gebhart/patrepeval huggingface dataset:
Exome sequencing identifies <i><scp>NFS</scp>1</i> deficiency in a novel Fe‐S cluster disease, infantile mitochondrial complex <scp>II</scp>/<scp>III</scp> deficiency (2013)

Iron-sulfur (Fe-S) clusters are a class of highly conserved and ubiquitous prosthetic groups with unique chemical properties that allow the proteins that contain them, Fe-S proteins, to assist in various key biochemical pathways. Mutations in Fe-S proteins often disrupt Fe-S cluster assembly leading to a spectrum of severe disorders such as Friedreich's ataxia or iron-sulfur cluster assembly enzyme (ISCU) myopathy. Herein, we describe infantile mitochondrial complex II/III deficiency, a novel autosomal recessive mitochondrial disease characterized by lactic acidemia, hypotonia, respiratory chain complex II and III deficiency, multisystem organ failure and abnormal mitochondria. Through autozygosity mapping, exome sequencing, in silico analyses, population studies and functional tests, we identified c.215G>A, p.Arg72Gln in NFS1 as the likely causative mutation. We describe the first disease in man likely caused by deficiency in NFS1, a cysteine desulfurase that is implicated in respiratory chain function and iron maintenance by initiating Fe-S cluster biosynthesis. Our results further demonstrate the importance of sufficient NFS1 expression in human physiology.
The High Specificity of the Manganese Pathway Through the Body12 (1958)

The accepted ideas about the physiological role of manganese have been derived predominantly from in vitro experiments. However, these are characterized by lack of specificity: Only a limited discrimination between manganese and some other metals is shown, for instance, in metal transport systems (1, 2), in enzyme activation (2-4) and in the preservation of mitochondria (5). The conclusions from these experiments were of in- terest because they threw new doubt on the func- tional specificity and even on the essentiality of manganese in the living organism. Thus it be- came necessary to ascertain whether manganese may be replaced in the intact animal as well. To investigate this, we have used a variation of the classical technique of flooding the organism with metals which we were led to believe would sub- stitute for manganese. Surprisingly, these kindred metals were ineffective in eluting radiomanganese from the body: Only manganese compounds proved effective in that regard.
Enhanced Neurovirulence of Borna Disease Virus Variants Associated with Nucleotide Changes in the Glycoprotein and L Polymerase Genes (2002)

Borna disease virus (BDV) infection produces a variety of clinical diseases, from behavioral illnesses to classical fatal encephalitis (i.e., Borna disease [BD]). Since the genomes of most BDV isolates differ by less than 5%, host factors are believed responsible for much of the reported variability in disease expression. The contribution of BDV genomic differences to variation in BD expression is largely unexplored. Here we compared the clinical outcomes of rats infected with one of two related BDV variants, CRP3 or CRNP5. Compared to rats inoculated with CRP3, adult and newborn Lewis rats inoculated with CRNP5 had more severe and rapidly fatal neurological disease, with increased damage to the hippocampal pyramidal neurons and rapid infection of brain stem neurons. To identify possible virus-specific contributions to the observed variability in disease outcome, the genomes of CRP3 and CRNP5 were sequenced. Compared to CRP3, there were four nucleotide changes in the CRNP5 variant, two each in the G protein and in the L polymerase, resulting in four amino acid changes. These results suggest that small numbers of genomic differences between BDV variants in the G protein and/or L polymerase can contribute to the variability in BD outcomes.
Protective role for proteoglycans against cationic lipid cytotoxicity allowing optimal transfection efficiency in vitro (1999)

