gebhart/patrepeval huggingface dataset:
Glycogen Storage Disease Type Ia in Canines: A Model for Human Metabolic and Genetic Liver Disease (2011)

A canine model of Glycogen storage disease type Ia (GSDIa) is described. Affected dogs are homozygous for a previously described M121I mutation resulting in a deficiency of glucose-6-phosphatase-α. Metabolic, clinicopathologic, pathologic, and clinical manifestations of GSDIa observed in this model are described and compared to those observed in humans. The canine model shows more complete recapitulation of the clinical manifestations seen in humans including "lactic acidosis", larger size, and longer lifespan compared to other animal models. Use of this model in preclinical trials of gene therapy is described and briefly compared to the murine model. Although the canine model offers a number of advantages for evaluating potential therapies for GSDIa, there are also some significant challenges involved in its use. Despite these challenges, the canine model of GSDIa should continue to provide valuable information about the potential for generating curative therapies for GSDIa as well as other genetic hepatic diseases.
Comparative in vitro performance of a new re-usable breath-actuated nebulizer (BAN) with other high performance systems intended for domiciliary use - 2: Portable battery-compressor (2012)

RATIONALE: Treatments with home based compressor/nebulizer systems can offer very different delivery characteristics. We evaluated a new, reusable BAN (AeroEclipse-XL®, Trudell Medical International) in breath-actuated mode with its portable (Ombra®) battery-compressor. METHODS: The nebulizer-on-test (n=5/group) was filled with 2.5-mL, 1.0 mg/mL albuterol (Ventolin®, GSK Canada Inc.), and connected to a breathing simulator (ASL5000, IngMar Medical, Pittsburgh, PA) mimicking adult tidal breathing (V t = 600-mL; duty cycle = 33%; rate = 10 cycles/min). Emitted aerosol was captured on a filter at the mouthpiece, replaced at minute intervals until onset of sputtering, defining run time. Recovery/assay of salbutamol was undertaken by HPLC-UV spectrophotometry. Fine droplet fraction (FDF ) was the product of total mass and FDF . Comparative measurements were made with the Sprint® (PARI, Germany) and MicroPlus® (Philips-Respironics, Germany) nebulizers using PARI BOY® Mobile S® and Inspiration Micro Elite® portable compressors respectively. RESULTS: See Table CONCLUSIONS: The BAN/Ombra® system provided highly respirable aerosol with FDM substantially greater than the benchmark systems. Its run time reflects the fact that aerosol is only delivered during inhalation and not wasted to the environment.
Transgenerational epigenetic programming via sperm microRNA recapitulates effects of paternal stress (2015)

Epigenetic signatures in germ cells, capable of both responding to the parental environment and shaping offspring neurodevelopment, are uniquely positioned to mediate transgenerational outcomes. However, molecular mechanisms by which these marks may communicate experience-dependent information across generations are currently unknown. In our model of chronic paternal stress, we previously identified nine microRNAs (miRs) that were increased in the sperm of stressed sires and associated with reduced hypothalamic-pituitary-adrenal (HPA) stress axis reactivity in offspring. In the current study, we rigorously examine the hypothesis that these sperm miRs function postfertilization to alter offspring stress responsivity and, using zygote microinjection of the nine specific miRs, demonstrated a remarkable recapitulation of the offspring stress dysregulation phenotype. Further, we associated long-term reprogramming of the hypothalamic transcriptome with HPA axis dysfunction, noting a marked decreased in the expression of extracellular matrix and collagen gene sets that may reflect an underlying change in blood-brain barrier permeability. We conclude by investigating the developmental impact of sperm miRs in early zygotes with single-cell amplification technology, identifying the targeted degradation of stored maternal mRNA transcripts including sirtuin 1 and ubiquitin protein ligase E3a, two genes with established function in chromatin remodeling, and this potent regulatory function of miRs postfertilization likely initiates a cascade of molecular events that eventually alters stress reactivity. Overall, these findings demonstrate a clear mechanistic role for sperm miRs in the transgenerational transmission of paternal lifetime experiences.
Urban America and the Community College Imperative: The Importance of Open Access and Opportunity (1999)

