Miracle-dz/UniFAR-FTUs huggingface dataset:
STAT mutations as program switchers: turning primary immunodeficiencies into autoimmune diseases

STAT proteins are a family of transcription factors that mediate cellular response to cytokines and growth factors. Study of patients with familial susceptibility to pathogens and/or autoimmune diseases has led to the identification of 7 inherited disorders that are caused by mutations of 4 STAT family genes. Homozygous or compound heterozygous mutations of STAT1 lead to complete or partial forms of STAT1 deficiency that are associated with susceptibility to intracellular pathogens and herpetic infections. Patients with heterozygous STAT1 gain‐of‐function (GOF) mutations usually present with chronic mucocutaneous candidiasis (CMC) but may also experience bacterial and viral infections, autoimmune manifestations, lymphopenia, cerebral aneurysms, and increased risk to develop tumors. STAT2 deficiency has been described in 5 family members and is characterized by selective susceptibility to viral infections, whereas STAT3 loss‐of‐function (LOF) mutations are causative of the autosomal‐dominant hyper‐IgE syndrome (HIES), a condition that is characterized by cutaneous and respiratory infections in association with mucocutaneous candidiasis, eczema, skeletal and connective tissue abnormalities, eosinophilia, and high levels IgE. STAT5B LOF and STAT3 GOF mutations are both associated with disorders characterized by autoimmune or allergic manifestations, together with increased risk of infections. Particularly, STAT5b deficiency results in growth hormone (GH) insensitivity, immunodeficiency, diarrhea, and generalized eczema, whereas STAT3 GOF mutations result in autoimmune cytopenia, lymphadenopathy, short stature, infections, enteropathy, and multiorgan autoimmunity, including early‐onset type I diabetes, thyroiditis, hepatitis, arthritis, and interstitial lung disease.
CEO, CFO & CIO Engagement in Information Technology Management: The Disciplinary Effects of Sarbanes-Oxley Information Technology Material Weaknesses

By demonstrating the negative consequences to CEOs, CFOs and CIOs from Sarbanes-Oxley (SOX)related reports regarding firms’ information technology (IT) material weaknesses, we provide evidence supportive of the often-voiced admonition for senior executives to be personally engaged with IT management activities. Further, by linking senior executive turnover to particular categories of IT material weaknesses, we suggest specific areas of IT management of salience to CEOs, CFOs and CIOs. ISR
Biosynthetic Pathway for Mannopeptimycins, Lipoglycopeptide Antibiotics Active against Drug-Resistant Gram-Positive Pathogens

ABSTRACT The mannopeptimycins are a novel class of lipoglycopeptide antibiotics active against multidrug-resistant pathogens with potential as clinically useful antibacterials. This report is the first to describe the biosynthesis of this novel class of mannosylated lipoglycopeptides. Included here are the cloning, sequencing, annotation, and manipulation of the mannopeptimycin biosynthetic gene cluster from Streptomyces hygroscopicus NRRL 30439. Encoded by genes within the mannopeptimycin biosynthetic gene cluster are enzymes responsible for the generation of the hexapeptide core (nonribosomal peptide synthetases [NRPS]) and tailoring reactions (mannosylation, isovalerylation, hydroxylation, and methylation). The NRPS system is noncanonical in that it has six modules utilizing only five amino acid-specific adenylation domains and it lacks a prototypical NRPS macrocyclizing thioesterase domain. Analysis of the mannopeptimycin gene cluster and its engineering has elucidated the mannopeptimycin biosynthetic pathway and provides the framework to make new and improved mannopeptimycins biosynthetically.
Sodium Transport Kinetics, Cell Membrane Lipid Composition, Neural Conduction and Metabolic Control in Type 1 Diabetic Patients

