gebhart/patrepeval huggingface dataset:
Clinical heterogeneity within xeroderma pigmentosum associated with mutations in the DNA repair and transcription gene ERCC3. (1994)

The human DNA excision repair gene ERCC3 specifically corrects the nucleotide excision repair (NER) defect of xeroderma pigmentosum (XP) complementation group B. In addition to its function in NER, the ERCC3 DNA helicase was recently identified as one of the components of the human BTF2/TFIIH transcription factor complex, which is required for initiation of transcription of class II genes. To date, a single patient (XP11BE) has been assigned to this XP group B (XP-B), with ther remarkable conjunction of two autosomal recessive DNA repair deficiency disorders: XP and Cockayne syndrome (CS). The intriguing involvement of the ERCC3 protein in the vital process of transcription may provide an explanation for the rarity, severity, and wide spectrum of clinical features in this complementation group. Here we report the identification of two new XP-B patients: XPCS1BA and XPCS2BA (siblings), by microneedle injection of the cloned ERCC3 repair gene as well as by cell hybridization. Molecular analysis of the ERCC3 gene in both patients revealed a single base substitution causing a missense mutation in a region that is completely conserved in yeast, Drosophila, mouse, and human ERCC3. As in patient XP11BE, the expression of only one allele (paternal) is detected. The mutation causes a virtually complete inactivation of the NER function of the protein. Despite this severe NER defect, both patients display a late onset of neurologic impairment, mild cutaneous symptoms, and a striking absence of skin tumors even at an age of > 40 years. Analysis of the frequency of hprt- mutant T-lymphocytes in blood samples suggests a relatively low in vivo mutation frequency in these patients. Factors in addition to NER deficiency may be required for the development of cutaneous tumors.
Self-attention Based Prosodic Boundary Prediction for Chinese Speech Synthesis (2019)

Predicting prosodic boundaries from input text plays an important role in Chinese text-to-speech (TTS) system, which directly influences the naturalness and intelligibility of synthesized speech. In this paper, we propose to combine self-attention with multitask learning for prosodic boundary prediction. Self-attention is used to capture the dependency between two arbitrary characters in the input sentence, while multitask learning models the relationships between prosodic boundaries and lexicon words by setting word segmentation as an auxiliary task. The proposed method can generate prosodic boundary labels directly from Chinese characters and achieve the whole process end-to-end. Experimental results show the effectiveness of our proposed model and prove that the performance can be further improved by pretraining the model with extra word segmentation data.
Detecting <i>Bacillus cereus</i> spores on a mail sorting system using Raman spectroscopy (2004)

Abstract The ability of Raman spectroscopy to detect anthrax‐causing spores as they pass through a mail sorting system was investigated. A pump was connected to an existing vacuum manifold on a commercial sorter, and a filter designed to capture 0.5–3 µm particles was placed in‐line. A standard business letter containing 0.23 g of Bacillus cereus spores, a Bacillus anthracis surrogate, was placed in a stack of 20 letters and passed through the system. Raman spectra of the filter positively identified the captured material as bacterial spores by the dominant calcium dipicolinate Raman spectral bands associated with the spore core. A limit of detection, using 400 mW of 785 nm laser excitation for a 1‐s acquisition, is estimated at 4.5 mg. The ability of a Raman spectroscopy based system to detect and prevent the distribution of a letter containing gram levels of anthrax spores is discussed. Copyright © 2004 John Wiley & Sons, Ltd.
El delito de fraude de subvenciones ante la Ley General de Subvenciones (2004)

La aprobacion de la tan esperada Ley General de Subvenciones ha supuesto un importante punto de inflexion en el tratamiento administrativo de la materia, regulando con detalle el status de los derechos y obligaciones de los beneficiarios y entidades colaboradoras, el sistema de justificacion de las subvenciones, su control asi como el regimen de infracciones y sanciones. Pero la nueva Ley tambien tiene una clara incidencia en el ambito penal, en cuanto representa un innegable referente para integrar las previsiones de este orden. En el trabajo se analizan los distintos aspectos del nuevo regimen administrativo al hilo de las reflexiones en torno a la forma en que sus previsiones inciden en la comprension del delito de fraude de subvenciones.
The mean field theory in EM procedures for blind Markov random field image restoration (1993)

