-end-of-line a-SiOxCy:H dielectrics for resistive memory. AIP Advances. This dataset including XPS on a-SiOxCy:H films and current-voltage measurements tests on W/a-SiOxCy:H/Cu resistive memories.
Proportion of idiopathic pulmonary fibrosis risk explained by known genetic loci (2020)

Abstract Genome-wide association studies have identified 14 genetic loci associated with susceptibility to idiopathic pulmonary fibrosis (IPF), a devastating lung disease with poor prognosis. Of these, the variant with the strongest association, rs35705950, is located in the promoter region of the MUC5B gene and has a risk allele (T) frequency of 30-35% in IPF cases. Here we present estimates of the proportion of disease liability explained by each of the 14 IPF risk variants as well as estimates of the proportion of cases that can be attributed to each variant. We estimate that rs35705950 explains 5.9-9.4% of disease liability, which is much lower than previously reported estimates. Of every 100,000 individuals with the rs35705950_GG genotype we estimate 30 will have IPF, whereas for every 100,000 individuals with the rs35705950_GT genotype 152 will have IPF. Quantifying the impact of genetic risk factors on disease liability improves our understanding of the underlying genetic architecture of IPF and provides insight into the impact of genetic factors in risk prediction modelling.
Requirement of Nuclear Factor-κB in Angiotensin II– and Isoproterenol-Induced Cardiac Hypertrophy In Vivo (2005)

These results indicate that targeted inhibition of NF-kappaB in cardiomyocytes in vivo is sufficient to impair Ang II- and isoproterenol-induced hypertrophy without increasing the susceptibility to apoptosis.
Simulation of the SEMATECH gravimetric mass flowmeter calibrator (1994)

Many different processes in the fabrication of microelectronic circuits require precise metering of process gas flows. A facility is being designed at Oak Ridge National Laboratory (ORNL) to provide calibration of high-precision gas flowmeters for the Semiconductor Manufacturing Technology (SEMATECH) consortium. To provide proof-of-principle and to aid in the design of the facility, a digital computer simulation was developed. The gravimetric mass flowmeter is a new and unique apparatus that has been proposed for use as a primary calibration standard. The basic operating principles suggest that system dynamics and control will influence measurement accuracy and precision. Hence, a simulation of high fidelity was required because of the precision and accuracy specifications of the proposed facility. This paper describes the mathematical model development and software implementation using the Advanced Continuous Simulation Language (ACSL). Results obtained establish the feasibility of the proposed calibration facility.
Towards Secure Cloud Computing Architecture - A Solution Based on Software Protection Mechanism. (2011)

Cloud computing grows as an important keyword to accelerate IT businesses. A feature of cloud computing is distributed architecture based on unfixed nodes, and security risks have been high-lighted for cloud computing services. In this paper, we present a novel approach for solving the essential issue of cloud computing: how to protect a program running in an untrusted cloud comput-ing environment. We apply a separation technique to the program and divide it into two pieces: a user program and a protected program. Our security analysis shows that both internal and external attacks require exponential computational costs; that is, our scheme is computationally secure against these attacks. 1
The effect of long-term ruxolitinib treatment on JAK2p.V617F allele burden in patients with myelofibrosis (2015)

The JAK2 c.1849G>T (p.V617F) mutation leads to constitutive activation of Janus kinase (JAK)2 and contributes to dysregulated JAK signaling in myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET). In the phase 3 Controlled Myelofibrosis Study with Oral JAK Inhibitor Treatment-I trial, patients with MF, post-PV MF, or post-ET MF achieved significant reductions in splenomegaly and improvements in symptoms with ruxolitinib vs placebo at week 24. This long-term follow-up analysis was performed to determine whether ruxolitinib therapy altered the JAK2p.V617F allele burden in JAK2p.V617F-positive patients. Assessments at baseline and weeks 24, 48, 120, 144, 168, and 216 demonstrated reductions in allele burden from baseline with ruxolitinib treatment that correlated with spleen volume reductions. Of 236 JAK2p.V617F-positive patients analyzed, 20 achieved partial and 6 achieved complete molecular responses, with median times to response of 22.2 and 27.5 months, respectively. Allele burden reductions were greater in patients with shorter disease duration, which suggests a potential benefit of earlier treatment. This trial was registered at www.clinicaltrials.gov as #NCT00952289.
Investigation of Protein-tyrosine Phosphatase 1B Function by Quantitative Proteomics (2008)

