Miracle-dz/UniFAR-FTUs huggingface dataset:
Sudden unilateral hearing loss and vascular loop in the internal auditory canal: case report and review of literature.

Objective We present a patient with unilateral sudden sensorineural hearing loss (SSNHL) who was found to have a vascular loop in the ipsilateral internal auditory canal (IAC), and we review the literature regarding this association. Underlying pathophysiologic factors surrounding microvascular compression of the vestibulocochlear nerve are poorly understood and make treatment recommendations, especially the option of microvascular decompression, difficult if not controversial. The current report represents an attempt to understand this clinical entity as discussed in the current literature.Case summary: A 77-year-old female with a long history of progressive right-sided hearing loss and episodic vertigo developed unilateral right SSNHL, tinnitus, vertigo, and disequilibrium. She was originally diagnosed with a vestibular schwannoma on magnetic resonance imaging (MRI) and was referred to our institution for Gamma Knife radiosurgery. Repeat MRI demonstrated a loop of the anterior inferior cerebellar artery (AICA) compressing the vestibulocochlear nerve within the right IAC. There was no evidence of a schwannoma on the repeat MRI. She was not offered radiosurgery, and she elected conservative management. Conclusion Vascular compression of cranial nerves can lead to neuronal dysfunction, and this has been rarely described in patients involving the vestibulocochlear nerve complex. There is evidence that microvascular decompression (MVD) of the vestibulocochlear nerve can be effective in selected patients who exhibit pulsatile tinnitus or disabling positional vertigo in the setting of a vascular loop within the ipsilateral IAC, but available evidence at this time does not support MVD for SSNHL.
Green, greener, greenest: Identifying ecological trends and leading entities by means of environmental ratings

Abstract In this article, we analyse whether industries worldwide significantly react to the changing environmental demands of their stakeholders. Specifically, we test the hypothesis of increasing environmentally responsible company behaviour in recent years. We do this by constructing environmental industry ratings based on a novel data-set covering three geographic regions and 42 industries from 2009 to 2015 and by fitting robust trend regression models to these ratings. Interestingly, we cannot observe a general upward trend in all industries and regions. In other words, the ‘green wave’ does not appear to carry all business sectors. This becomes particularly clear when looking at rankings derived from estimated trends. Industries in Europe show especially significant downward trends. Even though most European industries are currently rated higher than their counterparts in the Asia-Pacific region and North America, these trends indicate that Europe may lose this leading position in the future. Besides delivering such general tendencies, our estimated trends combined with the current rating levels provide important decision support. They show which industry sectors and regions are particularly (un)interesting for socially responsible investment products, ecologically conscious applicants or environmentally responsible consumers.
Functional Reconstitution in Situ of 5-Hydroxytryptamine2c (5HT2c) Receptors with αq and Inverse Agonism of 5HT2c Receptor Antagonists*

Membranes prepared after infection of Sf9 cells with recombinant baculovirus containing the rat 5HT2c receptor DNA, but not after infection with wild-type virus, expressed high affinity binding sites for 125I-lysergic acid diethylamide and [3H]mesulergine. The receptor site density reached an optimum of 50-70 pmol/mg membrane protein at 60 h postinfection. Extraction of peripheral membrane proteins from the postnuclear membrane fraction with 6 M urea depleted GTPγS-binding 4-fold without decreasing 5HT2c receptor binding activity. Urea-extracted Sf9 membranes expressing the 5HT2c receptor catalyzed the activation of squid retinal αq but not bovine retinal αt or bovine αo/αi. Productive interaction of 5HT2c receptors with squid αq was enhanced by the addition of βγ dimers prepared from either bovine brain or bovine rod outer segment discs. While the addition of serotonin increased 5HT2c receptor-catalyzed GTPγS binding to αq, the unoccupied receptor was also catalytically active. The 5HT2c receptor antagonists, mesulergine, mianserin, and ketanserin competitively inhibited 5HT activation of the receptor with predicted rank-order affinities; and mianserin and ketanserin markedly inhibited basal 5HT2c receptor activity. Interestingly, this “inverse agonist” efficacy did not correlate with antagonist affinity for the 5HT2c receptor. Baculoviral expression of the 5HT2c receptor and urea extraction of postnuclear Sf9 cell membranes have provided a high density of in situ, uncoupled, G-protein-linked receptor useful for reconstitution with purified G-protein subunits. This has allowed for independent manipulation of receptor and G-protein chemical concentrations and has revealed that a G-protein-linked receptor can possess a significant basal catalytic activity and that antagonist compounds can act as inverse agonists of this basal activity at the level of receptor activation of G-proteins.
The relationship between distal tubular proton secretion and dietary potassium depletion: evidence for up-regulation of H+ -ATPase.