Background: A decreased content of n–3 fatty acids in erythrocyte membrane of type 1 diabetic patients, which is inversely related to plasma levels of HbA1c, has been reported previously. Our aim in this study was to observe the changes after a low-dose n–3 fatty acid (330 mg/day docosahexaenoic acid and 630 mg/day eicosapentanoic acid) dietary intervention in the lipid composition of cell membrane and metabolic control (measured according to plasma HbA1c levels). Since changes in both parameters may alter transmembrane sodium transport or influence parameters measuring target organ damage, we also studied the neural conduction quality and activity of four sodium transporters. Methods: Eighteen type 1 diabetic patients were randomly assigned to continue their usual diet (control group) or to supplement their diet with a daily low dose of n–3 fatty acids (supplemented group). The changes between baseline and end values of the following parameters were compared: HbA1c, lipid and phospholipid composition of cell membrane, activity of four ion carriers and neural conduction quality. Results: The dietary supplementation caused statistically significant changes in membrane lipid composition, particularly an increase of C22:6 (n–3) and the total n–3 fatty acid (respectively +0.90 ± 1.14% vs. –0.44 ± 1.23% and +1.36 ± 1.62% vs. –0.5 ± 1.80%, p < 0.05). After the dietary supplementation, we also observed a significant decrease of HbA1c (–2.00 ± 1.9% vs. –0.13 ± 0.48%, p < 0.05), without significant changes in the dose of insulin required, an increase in the motor conduction velocity by the median nerve (+2.12 ± 1.35 m/s vs. –0.8 ± 2.34 m/s, p < 0.05) and a decrease of the Vmax of the Na+-Li+ countertransport (–96.6 ± 111.2 vs. +58.1 ± 81.3 μmol/l cell/h–1, p < 0.01). Conclusion: A low-dose omega–3 fatty acid dietary supplementation may change the fatty acid composition of the cell membrane and improve the metabolic control of diabetes. Using this dose, we also observed a decrease of the maximal rate of Na+-Li+ countertransport and a slight improvement of neural conduction.
Social Cost of Road Traffic Crashes in India 1

A very large number of high income countries (HICs) have been estimating the costs of road traffic crashes over the past three decades. The methods used and costs allocated have generated a great deal of discussion and debate, in particular because of the difficulty of putting monetary values on pain and suffering. Calculation of direct and indirect costs of injuries, deaths and damage due to road traffic crashes started in the 1970s and many such analyses have been done in USA and Europe The main objective of assessing costs has been to provide an objective tool for help in selecting more cost-effective countermeasures for road safety and also to justify expenditures for the same. However, critics like Hauer1 question the very basic principles of cost-benefit analysis where human lives, pain and suffering are involved. Professionals like Hauer working in this area take the position that putting a monetary value on human life is ethically unacceptable
Active immunization with recombinant V antigen from Yersinia pestis protects mice against plague

The gene encoding V antigen from Yersinia pestis was cloned into the plasmid expression vector pGEX-5X-2. When electroporated into Escherichia coli JM109, the recombinant expressed V antigen as a stable fusion protein with glutathione S-transferase. The glutathione S-transferase-V fusion protein was isolated from recombinant E. coli and cleaved with factor Xa to yield purified V antigen as a stable product. Recombinant V antigen was inoculated intraperitoneally into mice and shown to induce a protective immune response against a subcutaneous challenge with 3.74 x 10(6) CFU of virulent Y. pestis. Protection correlated with the induction of a high titer of serum antibodies and a T-cell response specific for recombinant V antigen. These results indicate that V antigen should be a major component of an improved vaccine for plague.
Multicopper Oxidases and Oxygenases.

Copper is an essential trace element in living systems, present in the parts per million concentration range. It is a key cofactor in a diverse array of biological oxidation-reduction reactions. These involve either outer-sphere electron transfer, as in the blue copper proteins and the Cu{sub A} site of cytochrome oxidase and nitrous oxide redutase, or inner-sphere electron transfer in the binding, activation, and reduction of dioxygen, superoxide, nitrite, and nitrous oxide. Copper sites have historically been divided into three classes based on their spectroscopic features, which reflect the geometric and electronic structure of the active site: type 1 (T1) or blue copper, type 2 (T2) or normal copper, and type 3 (T3) or coupled binuclear copper centers. 428 refs.
When Showrooming Increases Retailer Profit

Showrooming, the phenomenon of consumers visiting a brick-and-mortar (B&M) store to learn about products but then buying online to obtain lower prices, is attracting increased attention both in business practice and in academic literature. It is considered a major threat to the B&M retailers, and determining “how to fight it” seems to be the only consideration. However, the manufacturer's need for retail informational services has always been one of the essential reasons for retailers to exist and is a means for retailers to achieve profitability. The popular arguments about the threat of showrooming ignore the strategic role of the manufacturer in the distribution channel. This article analytically shows that when the manufacturer's decisions are considered (i.e., when the manufacturer–retailer contract is endogenous), consumers’ ability to engage in showrooming may lead to increased, rather than decreased, profitability for B&M retailer(s). Thus, retail efforts to restrict showrooming behavior may be misguided. This result holds even if the manufacturer is restricted to wholesale-only contracts and is not allowed to price discriminate between channels.
Inhibition of delayed hypersensitivity by pre-immunization without complete adjuvant.