Because of their antagonistic catalytic functions, protein-tyrosine phosphatases (PTPs) and protein-tyrosine kinases act together to control phosphotyrosine-mediated signaling processes in mammalian cells. However, unlike for protein-tyrosine kinases, little is known about the cellular substrate specificity of many PTPs because of the lack of appropriate methods for the systematic and detailed analysis of cellular PTP function. Even for the most intensely studied, prototypic family member PTP1B many of its physiological functions cannot be explained by its known substrates. To gain better insights into cellular PTP1B function, we used quantitative MS to monitor alterations in the global tyrosine phosphorylation of PTP1B-deficient mouse embryonic fibroblasts in comparison with their wild-type counterparts. In total, we quantified 124 proteins containing 301 phosphotyrosine sites under basal, epidermal growth factor-, or platelet-derived growth factor-stimulated conditions. A subset of 18 proteins was found to harbor hyperphosphorylated phosphotyrosine sites in knock-out cells and was functionally linked to PTP1B. Among these proteins, regulators of cell motility and adhesion are overrepresented, such as cortactin, lipoma-preferred partner, ZO-1, or p120ctn. In addition, regulators of proliferation like p62DOK or p120RasGAP also showed increased cellular tyrosine phosphorylation. Physical interactions of these proteins with PTP1B were further demonstrated by using phosphatase-inactive substrate-trapping mutants in a parallel MS-based analysis. Our results correlate well with the described phenotype of PTP1B-deficient fibroblasts that is characterized by an increase in motility and reduced cell proliferation. The presented study provides a broad overview about phosphotyrosine signaling processes in mouse fibroblasts and, supported by the identification of various new potential substrate proteins, indicates a central role of PTP1B within cellular signaling networks. Importantly the MS-based strategies described here are entirely generic and can be used to address the poorly understood aspects of cellular PTP function.
A 48 kframe/s CMOS image sensor for real-time 3-D sensing and motion detection (2001)

A CMOS image sensor with 192/spl times/124 pixels realizes video-rate range sensing with 500 /spl mu/m depth resolution, motion detection, and 12 b digital image output. Each pixel consists of four current copier cells as a frame memory block and a chopper comparator. The imager operates at 48 kframes/s maximum rate and dissipates 1.6 W for range sensing and 2 W for digital imaging at 3.3 V in a 0.35 /spl mu/m process.
Feeding Children's Desires? Child and Parental Perceptions of Food Promotion to the under-8's (2010)

Purpose – Extant research downplays the influence of children under the age of eight on food-related decision making and consumption within families. This paper seeks to address this issue. Design/methodology/approach – Utilising novel techniques to elicit responses, the research employed focus group and interviews of a sample of children aged between three and eight years and a sample of their parents. Findings – The exploratory findings of this study suggest that younger children apply effective if less sophisticated pestering techniques than older children, and play a significant role in determining family food consumption. They demonstrate a purposeful and directed pursuit of food brands and products, along with an awareness of the purpose of promotion and a desire to use a number of persuasive techniques in their dealings with parents. This contradicts some of the existing thinking that younger children in the 3-8 year age group have little/less influence on purchasing food. Originality/value – This research offers a number of contributions in that it presents the views of both children and parents, and uses novel techniques through visual representations of feelings and emotions to elicit findings.
A Chlamydia trachomatis VD1-MOMP vaccine elicits cross-neutralizing and protective antibodies against C/C-related complex serovars (2021)