A dependence on proteoglycans for cationic lipid-mediated gene transfer has been suggested in previous studies [Mislick and Baldeschwieler (1996) Proc. Natl. Acad. Sci. U.S.A. 93, 12349-12354; Mounkes, Zhong, Cipres-Palacin, Heath and Debs (1998) J. Biol. Chem. 273, 26164-26170]. We have evaluated the mechanism of proteoglycan involvement in cationic lipid-mediated gene transfer. DNA plasmid uptake and gene expression were studied in wild-type Chinese hamster ovary (CHO) cells (CHO-K1), heparan sulphate-deficient CHO cells (pgsD-677) and proteoglycan-deficient CHO cells (pgsB-618). At an optimal ratio of cationic lipid to DNA, a substantial decrease in reporter gene expression was observed in proteoglycan-deficient cells compared with that in heparan sulphate-deficient and wild-type cells. However, there were no differences in reporter gene expression between the cell lines when transfected by electroporation. Moreover, all cell lines exhibited equal cationic-lipid-DNA complex uptake activities, as assessed by the measurement of intracellular (32)P-labelled and rhodamine-labelled DNA plasmid. An analysis of reflected-light images of wild-type and proteoglycan-deficient cells suggested that cationic lipids were preferentially toxic to proteoglycan-deficient cells. Cell-growth assays confirmed this, showing that cationic lipids exhibited a greater anti-proliferative activity in proteoglycan-deficient cells and in chlorate-treated wild-type cells than in the other cell lines. The growth-inhibitory effect of cationic lipids was abrogated by the addition of exogenous sulphated glycosaminoglycans. We conclude that the glycosaminoglycan part of proteoglycans serves a protective role against cationic lipid cytotoxicity, allowing optimal transfection efficiency in vitro.
A crowdsourcing approach to support video annotation (2013)

In this paper we present an innovative approach to support efficient large scale video annotation by exploiting the crowdsourcing. In particular, we collect big noisy annotations by an on-line Flash game which aims at taking photos of objects appearing through the game levels. The data gathered (suitably processed) from the game is then used to drive image segmentation approaches, namely the Region Growing and Grab Cut, which allow us to derive meaningful annotations. A comparison against hand-labeled ground truth data showed that the proposed approach constitutes a valid alternative to the existing video annotation approaches and allow a reliable and fast collection of large scale ground truth data for performance evaluation in computer vision.
Altered bcl-2 and bax Expression and Intracellular Ca2+ Signaling in Apoptosis of Pancreatic Cells and the Impairment of Glucose-Induced Insulin Secretion (1998)

Apoptosis is the process of cellular self-destruction, and genes such as bcl-2 and bax are known to inhibit and promote apoptosis, respectively. In this study, we show that apoptosis can be induced in pancreatic β-cell lines, and we investigate the apoptotic pathways through the bcl-2 and bax genes and intracellular Ca2+. Serum deprivation induces apoptosis in the MIN6 and RINm5F pancreaticβ -cell lines, and alters the bcl-2 messenger RNA (mRNA) and protein. KCl, BayK, A23187, and ionomycin elicit an elevation of cytosolic/nuclear Ca2+, which, however, is insufficient to evoke apoptosis or to alter bcl-2 or bax mRNA expression in MIN6 cells. The extracellular Ca2+ chelators, EGTA and 1,2-Bis(2-aminophenoxy)ethane-N,N,N′,N′-tetraacetic acid, tetrapotassium salt, hydrate, evoke apoptosis and also alter the ratio of bcl-2 to bax mRNA and protein concomitantly with the depletion of cytosolic/nuclear Ca2+. This indicates that there are at least two apoptotic pathways in pancreatic β-cells: through serum deprivation and through a decrease in cytosolic/nuclear Ca2+. MIN6 cells exhibit reduced insulin secretion induced by glucose regardless of the molecular pathway of apoptosis. Apoptosis in pancreatic β-cells, therefore, may be closely related to the impairment of insulin secretion in certain pathological conditions such as diabetes mellitus.
A simple photonic generation of linearly chirped microwave pulse with large time-bandwidth product and high compression ratio (2013)

Based on the heterodyne beating between the pre-chirped optical pulse and the continuous wave (CW) light in a wideband photodetector (PD), linearly chirped microwave pulse with time duration of 3.2ns and bandwidth of 33GHz, which yields a large time-bandwidth product (TBWP) of 106 and high compression ratio of 160, is generated in our experiment. Dispersion compensation fiber (DCF) with uniform response across broad bandwidth is used for providing the original linear chirp in our method, which shows the promise to generate linearly chirped microwave pulse with bandwidth of up to THz. The flexibility of the center frequency and the stability of the time-frequency performance are demonstrated by generating different types of linearly chirped microwave pulses. The range resolution of our generated microwave pulse is also verified by off-line processing.
An adaptive rate control scheme for H.264 scalable video coding (2013)