Abstract Urban community colleges have unique missions geared to providing access and fostering community for the diverse student populations—majority minorities—living today in American cities.
PEROXIDASE-CATALYZED CROSSlINKING OF FUNCTIONALIZED POLYASPARTIC ACID POLYMERS (2002)

ABSTRACT The formation of biodegradable hydrogels is reported based on enzymatic crosslinking reactions of modified polyaspartic acid. Poly(aspartic acid) polymers functionalized with aromatic groups were crosslinked in aqueous solution via peroxidase with hydrogen peroxide to form hydrogels. The reaction products were characterized based on time to gel, swelling ratio, sol fraction, storage and loss modulus, and entrapped enzyme. Optimum reaction conditions for gel synthesis were determined. Poly(aspartic acid) hydrogels have significant potential for use in biomedical applications such as in drug delivery due to the aqueous, non-toxic synthesis conditions as well as their inherent biodegradability by proteases. Keywords: Peroxidase, CrosslinkingHydrogelPolyaspartic acid ACKNOWLEDGMENTS The authors would like to acknowledge Novo Nordisk and Rohm and Haas for their support of this research and for their helpful input during the course of this research. We would also like to thank Ann-Valerie Ruzette and Philip Soo of the A. Mayes group at MIT for their help with DMA analysis. Bhanu Kalra and Ajay Kumar of Polytechnic University are acknowledged for their early contributions towards the synthesis of the functionalized polymers.
A 60GHz CMOS Transceiver IC for a Short-Range Wireless System with Amplitude/Phase Imbalance Cancellation Technique (2012)

A 60GHz direct conversion transceiver which employs amplitude/phase imbalance cancellation technique is newly proposed. By using the proposed technique, the receive path of the transceiver achieves less than 0.2dB of amplitude error and less than 3° of phase error at 60GHz bands over a 10GHz bandwidth, which relaxes the design accuracy required for baluns used in the transceiver. It also employs a simple and fast calibration algorithm to adjust the locking range of the divide-by-3 injection locked divider in the phase locked loop. Fabricated in 90nm CMOS technology, the transceiver achieves a low power consumption of 230mW in transmit mode and 173mW in receive mode. The output spectrum of 1.76Gsps π/2-BPSK/QPSK modulation shows the excellent distortion and spurious suppression that meet the IEEE802.11ad draft standard.
A study of bandwidth-sharing mechanisms in connection-oriented networks (2008)

There is an increasing interest in optical connection-oriented networks to support the high-speed and predictable-service requirements of applications used in the scientific research community. Various experimental testbeds have been created. One area of networking research in these testbed projects is dynamic bandwidth sharing because the number of universities and national research laboratories involved in these scientific projects is large enough to make the use of dedicated circuits (leased lines) prohibitively expensive. 
We propose two new bandwidth-sharing mechanisms, both of which are book-ahead (BA) reservation schemes for high-speed connection-oriented networks. First, we define two types of reservation requests: (i) session-type requests, which specify the required bandwidth and duration, and (ii) data-type requests, which specify the amount of data to be transferred. For session-type requests, we propose two variants: a BA-n scheme, in which a user specifies n acceptable call-initiation time options, and a BA-First scheme, in which a user accepts any call-initiation time. We construct two novel analytical models for these two variants of BA schemes. Using the analytical and simulation models, we show that BA schemes overcome the limitation of the immediate-request (IR) mechanism, making it feasible to support applications even when the per-call bandwidth is large relative to link capacity. 
Our second book-ahead mechanism called Varying-Bandwidth Delayed Start (VBDS) is for data-type (file transfer) requests. By having applications specify file sizes in their reservation requests, a VBDS scheduler is able to assign a varying-bandwidth allocation for different time ranges instead of a single fixed-rate. Through simulations, we show that this approach improves system performance when compared to fixed-bandwidth schemes significantly. 
As a complement to the analytical and simulation studies used in the design of our two proposed BA schemes, we undertake an implementation and experimental study of the IR bandwidth-sharing mode in high-speed connection-oriented networks. We deployed a three-node connection-oriented network called CHEETAH, and implemented a software package to support the IR bandwidth-sharing mode in this network. We obtain measurements for end-to-end circuit setup delays and per-switch signaling message processing delays. These measurements are useful for analytical and simulation studies of connection-oriented networks, and in network design and configurations.
Src stimulates insulin-like growth factor I (IGF-I)-dependent cell proliferation by increasing IGF-I receptor number in human pancreatic carcinoma cells. (1998)