Ocular and urogenital infections with Chlamydia trachomatis (C.t.) are caused by a range of different serovars. The first C.t. vaccine in clinical development (CTH522/CAF®01) induced neutralizing antibodies directed to the variable domain 4 (VD4) region of major outer membrane protein (MOMP), covering predominantly B and intermediate groups of serovars. The VD1 region of MOMP contains neutralizing B-cell epitopes targeting serovars of the C and C-related complex. Using an immuno-repeat strategy, we extended the VD1 region of SvA and SvJ to include surrounding conserved segments, extVD1<sup>A</sup> and extVD1<sup>J</sup>, and repeated this region four times. The extVD1<sup>A</sup>*4 was most immunogenic with broad cross-surface and neutralizing reactivity against representative members of the C and C-related complex serovars. Importantly, in vitro results for extVD1<sup>A</sup>*4 translated into in vivo biological effects, demonstrated by in vivo neutralization of SvA and protection/cross-protection against intravaginal challenge with both SvA and the heterologous SvIa strain.
The Effects of Nuclear Magnetic Resonance Imagers on External and Implantable Pulse Generators (1984)

This study evaluates the effect of nuclear magnetic resonance (NMR) scanning on pacemaker function. It must be emphasized that each manufacturer's pulse generators and each pacing modality may behave differently and, therefore, require individual evaluation. According to our results, patients with pacemakers should have their pacing activity monitored continuously during scanning with the NMR 1500 gauss imaging system. External pulse generators should be set to the asynchronous mode and placed outside the NMR image volume but within the radiofrequency (RF) shield. Implanted pacemakers should be verified for type and mode of operation. All implantable pulse generators evaluated reverted from the demand to the asynchronous mode within the magnetic field of the scanner. There was no observable damage to the discrete pacemaker components that were tested. In vivo testing of implantable single-chamber pulse generators did not significantly alter the pacemaker's operating parameters. Changes in stimulation rate analogous to the RF field pulse rate were seen. In single-chamber devices the resultant rate was a multiple of the RF frequency, changing to a value less than the normal asynchronous magnetic rate. With more sophisticated dual-chamber devices the results varied. With VDD pacing during RF scanning, the cardiac stimulation rate increased to a value analogous to the RF field modulation period. More extensive in vivo testing using different models of pulse generators of various manufacturers is needed to identify specific device susceptibility to the RF, time variance and steady-state magnet fields. From these data a comprehensive statement about NMR scanning of patients with implanted pacemakers can be made.
Reference governor design for computationally efficient attitude and tether tension constraint enforcement on a lighter-than-air wind energy system (2013)

In this paper, we propose a reference governor-based approach to guarantee enforcement of critical flight constraints on the Altaeros tethered, lighter-than-air wind energy system. While the high-altitude flight made available by the tethered system leads to significant increases in power production over traditional, tower-mounted systems, the freedom of motion resulting from the tethers and aerodynamic shell introduces critical attitude and tether tension constraints. To date, methods considered for enforcing these constraints have relied upon heuristic static maps or model predictive control (MPC). The former cannot guarantee transient constraint satisfaction, whereas the latter is computationally burdensome given Altaeros's current microcontroller capabilities. The approach pursued in this paper uses a reference governor, which is a computationally simple add-on to the existing controller that enforces transient and steady-state constraints. The methodology proposed in this paper is demonstrated through simulations on linear and nonlinear models of the longitudinal dynamics of the Altaeros system with wind gust disturbances.
Suppression of adaptive immune responses during primary SIV infection of sabaeus African green monkeys delays partial containment of viremia but does not induce disease (2010)