BACKGROUND Dietary potassium depletion is associated with elevated plasma bicarbonate concentration and enhanced bicarbonate reabsorption in the distal tubule. The relationship between distal proton secretion and potassium status was investigated by in vivo microperfusion of the superficial distal tubule. METHODS Experiments were performed on anaesthetized rats that had been maintained on either a low-potassium or control diet for 3-5 weeks prior to experimentation. The distal tubules were perfused at 10 nl/min with either a standard or a barium chloride-containing solution, and the late distal tubular transepithelial potential difference (Vte) and pH of the luminal fluid were recorded using a double-barrelled voltage and ion-sensitive microelectrode. RESULTS In control rats, the Vte was -40.7+/-2.4 mV and the tubular fluid pH was 6.44+/-0.07; in potassium-depleted animals, the Vte was -15.0+/-1.4 mV and the pH was 6.76+/-0.03. The pH values in both groups of animals were significantly lower than would be predicted from the Vte and systemic pH for passive H+ distribution, indicating active proton secretion. Moreover, in hypokalaemic rats, this difference from predicted pH was significantly greater than in control animals (control = 0.27+/-0.06 vs. low-potassium = 0.46+/-0.03; P<0.01), suggesting enhanced active proton secretion. During perfusion with a solution containing BaCl2, the late distal tubule Vte became lumen positive in potassium-depleted rats, contrasting with an increased lumen negativity in potassium-replete controls. The barium-induced lumen-positive potential difference observed in the hypokalaemic rats was abolished by intravenous administration of acetazolamide. CONCLUSION These data are consistent with enhanced electrogenic proton secretion (H+ -ATPase) during dietary potassium deprivation.
The role of molecular testing and enzyme analysis in the management of hypomorphic citrullinemia

Expanded newborn screening detects patients with modest elevations in citrulline; however it is currently unclear how to treat these patients and how to counsel their parents. In order to begin to address these issues, we compared the clinical, biochemical, and molecular features of 10 patients with mildly elevated citrulline levels. Three patients presented with clinical illness whereas seven came to attention as a result of expanded newborn screening. One patient presented during pregnancy and responded promptly to IV sodium phenylacetate/sodium benzoate and arginine therapy with no long‐term adverse effects on mother or fetus. Two children presented with neurocognitive dysfunction, one of these responded dramatically to dietary protein reduction. ASS enzyme activity was not deficient in all patients with biallelic mutations suggesting this test cannot exclude the ASS1 locus in patients with mildly elevated plasma citrulline. Conversely, all symptomatic patients who were tested had deficient activity. We describe four unreported mutations (p.Y291S, p.R272H, p.F72L, and p.L88I), as well as the common p.W179R mutation. In silico algorithms were inconsistent in predicting the pathogenicity of mutations. The cognitive benefit in one patient of protein restriction and the lack of adverse outcome in seven others restricted from birth, suggest a role for protein restriction and continued monitoring to prevent neurocognitive dysfunction. © 2008 Wiley‐Liss, Inc.
Protein Geranylgeranylation Is Required for Osteoclast Formation, Function, and Survival: Inhibition by Bisphosphonates and GGTI‐298