We examined the potential function of Src in human pancreatic carcinoma. Overexpression of kinase-activated SrcY527F resulted in a significant increase of insulin-like growth factor I (IGF-I)-dependent cell proliferation in the cell line PANC-1. Western blotting and competition binding studies demonstrated 2.3 +/- 0.2-fold increase in IGF-I receptor expression and 2.8 +/- 0.4-fold increase in IGF-I-specific binding sites/cell. SrcY527F transfection alone did not change receptor affinity or basal receptor tyrosine phosphorylation, whereas IGF-I-stimulated receptor phosphorylation was increased by 2.1 +/- 0.5-fold. IGF-I mRNA expression and protein secretion did not change to exclude autocrine activation. We conclude that Src stimulates IGF-I-dependent proliferation of PANC-1 cells by increasing the number of IGF-I receptors/cell.
A Multi-parameter Comprehensive Analytical Method for Online Assessing Power Transformer Condition (2013)

The effective assessment on the operation condition of power transformer is an important means to conduct the condition maintenance for transformer and to improve the reliability of power system. Based on the amount of information of all kinds of online monitoring conditions obtained from the transformer intelligent components, the assessment model for comprehensive condition of transformer is established in this study through the introduction of extension theory. First, with respect to the disadvantage that there is considerable subjectivity in constructing judgment matrix by the traditional analytic hierarchy process (AHP), a new method is adopted to construct the judgment matrix. The orthogonal experimental design method, finite element simulation as well as AHP are adopted to determine the weighting of condition information index with scientific and objective attitude; next, the framework for comprehensive assessment of transformer condition is established with the extension-based comprehensive assessment method to achieve the qualitative and quantitative assessment on the transformer condition. The method is proved to be correct and valid through analysis of examples. Streszczenie. W artykule przedstawiono wyniki badan dotyczących opracowania modelu szacowania warunkow pracy transformatora. W pierwszym kroku stworzono metode budowy macierzy orzeczen, z uwzglednieniem pewnych subiektywności tradycyjnej metody tworzenia tej macierzy (ang. Analytic Hierarchy Process). Do oszacowania wagi wskaźnika informacji o warunkach pracy, wykorzystano metode eksperymentalną, symulacje elementow skonczonych oraz AHP. Wykonane badania wykazaly poprawnośc dzialania metody. (Wszechstronna, wieloparametrowa metoda analizy do dynamicznego szacowania warunkow pracy transformatora).
Implementation and utilization of genetic testing in personalized medicine (2014)