Rate Control plays an important role in video coding, which performs successful transmission of all encoded bits with available limited bandwidth. This paper aims to provide an adaptive rate controller for H.264 scalable video coding. The Quantization Parameter (QP) plays an important role in any rate controller for encoding demanded bits. The Quantization parameter estimation and the rate controller algorithm follows two pass 1) Initial Quantization Parameter value estimated by simplified Rate Distortion model using Cauchy Density based PDF and Buffer status 2) Rate Distortion Optimization method to determine minimum cost for the mode and motion vector using the estimated Quantization Parameter. The experimental result shows that our rate control algorithm is better than the FixedQPencoder of JSVM 9.19.15 in terms of PSNR and bit rate. Moreover our scheme performs in single iteration to encode the frame with the desired QP and Buffer status calculated to prevent from overflow and underflow.
Role of P/Q-Ca<sup>2+</sup> Channels in Metabotropic Glutamate Receptor 2/3-Dependent Presynaptic Long-Term Depression at Nucleus Accumbens Synapses (2002)

The nucleus accumbens (NAc) is an important cerebral area involved in reward and spatial memory (Pennartz et al., 1994), but little is known about synaptic plasticity in this region. Here, electron microscopy revealed that, in the NAc, metabotropic glutamate receptors 2/3 (mGlu2/3) immunostaining was essentially associated with axonal terminals and glial processes, whereas postsynaptic dendrites and neuronal cell bodies were unstained. Electrophysiological techniques in the NAc slice preparation demonstrated that activation of mGlu2/3 with synaptically released glutamate or specific exogenous agonist, such as LY354740 (200 nm, 10 min), induced long-term depression of excitatory synaptic transmission (mGlu2/3-LTD). Tetanic-LTD and pharmacological mGlu2/3-LTD occluded each other, suggesting common mechanisms. The mGlu2/3-LTD did not require synaptic activity but depended on the cAMP-protein kinase A cascade. Selective inhibition of P/Q-type Ca(2+) channels with omega-agatoxin-IVA occluded the expression of mGlu2/3-LTD, and, conversely, the inhibitory effects of omega-agatoxin-IVA were abolished during mGlu2/3-LTD. Thus, mGlu2/3 play an important role in the control of use-dependent synaptic plasticity at prelimbic cortex-NAc synapses: their activation causes a form of LTD mediated by the long-lasting reduction of P/Q-type Ca(2+)channels contribution to transmitter release.
Temperature-dependent translational regulation of the ER omega-3 fatty acid desaturase gene in wheat root tips (2000)

The proportion of alpha-linolenic acid (18:3) among the total fatty acids in root tissue increases as the growth temperature decreases. Endoplasmic reticulum omega-3 fatty acid desaturase is responsible for the production of most of the 18:3 in root tissue. In this study, the effect of temperature on expression of the TaFAD3 gene, which encodes endoplasmic reticulum omega-3 desaturase in wheat (Triticum aestivum L. cv. Chihoku), was analysed at the mRNA and protein levels. In wheat root tips grown at 30 and 10 degrees C, the proportions of 18:3 among total fatty acids were 22% and 55%, respectively. The level of TaFAD3 protein in microsomal preparations of the root tips grown at 10 degrees C was approximately 7.5 times higher than that of the root tips grown at 30 degrees C. The increased level of TaFAD3 protein at the lower temperature was accompanied by enhanced association of TaFAD3 mRNA with polyribosomes. In contrast, the level of TaFAD3 mRNA in root tips grown at 10 degrees C was slightly higher than in those grown at 30 degrees C. These results suggest that, in root tips, the increase in the 18:3 level at low temperature is achieved directly by an increase in the amount of TaFAD3 protein, and that temperature-dependent translational regulation of the TaFAD3 gene, rather than its transcriptional regulation, contributes to modulation of the TaFAD3 protein accumulation.
Dose‐related effects of motilin on proximal gastrointestinal motility (2002)

Motilin increased antrum contraction frequency, whereas no effect on gastric myoelectrical activity was observed. Antrum contraction frequency appears to be a useful biomarker for motilin efficacy, and motilin doses of 2 and 4 pmol x min/kg were equally effective.
Convergence analysis of LMS filters with uncorrelated Gaussian data (1985)