A Markov random field (MRF) model-based EM (expectation-maximization) procedure for simultaneously estimating the degradation model and restoring the image is described. The MRF is a coupled one which provides continuity (inside regions of smooth gray tones) and discontinuity (at region boundaries) constraints for the restoration problem which is, in general, ill posed. The computational difficulty associated with the EM procedure for MRFs is resolved by using the mean field theory from statistical mechanics. An orthonormal blur decomposition is used to reduce the chances of undesirable locally optimal estimates. Experimental results on synthetic and real-world images show that this approach provides good blur estimates and restored images. The restored images are comparable to those obtained by a Wiener filter in mean-square error, but are most visually pleasing.
Dual-Carrier Floquet Circulator with Time-Modulated Optical Resonators (2018)

Spatiotemporal modulation has shown great promise as a strong time-reversal symmetry breaking mechanism that enables integrated nonreciprocal devices and topological materials at optical frequencies. However, ideal circulator and isolator performance has relied on spatial symmetry or momentum matching between modulation and optical modes. The resulting systems have been challenging to experimentally realize, due to the prohibitively complex and lossy biasing networks and tight fabrication tolerances that maintain the desired rotational and mirror symmetries. In this work, we propose a microresonator Floquet circulator that leverages the previously untapped degrees of freedom of the modulation, through waveforms with strong harmonic components. The Floquet circulator response exhibits ideal on-resonance isolation and supports broadband forward transmission with no trade-off in insertion loss. We present a numerical demonstration in an on-chip photonic crystal platform with just two modulated resonators requiring no rotational symmetry. Moreover, this approach is general and can leverage a variety of modulation mechanisms while not being limited by pump depletion and signal distortion associated with parametric nonlinear systems.
Glymphatic distribution of CSF-derived apoE into brain is isoform specific and suppressed during sleep deprivation (2016)

Thus, choroid plexus/CSF provides an additional source of apoE and the glymphatic fluid transporting system delivers it to brain via the periarterial space. By implication, failure in this essential physiological role of the glymphatic fluid flow and ISF clearance may also contribute to apoE isoform-specific disorders in the long term.
Autosomal dominant VCP hypomorph mutation impairs disaggregation of PHF-tau (2020)

Neurodegeneration in Alzheimer's disease (AD) is closely associated with the accumulation of pathologic tau aggregates in the form of neurofibrillary tangles. We found that a p.Asp395Gly mutation in <i>VCP</i> (valosin-containing protein) was associated with dementia characterized neuropathologically by neuronal vacuoles and neurofibrillary tangles. Moreover, VCP appeared to exhibit tau disaggregase activity in vitro, which was impaired by the p.Asp395Gly mutation. Additionally, intracerebral microinjection of pathologic tau led to increased tau aggregates in mice in which p.Asp395Gly <i>VCP</i> mice was knocked in, as compared with injected wild-type mice. These findings suggest that p.Asp395Gly <i>VCP</i> is an autosomal-dominant genetic mutation associated with neurofibrillary degeneration in part owing to reduced tau disaggregation, raising the possibility that VCP may represent a therapeutic target for the treatment of AD.
Nieuwkomers in kasconcepten naar Bleiswijk: Zowakas: Verbeterde versie van energieproducerende kas (2007)

De wedstrijd Kas als Energiebron, waarbij met nieuwe kasconcepten geprobeerd wordt de glastuinbouw minder afhankelijk te maken van aardgas, kent drie winnaars; ZonWaKas, ZoWaKas en ZonWindkas. Onhandig dat die namen zo op elkaar lijken. Des te beter is het dat deze ‘papieren’ ontwerpen zo snel mogelijk een demokas krijgen. Het gebeurt in 2008 bij WUR Glastuinbouw in Bleiswijk. Alvast een voorproefje van de drie winnende concepten in dit artikel
Fragment identifiers for plain text files (2005)

Hypermedia systems like the Web heavily depend on their ability to link resources. One of the key features of the Web's URIs is their ability to not only specify a resource, but to also identify a subresource within that resource, by using a fragment identifier. Fragment identification enables user to create better hypermedia. We present a proposal for fragment identifiers for plain text files, which makes it possible to identify character or line ranges, or subresources identified by regular expressions. Using these fragment identifiers, it is possible to create more specific hyperlinks, by not only linking to a complete plain text resource, but only the relevant part of it. Along with this proposal, a prototype implementation is described which can be used both as a server-side testbed and as a client-side extension for the Firefox browser.
Variation of neutrophil function with age in calves. (1986)