One of the most puzzling observations in HIV research is the lack of pathogenicity in most nonhuman primate species that are natural hosts of simian immunodeficiency virus (SIV) infection. Despite this, natural hosts experience a level of viremia similar to humans infected with HIV or macaques infected with SIV. To determine the role of adaptive immune responses in viral containment and lack of disease, we delayed the generation of cellular and humoral immune responses by administering anti-CD8- and anti-CD20 lymphocyte-depleting antibodies to sabaeus African green monkeys (Chlorocebus sabaeus) before challenge with SIV(sab9315BR). In vivo lymphocyte depletion during primary infection resulted in a brief elevation of viremia but not in disease. Based on the magnitude and timing of SIV-specific CD8(+) T-cell responses in the lymphocyte-depleted animals, CD8(+) T-cell responses appear to contribute to viral containment in natural hosts. We found no evidence for a contribution of humoral immune responses in viral containment. These studies indicate that natural hosts have developed mechanisms in addition to classic adaptive immune responses to cope with this lentiviral infection. Thus, adaptive immune responses in natural hosts appear to be less critical for viral containment than in HIV infection.
Tailoring the functional profile of niobium carbide (NbC) as cutting tool materials and for wear protection (2018)

The present work deals with Niobcarbid (NbC). Strategic reflections on tungsten carbide and more and more stringent toxicological restrictions for cobalt associated for both with spiraling stock market prices have attracted recently some attention for Niobium carbide as a substitute for tungsten carbide in machining.
Multidimensional fluorescence spectroscopy of single molecules (2001)

Multichannel-detection for simultaneous registration of all information on a signal burst (intensity, lifetime, anisotropyr and spectral range) is used for analytics and studies of conformational dynamics of a single fluorescent labeled molecule in solution.
GIS Integrated Automation of a Near Real-Time Power-Flow Service for Electrical Grids (2018)

Electrical grid's modernization and the need for sustainable sources of energy lead to changes in physical infrastructure and subsequent operations of the grid, such as penetration of renewables and local power generation from them. There is a shift in paradigm from remotely concentrated generation toward distributed generations that are local to the loads. To meet the objective of sustainability, the local generations must include renewable energy conversions. This requires power-flow management of power variability arising from the spatial and temporal characteristics of the renewables like solar energy. Such management must require least human intervention, and it is achievable through fast and reliable automations. This paper presents geographic information system integrated automation of a noniterative power-flow method known as holomorphic embedded power flow (HEPF), thereby providing local grids the ability to manage themselves. Since HEPF reliably guarantees the solution and reaches it very fast through constructing the solution, it is an ideal candidate for near real-time updates of the electrical states of a power system. The role of the HEPF as a key enabler for the services of a power-flow manager, that performs optimizations for a cluster of buildings, is then illustrated.
Correction to: Multiscale imaging of therapeutic anti-PD-L1 antibody localization using molecularly defined imaging agents Iris (2022)

Following publication of the original article [1], the authors identified an error in Fig.
Venus Corona and Tessera Explorer (VeCaTEx) (2021)

Venus Corona and Tessera Explorer (VeCaTEx) would use an aerobot to descend repeatedly beneath the dense clouds for imaging targeted area of the surface in the near infrared to address six of the prime investigations prioritized by VEXAG. The technologies needed could be matured during the next decade.
Novel Lifshitz tricritical point and critical dynamics (1985)

The d-dimensional time-dependent Ginzburg-Landau (TDGL) model is mapped onto a special (d+1)-dimensional model which exhibits a Lifshitz tricritical point (LTP). Many of the LTP critical properties follow from those of the TDGL model, and are shown to belong to a novel universality class of LTP's which results from a (previously ignored) relevant, nonlocal, quartic spin operator. These properties are analyzed with the use of scaling, an \ensuremath{\epsilon} expansion, and the n\ensuremath{\rightarrow}\ensuremath{\infty} limit.
3.1 polar antenna impedance detection and tuning for efficiency improvement in a 3G/4G CMOS Power Amplifier (2014)

One of the ultimate goals in power amplifier design is to enhance the effective efficiency and achieve a long battery life. Therefore, both the peak efficiency and the efficiency loss due to antenna impedance mismatch or power back-off are highly critical design issues. In particular, the challenge of the antenna impedance mismatch is becoming more severe, due to the increased frequency band and smaller antenna size. Moreover, the antenna mismatch also changes with time due to the user proximity effect [1].