Clinical genetic testing began over 30 years ago with the availability of mutation detection for sickle cell disease diagnosis. Since then, the field has dramatically transformed to include gene sequencing, high-throughput targeted genotyping, prenatal mutation detection, preimplantation genetic diagnosis, population-based carrier screening, and now genome-wide analyses using microarrays and next-generation sequencing. Despite these significant advances in molecular technologies and testing capabilities, clinical genetics laboratories historically have been centered on mutation detection for Mendelian disorders. However, the ongoing identification of deoxyribonucleic acid (DNA) sequence variants associated with common diseases prompted the availability of testing for personal disease risk estimation, and created commercial opportunities for direct-to-consumer genetic testing companies that assay these variants. This germline genetic risk, in conjunction with other clinical, family, and demographic variables, are the key components of the personalized medicine paradigm, which aims to apply personal genomic and other relevant data into a patient's clinical assessment to more precisely guide medical management. However, genetic testing for disease risk estimation is an ongoing topic of debate, largely due to inconsistencies in the results, concerns over clinical validity and utility, and the variable mode of delivery when returning genetic results to patients in the absence of traditional counseling. A related class of genetic testing with analogous issues of clinical utility and acceptance is pharmacogenetic testing, which interrogates sequence variants implicated in interindividual drug response variability. Although clinical pharmacogenetic testing has not previously been widely adopted, advances in rapid turnaround time genetic testing technology and the recent implementation of preemptive genotyping programs at selected medical centers suggest that personalized medicine through pharmacogenetics is now a reality. This review aims to summarize the current state of implementing genetic testing for personalized medicine, with an emphasis on clinical pharmacogenetic testing.
Coculture of dorsal root ganglion neurons and differentiated human corneal stromal stem cells on silk-based scaffolds (2015)

Corneal tissue displays the highest peripheral nerve density in the human body. Engineering of biomaterials to promote interactions between neurons and corneal tissue could provide tissue models for nerve/cornea development, platforms for drug screening, as well as innovative opportunities to regenerate cornea tissue. The focus of this study was to develop a coculture system for differentiated human corneal stromal stem cells (dhCSSCs) and dorsal root ganglion neurons (DRG) to mimic the human cornea tissue interactions. Axon extension, connectivity, and neuron cell viability were studied. DRG neurons developed longer axons when cocultured with dhCSSCs in comparison to neuron cultures alone. To assess the mechanism involved in the coculture response, nerve growth factors (NGF) secreted by dhCSSCs including NGF, brain-derived neurotrophic factor (BDNF), glial cell-derived neurotrophic factor (GDNF), and neurotrophin-3 were characterized with greater focus on BDNF secretion. DhCSSCs also secreted collagen type I, an extracellular matrix molecule favorable for neuronal outgrowth. This coculture system provides a slowly degrading silk matrix to study neuronal responses in concert with hCSSCs related to innervation of corneal tissue with utility toward human corneal nerve regeneration and associated diseases.
Rank correlation inferences for clustered data with small sample size (2022)

This paper develops methods to test for associations between two variables with clustered data using a <i>U</i>-Statistic approach with a second-order approximation to the variance of the parameter estimate for the test statistic. The tests that are presented are for clustered versions of: Pearsons <i>χ</i> <sup>2</sup> test, the Spearman rank correlation and Kendall's <i>τ</i> for continuous data or ordinal data and for alternative measures of Kendall's <i>τ</i> that allow for ties in the data. Shih and Fay use the <i>U</i>-Statistic approach but only consider a first-order approximation. The first-order approximation has inflated significance level in scenarios with small sample sizes. We derive the test statistics using the second-order approximations aiming to improve the type I error rates. The method applies to data where clusters have the same number of measurements for each variable or where one of the variables may be measured once per cluster while the other variable may be measured multiple times. We evaluate the performance of the test statistics through simulation with small sample sizes. The methods are all available in the R package cluscor.
Multiple paradigm shifts in multiple sclerosis (2003)

Purpose of review The present article reviews the currently ongoing scientific debate of our changing views on the pathogenesis of multiple sclerosis and the therapeutic strategies currently available for multiple sclerosis. Recent findings The most important observations include that (a) axonal loss accounts for permanent disability in multiple sclerosis, (b) remyelination should be possible in theory but fails for unknown reasons in the multiple sclerosis lesion, (c) inflammation can be beneficial, (d) treatment should be initiated early, and (e) immunosuppressive strategies exhibit beneficial effects in progressive forms of the disease. Summary Our current understanding of the immunopathogenesis of multiple sclerosis has changed in the past. Whereas demyelination was originally thought to be relevant for the lasting neurological deficit, it is nowadays commonly accepted that the extent of axonal loss dictates the degree of permanent clinical disability. How axonal damage can be prevented remains elusive. The interaction between the myelinating cell and the neuron gains increasing attention, however the evolving knowledge has not yet yielded new treatment concepts. Hence for the time being, it seems prudent to make optimal use of current approved therapies. Recent trials underlined the need for early initiation of treatment with immunomodulatory drugs. The superiority of one of the interferons is still a matter of debate, and a conclusive answer cannot be given at present. Finally, with mitoxantrone we have a drug at hand which can be used in progressive forms of multiple sclerosis, especially when other disease modifying drugs are not or no longer effective.
The force applied to the knee extensor mechanism differs between flat-footed and normal subjects during walking (2018)