Neutrophil function was evaluated on 2 occasions in 5 calves from each of the following age groups: 4 to 5 weeks, 9 to 11 weeks, 16 to 19 weeks, and 12 to 14 months. Of the neutrophil functions examined, the iodination reaction, which evaluates the activity of the myeloperoxidase-hydrogen peroxide-halide antibacterial system of the neutrophil, reflected the most marked differences among age groups. The iodination values for the 2 youngest age groups were approximately 52% of the value for the oldest cattle. This difference could not be attributed to the amount of myeloperoxidase in the neutrophil granules. Antibody-dependent cell-mediated cytotoxicity also tended to be lower in neutrophils from the 3 younger age groups. Nitroblue tetrazolium reduction, a measure of superoxide anion generation by neutrophils, was lower in the youngest age group only. The capability of neutrophils to ingest Staphylococcus aureus was higher in the 3 youngest groups of calves than in the oldest group. The observed differences in neutrophil function in young vs older calves may be partially responsible for the increased susceptibility of young calves to infectious disease.
Detection of Mycobacterium tuberculosis in cerebrospinal fluid following immunomagnetic enrichment (1996)

The detection of Mycobacterium tuberculosis by culture of cerebrospinal fluid (CSF) is unacceptably slow. Low numbers of organisms and the presence of reaction inhibitors may prevent detection of M. tuberculosis by PCR. We used immunomagnetic enrichment to accelerate and enhance the detection of mycobacteria in CSF after demonstrating the utility of the method with pure suspensions. Growth was detected earlier in Bactec cultures of magnetically recovered mycobacteria than in untreated CSF (7 versus 15 days). We detected M. tuberculosis DNA by PCR in the immunomagnetically enriched sample but not in untreated CSF. PCR fingerprintings of the immunomagnetically recovered M. tuberculosis and of the isolate subsequently recovered by culture were identical.
Differential renal effects of cyclooxygenase inhibition in sodium-replete and sodium-deprived dog (1980)

We studied the renal effects of the cyclooxygenase inhibitor sodium meclofenamate (M) (5 mg/kg, iv) in the pentobarbital-anesthetized dog that had been maintained on an elevated (100 meq/day) or on a reduced (< meq/day) sodium intake, and during the administration of antiotensin II in the sodium-replete dog, or the angiotensin receptor blocker [Sar1-Ala8]angiotensin II in the sodium-deprived dog. In the sodium-replete dog, M did not affect mean arterial blood pressure (MABP), renal blood flow (RBF), glomerular filtration rate, (GFR), or urine volume (V), but reduced the urinary excretion of sodium (UNaV) by 47%, and of immunoreactive PGE2 (iPGE2) by 90%. However, in the sodium-replete dog during angiotensin II infusion (3 ng . kg-1 . min-1, iv), M reduced RBF by 35%, GFR by 24%, V by 72%, and iPGE2 by 94%. Similarly, in the sodium-deprived dog M reduced RBF by 34%, GFR by 28%, and iPGE2 excretion by 89%. However, M did not affect RBF or GFR in the sodium-deprived dog during infusion of [Sar1-Ala8]angiotensin II (6 microgram . kg-1 . min-1, iv), although iPGE2 excretion was reduced by 84%. This study demonstrates that the effects of M on renal hemodynamics in the dog vary with the state of sodium balance and suggests that a prostaglandin(s) contributes to maintenance of renal blood flow during activation of the renin-angiotensin system.
Inhibition of human ovarian cancer cell proliferation in vitro by ginsenoside Rh2 and adjuvant effects to cisplatin in vivo (1991)

In vitro and in vivo effects of ginsenoside Rh2 on human ovarian tumor growth were examined by using a cell line (HRA) derived from ascites of a patient with serous cystadenocarcinoma of the ovary. The HRA cell proliferation in vitro was inhibited in a dose-dependent manner with dosages of 10-100 microM of ginsenoside RH2. DNA, RNA and protein synthesis by the HRA cells was inhibited in a dose-dependent manner at more than 15 microM of ginsenoside RH2. However, the growth of HRA cells transplanted in nude mice was not significantly inhibited by ginsenoside RH2. On the contrary, when cisplatin was administered together with 10 microM (but not 1 microM or 100 microM) ginsenoside RH2, the tumor growth was significantly inhibited 31 days after inoculation and the survival was also significantly prolonged, compared with not only the untreated group but also the groups given cisplatin alone or ginsenoside RH2 alone. This indicates synergistic effects between cisplatin and ginsenoside RH2. From monitoring of body weight and hematocrit, concentrations of ginsenoside RH2 used in this study did not seem to cause any adverse effect.
Kinetic modeling of the enzymatic hydrolysis of pretreated cellulose (1985)