Bisphosphonates are the important class of antiresorptive drugs used in the treatment of metabolic bone diseases. Although their molecular mechanism of action has not been fully elucidated, recent studies have shown that the nitrogen‐containing bisphosphonates can inhibit protein prenylation in macrophages in vitro. In this study, we show that the nitrogen‐containing bisphosphonates risedronate, zoledronate, ibandronate, alendronate, and pamidronate (but not the non nitrogen‐containing bisphosphonates clodronate, etidronate, and tiludronate) prevent the incorporation of [14C]mevalonate into prenylated (farnesylated and geranylgeranylated) proteins in purified rabbit osteoclasts. The inhibitory effect of nitrogen‐containing bisphosphonates on bone resorption is likely to result largely from the loss of geranylgeranylated proteins rather than loss of farnesylated proteins in osteoclasts, because concentrations of GGTI‐298 (a specific inhibitor of geranylgeranyl transferase I) that inhibited protein geranylgeranylation in purified rabbit osteoclasts prevented osteoclast formation in murine bone marrow cultures, disrupted the osteoclast cytoskeleton, inhibited bone resorption, and induced apoptosis in isolated chick and rabbit osteoclasts in vitro. By contrast, concentrations of FTI‐277 (a specific inhibitor of farnesyl transferase) that prevented protein farnesylation in purified rabbit osteoclasts had little effect on osteoclast morphology or apoptosis and did not inhibit bone resorption. These results therefore show the molecular mechanism of action of nitrogen‐containing bisphosphonate drugs in osteoclasts and highlight the fundamental importance of geranylgeranylated proteins in osteoclast formation and function.
Evaluation of suppressive and pro-resolving effects of EPA and DHA in human primary monocytes and T-helper cells[S]

Despite their beneficial anti-inflammatory properties, eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) may increase the infection risk at high doses, likely by generating an immune-depressed state. To assess the contribution of different immune cell populations to the immunomodulatory fatty acid effect, we comparatively investigated several aspects of inflammation in human T-helper (Th) cells and monocytes. Both fatty acids, but DHA to a lesser extent compared with EPA, selectively and dose-dependently reduced the percentage of cytokine-expressing Th cells in a peroxisome proliferator-activated receptor (PPAR)γ-dependent fashion, whereas the expression of the cell surface marker CD69 was unaltered on activated T cells. In monocytes, both EPA and DHA increased interleukin (IL)-10 without affecting tumor necrosis factor (TNF)-α and IL-6. Cellular incorporation of EPA and DHA occurred mainly at the expense of arachidonic acid. Concomitantly, thromboxane B (TXB)2 and leukotriene B (LTB)4 in supernatants decreased, while levels of TXB3 and LTB5 increased. This increase was independent of activation and in accordance with cyclooxygenase expression patterns in monocytes. Moreover, EPA and DHA gave rise to a variety of mono- and trihydroxy derivatives of highly anti-inflammatory potential, such as resolvins and their precursors. Our results suggest that EPA and DHA do not generally affect immune cell functions in an inhibitory manner but rather promote pro-resolving responses.
The oxidative stress response in Enterococcus faecalis: relationship between H2O2 tolerance and H2O2 stress proteins

The hydrogen peroxide (H2O2) stress response in Enterococcus faecalis ATCC19433 was investigated. A 2·4 mmol l−1 H2O2 pretreatment conferred protection against a lethal concentration (45 mmol l−1) of this agent. The relatively high concentrations of H2O2 used for adaptation and challenge treatments in Ent. faecalis emphasised the strong resistance towards oxidative stress in this species. Various stresses (NaCl, heat, ethanol, acidity and alkalinity) induced weak or strong H2O2 cross‐protection. This paper describes the involvement of protein synthesis in the active response to lethal dose of H2O2, in addition to the impressive enhancement of synthesis of five H2O2 stress proteins. Combined results suggest that these proteins might play an important role in the H2O2 tolerance response.
p53+/mdm2− Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma in Young Children: An Early Expression of Li-Fraumeni Syndrome