Statistical analysis of the least mean-squares (LMS) adaptive algorithm with uncorrelated Gaussian data is presented. Exact analytical expressions for the steady-state mean-square error (mse) and the performance degradation due to weight vector misadjustment are derived. Necessary and sufficient conditions for the convergence of the algorithm to the optimal (Wiener) solution within a finite variance are derived. It is found that the adaptive coefficient μ, which controls the rate of convergence of the algorithm, must be restricted to an interval significantly smaller than the domain commonly stated in the literature. The outcome of this paper, therefore, places fundamental limitations on the mse performance and rate of convergence of the LMS adaptive scheme.
Effects of benazepril and hydrochlorothiazide, given alone and in low- and high-dose combinations, on blood pressure in patients with hypertension (1996)

Benazepril in combination with hydrochlorothiazide, including a low-dose combination of 5/6.25 mg, is effective in reducing sitting diastolic and systolic blood pressure in patients with hypertension.
Time reversal space time block coding with channel estimation errors (2004)

Time reversal (TR) space time block coding (STBC) is a novel time domain technique to mitigate inter-symbol interference (ISI) in frequency-selective fading channels. This paper derives time-domain TR equations without requiring the q-operator, and presents simulation results for the case when channel estimates are not accurately determined through training. The time-domain TR equations have particular relevance towards the architecture of a TR-STBC system implementation whilst the calculated effects of channel model inaccuracy impact system packet structure and protocol issues.
Wireless communications in the era of big data (2015)

The rapidly growing wave of wireless data service is pushing against the boundary of our communication network's processing power. The pervasive and exponentially increasing data traffic present imminent challenges to all aspects of wireless system design, such as spectrum efficiency, computing capabilities, and fronthaul/backhaul link capacity. In this article, we discuss the challenges and opportunities in the design of scalable wireless systems to embrace the big data era. On one hand, we review the state-of-the-art networking architectures and signal processing techniques adaptable for managing big data traffic in wireless networks. On the other hand, instead of viewing mobile big data as an unwanted burden, we introduce methods to capitalize on the vast data traffic, for building a big-data-aware wireless network with better wireless service quality and new mobile applications. We highlight several promising future research directions for wireless communications in the mobile big data era.
Utilization of Exhaust Waste Heat from Power Plants for Sea Water Desalination (2009)

The international demand of water increased compared to the available water resources due torecent development water resources for different uses. In many areas especially in Arabian GulfAreas, a shortage of portable water. This study deals with design a model to combine the multi effect desalination water (MED) with the steam power plant. Using the exhaust waste heat of the power plant to heat the sea water. The model has been based upon various heat and mass transfer equations. Taking in consideration all requirements of making available safe drinking water for requirement. The waste heat of the exhaust gases of power plant is captured before exhausting it to the atmosphere and transferred to salty water. The temperature of salty water rise to level 90C to130C. This is ideal temperature for desalination in multiple effect distillation MED plant. The hot salty water then passes through a flash into the evaporator then condenses on the condenser tube bank, and internal heat recovery is achieved by transferring its heat of condensation to the sea water feed that is thus being preheated.In this study selective an exist steam power plants, combine with MED to produce 1000 m3/day to15000 m3/day drinking water from salty water. desalination.
Variability of Doppler ankle pressures with arterial occlusive disease: an evaluation of ankle index and brachial-ankle pressure gradient. (1981)

The variability of Doppler ankle pressures was studied in 35 men with clinically stable claudication. An average of 6.8 measurements was obtained from each man. The mean range of ankle index was 0.18 with a mean standard deviation of 0.07. The brachial-ankle pressure gradient had a wider spread, with a mean range of 38 mm Hg and a standard deviation of 13.9. The amount of variation in a given patient was unrelated to mean blood pressure or mean ankle index. The results of this study show that the ankle index must change at least 0.15 before it can be considered significant. Based on the variability found, we recommend that multiple baseline determinations be obtained on patients who are to be followed longitudinally.
Separation of γ-Casein<sup>2</sup> (1950)