The production of sugars by the enzymatic hydrolysis of cellulose is a two-step process that includes conversion of the intermediate cellobiose to glucose by beta-glucosidase. The hydrolysis was followed by analyzing the two sugar products (cellobiose and glucose). The enzyme showed maximum activity at pH 4.8. Thermal deactivation was significant at temperatures above 45 degrees C. At 50 degrees C (optimum temperature) thermal deactivation was found to follow first-order kinetics. Several models were tested by modeling the kinetics of the reaction. Their parameter values were determined by numerical optimization, including temperature dependence. The best fitting model was a competitive product inhibition for the two reactions in the operational range.
Efficacy of alpha1-antitrypsin augmentation therapy in conditions other than pulmonary emphysema (2011)

Up to now alpha 1-antitrypsin (AAT) augmentation therapy has been approved only for commercial use in selected adults with severe AAT deficiency-related pulmonary emphysema (i.e. PI*ZZ genotypes as well as combinations of Z, rare and null alleles expressing AAT serum concentrations <11 μmol/L). However, the compassionate use of augmentation therapy in recent years has proven outstanding efficacy in small cohorts of patients suffering from uncommon AAT deficiency-related diseases other than pulmonary emphysema, such as fibromyalgia, systemic vasculitis, relapsing panniculitis and bronchial asthma. Moreover, a series of preclinical studies provide evidence of the efficacy of AAT augmentation therapy in several infectious diseases, diabetes mellitus and organ transplant rejection. These facts have generated an expanding number of medical applications and patents with claims for other indications of AAT besides pulmonary emphysema. The aim of the present study is to compile and analyze both clinical and histological features of the aforementioned published case studies and reports where AAT augmentation therapy was used for conditions other than pulmonary emphysema. Particularly, our research refers to ten case reports and two clinical trials on AAT augmentation therapy in patients with both AAT deficiency and, at least, one of the following diseases: fibromyalgia, vasculitis, panniculitis and bronchial asthma. In all the cases, AAT was successfully applied whereas previous maximal conventional therapies had failed. In conclusion, laboratory studies in animals and humans as well as larger clinical trials should be, thus, performed in order to determine both the strong clinical efficacy and security of AAT in the treatment of conditions other than pulmonary emphysema.
Pythagoras and Aristoxenos Reconciled (1958)

Research Article| July 01 1958 Pythagoras and Aristoxenos Reconciled Norman Cazden Norman Cazden Search for other works by this author on: This Site PubMed Google Scholar Journal of the American Musicological Society (1958) 11 (2-3): 97–105. https://doi.org/10.2307/829897 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Share Icon Share Twitter LinkedIn Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Norman Cazden; Pythagoras and Aristoxenos Reconciled. Journal of the American Musicological Society 1 July 1958; 11 (2-3): 97–105. doi: https://doi.org/10.2307/829897 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search Search nav search search input Search input auto suggest search filter All ContentJournal of the American Musicological Society Search This content is only available via PDF. Copyright 1959 The American Musicological Society Article PDF first page preview Close Modal You do not currently have access to this content.
Human Embryonic Stem Cells Have Enhanced Repair of Multiple Forms of DNA Damage (2008)

Embryonic stem cells need to maintain genomic integrity so that they can retain the ability to differentiate into multiple cell types without propagating DNA errors. Previous studies have suggested that mechanisms of genome surveillance, including DNA repair, are superior in mouse embryonic stem cells compared with various differentiated murine cells. Using single-cell gel electrophoresis (comet assay) we found that human embryonic stem cells (BG01, I6) have more efficient repair of different types of DNA damage (generated from H2O2, UV-C, ionizing radiation, or psoralen) than human primary fibroblasts (WI-38, hs27) and, with the exception of UV-C damage, HeLa cells. Microarray gene expression analysis showed that mRNA levels of several DNA repair genes are elevated in human embryonic stem cells compared with their differentiated forms (embryoid bodies). These data suggest that genomic maintenance pathways are enhanced in human embryonic stem cells, relative to differentiated human cells.
Protection against infection in mice vaccinated with a Brucella abortus mutant (1997)