The spectrum of lipomatous tumors differs in the adult and pediatric populations, with liposarcoma being rare in children. Nearly 10% of individuals with Li-Fraumeni syndrome develop sarcomas in the first 2 decades of life; however, the frequency of sarcoma types and subtypes in this syndrome is unknown. Two atypical lipomatous tumors/well-differentiated liposarcomas (ALT/WDLS) were identified in the pathology files of our institution in young children from “classical” Li-Fraumeni and Li-Fraumeni variant kindreds with a known germline TP53 mutation (Y220C) in one of the families. The patients were 5 and 6 years of age and the ALT/WDLSs were the first expression of the syndrome. The tumors had a high degree of cellular atypia and differed from sporadic ALT/WDLS by strong nuclear immunoreactivity for p53 and absent mdm2 expression. This is the first report of 2 ALT/WDLSs presenting in children before 10 years of age, both in association with Li-Fraumeni syndrome/variant. ALT/WDLS in a young child should raise the possibility of a cancer predisposition syndrome and, in this setting, the p53+/mdm2− immunophenotype might be characteristic. Recognition of this lesion and its association is important for early diagnosis and subsequent tumor surveillance in the proband and affected family members.
PATTERNS OF INTRASPECIFIC DIVERSIFICATION IN THE PIRIQUETA CAROLINIANA COMPLEX IN SOUTHEASTERN NORTH AMERICA AND THE BAHAMAS

Abstract We use chloroplast DNA (cpDNA) variation and nested clade phylogeographic analyses to infer the historical processes that have contributed to the high level of morphological and ecological diversification present in a group of herbaceous perennials (the Piriqueta caroliniana complex) in North America and the Bahamas. The presence of morphologically distinct and intercompatible varieties (morphotypes) that can be distinguished based on suites of taxonomic characters (e.g., leaf shape, pubescence type, stature) and contrasting habitat affinities (from marshes to dry pinelands and sand scrub) makes this group particularly appropriate for studies of intraspecific diversification. To examine the distribution of haplotypes among populations, we sampled 467 individuals from 55 locations in Florida, Georgia, and the northern Bahamas (Grand Bahama and Abaco) and screened each individual for cpDNA variation using restriction fragment length polymorphism (RFLP) and heteroduplex analyses. We develop a one-step haplotype phylogeny for this group and use the geographic distributions of haplotypes and clades to test specific phylogeographic hypotheses using the methods developed by Templeton and his colleagues (Templeton 1998). In general, the distribution of haplotypes was strongly influenced by limited dispersal distances, with the more recently derived haplotypes having much lower levels of dispersion and lower frequencies in populations than the ancestral haplotypes. The patterns of clade and haplotype dispersion and displacement and the distribution of morphotypes imply at least three cases of long-distance dispersal and one case of historical fragmentation. The historical patterns inferred for populations of Piriqueta are consistent with known biogeographical events, historical vegetation change, and the concordant patterns of multiple Pleistocene refugia that have been observed for a number of other taxa in southeastern North America. Corresponding Editor: M. Dudash
Drivers of Populism: A Four-country Comparison of Party Communication in the Run-up to the 2014 European Parliament Elections

The aim of this article is to analyse what different types of populist communication styles emerged during the 2014 European Parliament election campaign and under which conditions political parties selected specific populist communication styles. To do this, a comparative quantitative content analysis of press releases in the run-up of the latest European Parliament elections has been conducted for parties in France, Germany, Austria and Greece. The article presents a definition of populism based on the contemporary academic discourse, which focuses on the transnational nature of the European political field. It is shown that populist party communication is more pronounced on the fringes of the political spectrum and in countries struggling with severe macroeconomic difficulties. Contrary to intuitive expectations, the perceived populist rhetoric of exclusivity in the context of the European sovereign debt crisis, which is identified as a central feature of right-wing populism, barely takes place within populist party communication.
The value of crowdsourcing in public policymaking: epistemic, democratic and economic value