ADVERTISEMENT RETURN TO ISSUEPREVArticleNEXTSeparation of γ-Casein2N. J. Hipp, M. L. Groves, J. H. Custer, and T. L. McMeekinCite this: J. Am. Chem. Soc. 1950, 72, 11, 4928–4931Publication Date (Print):November 1, 1950Publication History Published online1 May 2002Published inissue 1 November 1950https://doi.org/10.1021/ja01167a023RIGHTS & PERMISSIONSArticle Views118Altmetric-Citations67LEARN ABOUT THESE METRICSArticle Views are the COUNTER-compliant sum of full text article downloads since November 2008 (both PDF and HTML) across all institutions and individuals. These metrics are regularly updated to reflect usage leading up to the last few days.Citations are the number of other articles citing this article, calculated by Crossref and updated daily. Find more information about Crossref citation counts.The Altmetric Attention Score is a quantitative measure of the attention that a research article has received online. Clicking on the donut icon will load a page at altmetric.com with additional details about the score and the social media presence for the given article. Find more information on the Altmetric Attention Score and how the score is calculated. Share Add toView InAdd Full Text with ReferenceAdd Description ExportRISCitationCitation and abstractCitation and referencesMore Options Share onFacebookTwitterWechatLinked InReddit PDF (508 KB) Get e-Alerts Get e-Alerts
Flexible Control of Parallelism in a Multiprocessor PC Router (2001)

SMP Click is a software router that provides both flexibility and high performance on stock multiprocessor PC hardware. It achieves high performance using device, buffer, and queue management techniques optimized for multiprocessor rout-ing. It allows vendors or network administrators to configure the router in a way that indicates parallelizable packet pro-cessing tasks, and adaptively load-balances those tasks across the available CPUs. SMP Click’s absolute performance is high: it can forward 494,000 64-byte IP packets per second on a 2-CPU 500 MHz Intel Xeon machine, compared to 302,000 packets per second for uniprocessor Click. SMP Click also scales well for CPU intensive tasks: 4-CPU SMP Click can encrypt and forward 87,000 64-byte packets per second using IPSec 3DES, com-pared to 23,000 packets per second for uniprocessor Click. 1
Metabolism and excretion of etoposide in isolated, perfused rat liver models. (1988)

The clearance of etoposide and formation of etoposide glucuronide have been measured in an isolated, perfused rat liver model to evaluate the effect of impaired hepatic function on etoposide kinetics. Hepatocellular injury was produced by pretreatment of rats with allyl alcohol or carbon tetrachloride; ligation of the bile duct simulated obstructive biliary disease. Etoposide clearance (3.59 +/- 1.06 ml/min) was reduced by both carbon tetrachloride (2.07 +/- 0.64 ml/min; P = 0.05) and allyl alcohol treatment (2.14 +/- 0.62 ml/min; P = 0.05). Biliary obstruction also impaired etoposide clearance but to a lesser extent than hepatocellular injury (2.47 +/- 0.69 ml/min; P = 0.20 versus control). In both hepatocellular and obstructive models, direct biliary etoposide excretion decreased. The metabolic clearance of etoposide to its glucuronide declined by 36% in the hepatotoxin models but was not decreased by biliary obstruction. Following hepatic injury, there is a reduction in the metabolism and excretion of etoposide by the liver. This effect is most marked on biliary drug excretion. Obstructive biliary disease does not significantly alter etoposide glucuronidation. Since most cancer patients have increased bilirubin on the basis of obstructive disease, little or no etoposide dose alteration will be needed. However, in the patient with significant hepatocellular injury, impaired etoposide clearance will be more pronounced, and etoposide dose alterations may be needed.
Effects of a 33 residue interleukin-1 beta peptide and the antioxidant PQQ on interleukin-1 beta-mediated inhibition of glucose-stimulated insulin release from cultured mouse pancreatic islets. (1996)

Interleukin-1 beta (IL-1 beta) significantly inhibits insulin secretion from glucose stimulated islet cells. The mechanism for this inhibition has been hypothesized to be due to stimulation of the inducible form of nitric oxide synthase and a resulting increase in nitric oxide (NO) concentration. Ways to block the effect of IL-1 beta have focused on blocking the binding of IL-1 beta to the IL-1 receptor and the use of antioxidants to neutralize increases in NO. This report focuses on a 33 residue peptide synthesized based on the C-terminal region of the IL-1 beta molecule, a reported binding site of the IL-1 beta molecule, and the redoxcycling antioxidant pyrroloquinoline quinone (PQQ). The 33 residue peptide did not function as an antagonist, but as a weak agonist. High concentrations of PQQ itself inhibited glucose-dependent insulin release while low concentrations did not. PQQ had no effect on the actions of IL-1 beta. Three isosteric and isomeric analogues of PQQ were also investigated. One of the PQQ isomers had an inhibitory effect on insulin secretion at low concentrations where PQQ had no effect. These results reflect the sensitivity of islets to oxidative stress.