This study determines whether a genetically engineered mutant of Brucella abortus, strain M-1, possesses differences in protective properties compared to the parental strain, vaccine S19. M-1 is a mutant unable to express BP26, a periplasmic protein with potential use in diagnosis. Mice vaccinated with S19 developed antibodies against BP26, while those vaccinated with M-1 did not. However, mice vaccinated with S19 or M-1 were similarly protected against challenge with pathogenic strain 2308, suggesting that the lack of BP26 does not affect the induction of the protective immune response exerted by S19. These and previous results showing that bacterial invasion and growth or replication in mouse spleens were indistinguishable between strains M-1 and S19 could indicate that the mutant is an attenuated strain which maintains the same protective properties as S19.
2D and 3D anisotropic depth migration case histories (2002)

PreviousNext No AccessSEG Technical Program Expanded Abstracts 20022D and 3D anisotropic depth migration case historiesAuthors: Rob VestrumRob VestrumVeritas GeoServices Ltd.https://doi.org/10.1190/1.1816881 SectionsAboutPDF/ePub ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinked InRedditEmail Permalink: https://doi.org/10.1190/1.1816881FiguresReferencesRelatedDetailsCited byDepth migration as a valuable tool for prospect derisking in the Colombian FoothillsSergio Ibanez Poveda, Mario Patino, and John Mathewson5 July 2017 | Interpretation, Vol. 5, No. 4Reflection point sideslip and smear in imaging below dipping anisotropic media1 December 2009 | Geophysical Prospecting, Vol. 58, No. 4The effects of anisotropy on Canadian foothills exploration: A case historyMargaret A. Stratton and Peter Vermeulen6 October 2006Overcoming thrust‐belt imaging problems in Magdalena Valley, ColombiaJon Gittins, Rob Vestrum, and Ralph Gillcrist3 January 2005Sideslip and smear beneath dipping transversely isotropic strataRob Vestrum and Peter Vermeulen3 January 20053D anisotropic depth migration in Blackstone, Alberta: A case historyRob Vestrum, Dave Mackie, and Alana Schoepp3 January 2005Impact of the effects of anisotropy in Canadian foothills exploration: A case historyMargaret A. Stratton3 January 2005 SEG Technical Program Expanded Abstracts 2002ISSN (print):1052-3812 ISSN (online):1949-4645Copyright: 2002 Pages: 2478 publication data© 2002 Copyright © 2002 Society of Exploration GeophysicistsPublisher:Society of Exploration Geophysicists HistoryPublished Online: 03 Jan 2005 CITATION INFORMATION Rob Vestrum, (2002), "2D and 3D anisotropic depth migration case histories," SEG Technical Program Expanded Abstracts : 1256-1259. https://doi.org/10.1190/1.1816881 Plain-Language Summary PDF DownloadLoading ...
Oral Administration in Combination with Zinc Enhances .BETA.-Cryptoxanthin-Induced Anabolic Effects on Bone Components in the Femoral Tissues of Rats in Vivo (2006)

The effects of combined beta-cryptoxanthin and zinc on bone components in the femoral-diaphyseal (cortical bone) and -metaphyseal (trabecular bone) tissues of rats in vivo were investigated. Rats were orally administered either vehicle, beta-cryptoxanthin (5 or 10 microg/100 g body weight), zinc sulfate (0.1 or 0.5 mg Zn/100 g), or their combination once a day for 7 d. Calcium content, alkaline phosphatase activity, and DNA content in the femoral-diaphyseal tissues was not significantly altered by the administration of beta-cryptoxanthin (5 microg/100 g) or zinc (0.1 or 0.5 mg/100 g). Combined administration of beta-cryptoxanthin (5 microg/100 g) and zinc (0.1 or 0.5 mg/100 g) caused a synergistic increase in calcium content, alkaline phosphatase activity, and DNA content in the diaphyseal tissues. The effect of beta-cryptoxanthin (5 or 10 microg/100 g) in increasing calcium and DNA contents in the metaphyseal tissues was significantly enhanced by the combined administration of zinc (0.1 or 0.5 mg/100 g), but did not have a significant effect on the metaphyseal components. The metaphyseal alkaline phosphatase activity was markedly increased by the combination of beta-cryptoxanthin (5 microg/100 g) and zinc (0.1 or 0.5 mg/100 g). This study demonstrates that the oral administration of the combination of zinc at lower doses synergistically enhances beta-cryptoxanthin-induced anabolic effects on bone components in the femoral tissues of rats in vivo.