Abstract Study aim: There is a lack of evidence to show the presence or absence of a relationship between foot morphology and changes of the force applied to the knee extensor mechanism. The purpose of this study was to examine whether the type of foot is a determining factor in the force applied to the extensor mechanism during walking. Materials and methods: Twenty female subjects (18-30 years), 10 with neutrally aligned feet and 10 with functional flat foot, participated in this study. Data were collected by employing a three dimensional motion capture system and a force platform, while the subjects were walking at their preferred speed. Knee extensor mechanism force was measured at sub-phases of gait (heel strike and toe-off). Results: A significant interaction was found between groups and sub-phases of gait for all the variables tested. The subjects with flat foot exhibited a significantly higher extensor mechanism force at toe-off compared to the control group (p &lt; 0.05). Conclusion: It can be concluded that subtalar hyper-pronation would increase the force applied to the knee extensor mecha­nism at toe-off, through increasing the knee sagittal angle, net external flexion moment and extensor mechanism moment arm. Therefore it may increase the possibility of musculoskeletal injuries
Online mixture and connection of basic motions for humanoid walking control by footprint specification (2002)

This paper introduces and describes a novel method which enables the online generation of humanoid walking patterns that follow desired footprint locations. Online generation is realized by the dynamically stable mixture and connection of pre-designed motions. Characteristics of zero moment point (ZMP) are utilized in order to maintain the overall dynamic stability of "mixed" motions. Experiments using an online pattern generation software system and online walking control joystick interface for the humanoid robot H6 are introduced to show the validity of the method.
From Intramolecular (Circular) in an Isolated Molecule to Intermolecular Hole Delocalization in a Two‐Dimensional Solid‐State Assembly: The Case of Pillarene (2018)

To achieve long-range charge transport/separation and, in turn, bolster the efficiency of modern photovoltaic devices, new molecular scaffolds are needed that can self-assemble in two-dimensional (2D) arrays while maintaining both intra- and intermolecular electronic coupling. In an isolated molecule of pillarene, a single hole delocalizes intramolecularly via hopping amongst the circularly arrayed hydroquinone ether rings. The crystallization of pillarene cation radical produces a 2D self-assembly with three intermolecular dimeric (sandwich-like) contacts. Surprisingly, each pillarene in the crystal lattice bears a fractional formal charge of +1.5. This unusual stoichiometry of oxidized pillarene in crystals arises from effective charge distribution within the 2D array via an interplay of intra- and intermolecular electronic couplings. This important finding is expected to help advance the rational design of efficient solid-state materials for long-range charge transfer.
Topological guided‐mode resonances at non‐Hermitian nanophotonic interfaces (2021)

Abstract The topological properties of photonic microstructures are of great interest because of their experimental feasibility for fundamental study and potential applications. Here, we show that robust guided‐mode‐resonance states exist in photonic domain‐wall structures whenever the complex photonic band structures involve certain topological correlations in general. Using the non‐Hermitian photonic analogy of the one‐dimensional Dirac equation, we derive essential conditions for photonic Jackiw‐Rebbi‐state resonances taking advantage of unique spatial confinement and spot‐like spectral features which are remarkably robust against random parametric errors. Therefore, the proposed resonance configuration potentially provides a powerful method to create compact and stable photonic resonators for various applications in practice.