ABSTRACT While national and local governments increasingly deploy crowdsourcing in lawmaking as an open government practice, it remains unclear how crowdsourcing creates value when it is applied in policymaking. Therefore, in this article, we examine value creation in crowdsourcing for public policymaking. We introduce a framework for analysing value creation in public policymaking in the following three dimensions: democratic, epistemic and economic. Democratic value is created by increasing transparency, accountability, inclusiveness and deliberation in crowdsourced policymaking. Epistemic value is developed when crowdsourcing serves as a knowledge search mechanism and a learning context. Economic value is created when crowdsourcing makes knowledge search in policymaking more efficient and enables government to produce policies that better address citizens’ needs and societal issues. We show how these tenets of value creation are manifest in crowdsourced policymaking by drawing on instances of crowdsourced lawmaking, and we also discuss the contingencies and challenges preventing value creation.
An empirical analysis of ISO 9001:2008 application in Italian services and manufacturing companies

The certification of Quality System is a contractual requirement that is sometimes essential in business relationships. ISO 9001 consolidates governance and represents the basis for the implementation of other management systems, as well as for the adoption of schemes or standards in specialist sectors. Moreover, the scientific literature focused on the benefits and barriers of ISO 9001 application is often in disagreement and strongly differs in dependence of countries and sectors. Based on these considerations, the aim of this paper is to realise a comparative analysis between Services Companies (SeCo) and Manufacturing Companies (MaCo), in order to identify the advantages and disadvantages perceived from ISO 9001:2008 applications. The research was carried out through a questionnaire proposed to 1692 Italian Companies (response rate = 15%). The results clearly showed that SeCo and MaCo present few statistical differences in the benefits and limitations derived from ISO 9001:2008 applications; the main perceived benefits were a greater awareness of business opportunities in terms of continuous improvement, and the increase in business results in terms of organisation; increasing complexity of procedures in a company and bureaucratisation is the greatest disadvantage. Finally, the companies’ expectations for the new version ISO 9001:2015 were analysed.
Tipping the Scales ‐ Specifier Proteins in Glucosinolate Hydrolysis

Glucosinolates are a group of secondary plant metabolites found in the Brassicales order that are beneficial components of our diet, determine the flavor of a number of vegetables and spices and have been implicated in pest management strategies. These properties, most of the biological activities and the pungent odor and taste associated with glucosinolate‐containing plants are due to the products formed from glucosinolates by their hydrolytic enzymes, myrosinases, upon tissue disruption. Specifier proteins impact the outcome of glucosinolate hydrolysis without having hydrolytic activity on glucosinolates themselves. In the presence of specifier proteins, glucosinolate hydrolysis results in nitriles, epithionitriles and organic thiocyanates whose biological functions are currently unknown. In contrast, isothiocyanates formed in the absence of specifier proteins have been demonstrated to possess a variety of biological activities and are thought to protect plants from herbivore and pathogen attack. This review discusses the current knowledge on plant and insect specifier proteins with special emphasis on their biochemical properties and possible mechanisms of action.
Enhancing the Effects of "The Day After" with an Educational Intervention.

ABSTRACT The major purpose of this study was to investigate the possible enhancement of the effects of the movie "The Day After" via an educational lecture on the arms race that was given seven days after the movie presentation. Subjects were 370 college students in ten introductory psychology classes. A Nuclear Weapons Policies questionnaire was administered to students before and immediately after the telecast of the movie. Seven days after the movie telecast, four class sections attended a previously scheduled lecture on the psychological aspects of the nuclear arms race. The questionnaire was again administered to all students ten days after the movie was televised. Results showed that the lecture did not enhance the effects of the movie. The lecture was then modified by introducing more psychologically oriented information and tested on another group of 241 students. In addition to replicating most of the effects produced by the film and the film with lecture, the modification of the lecture format produced significant changes in estimates of the probability of nuclear war, and most importantly, in arms control opinions. (RN)