Guinea-pigs were immunized by injections of blood group substance with incomplete adjuvant, followed after an interval of approximately 2 weeks, by intracutaneous immunization with the same antigen and Freund's adjuvant containing M. tuberculosis. This treatment inhibited the appearance of delayed skin reactions, while circulating antibody production took place as in controls which had received complete adjuvant only with blood group substance, and had delayed skin reactions. The inhibition of the skin reaction was found to be antigen-specific with regard to unrelated antigens, but showed cross-inhibition for serologically different human blood group substances. The first immunization had to be given more than 2 days before the immunization with complete adjuvant. A similar phenomenon was seen with ovalbumin as antigen. In addition to inhibition of the delayed skin reaction, there appeared to be less γ2-antibody to ovalbumin than in ovalbumin plus complete adjuvant-only controls. Passive administration of antibody did not affect the development of a delayed hypersensitivity state in complete adjuvant-immunized animals with blood group substance or ovalbumin as antigen. Present evidence favours an explanation of the phenomenon in terms of temporary paralysis on the part of some of the antibody-producing cells—viz. those concerned with delayed hypersensitivity and γ2-antibody production.
Superthin O/N/O stacked dielectrics formed by oxidizing thin nitrides in low pressure oxygen for high-density memory devices

High-performance superthin oxide/nitride/oxide (O/N/O) stacked dielectrics have been successfully achieved by oxidizing thin nitride films in low-pressure dry-oxygen at 850/spl deg/C for 30 min. Since the nitrides exhibit a better oxidation resistance to the low-pressure dry-oxygen than to the atmospheric-pressure dry-oxygen and wet-oxygen, the low pressure oxidation obtains a thinner oxidized nitride for the high-density dynamic-random-access-memories (DRAM's) and metal-oxide-nitride-oxide-semiconductor (MONO'S) memory devices. In addition, this dielectric possesses low leakage current and excellent time-dependent-dielectric-breakdown (TDDB) characteristics. Therefore, this novel recipe is promising for future ULSI technology. <<ETX>>
PENGARUH EFEKTIVITAS KOMITE AUDIT TERHADAP FINANCIAL DISTRESS(Studi Empiris pada Perusahaan Manufaktur yang Terdaftar di Bursa Efek Indonesia tahun 2012-2014)

This study investigates the impact of audit committee effectivity on financial distress. This study use three charachteristics of audit committee that is size of audit committee, frequency of audit committee meeting and competence of audit committee. This study use one control variable is leverage. Population that use in this study is 405 manufacture listed firms in Indonesia Stock Exchange in 2012-2014. Based on purposive sampling method, there are 211 samples that consist of 55 financially distressed firms and 156 non financially distressed firms. Financial distress criteria is measure by discriminant value by Altman 1968 (Z-score). Data analysis using logistic regression with SPSS 21. The result from this study show that frequency audit committee meeting has significant negative affect with financial distress
Title: Distinguishing the relevant features of frequent suicide attempters Running title: Features of frequent suicide attempters

Service de Psychologie Medicale et Psychiatrie, CHU Montpellier F -34000, France D epartment in which the work was done : Department of Psychiatry in Ramon y Cajal University Hospital in Madrid and Lapeyronie University Hospital in Montpellier . Contributors Both groups of authors have contrib uted equally to the final manuscript.
Compressive properties of the cartilaginous end‐plate of the baboon lumbar spine

The viscoelastic behavior of the cartilaginous end‐plate of the baboon (Papio anubis) was studied in an experiment on compressive creep. Data were analyzed with the biphasic poroviscoelastic constitutive theory to assess the relative contributions of flow‐dependent and flow‐independent viscoelastic mechanisms to the observed creep behavior. Material coefficients describing the equilibrium compressive behavior (HA) and both flow‐independent (c, τ1, and τ2) and flow‐dependent (K) viscoelastic effects were determined for the end‐plate by the curve‐fitting of the theoretical solution to the experimental creep data. Biochemical analyses were performed to test for potential relationships between material properties and composition which may give rise to the viscoelastic behavior of the end‐plate. The results indicate that the cartilaginous end‐plate has a hydraulic permeability of 14.3 × 10−14 m4/N‐s, which is associated with rapid transport and pressurization of the interstitial fluid in response to loading and an increased emphasis on flow‐independent viscoelastic effects. Biochemical analyses for water, sulfated glycosaminoglycan content, and hydroxyproline indicate that end‐plate of the baboon is compositionally similar of the cartilaginous end‐plate in humans. Interpretation of the mechanical and compositional data suggests that fluid pressurization in the cartilaginous end‐plate may be important in the maintenance of a uniform stress distribution across the boundary between vertebral body and intervertebral disc.
Arabidopsis COP 1 and SPA Genes Are Essential for Plant Elongation But Not for Acceleration of Flowering Time in Response to a Low Red Light to Far-Red Light Ratio 1 [ W ]

Plants sense vegetative shade as a reduction in the ratio of red light to far-red light (R:FR). Arabidopsis (Arabidopsis thaliana) responds to a reduced R:FR with increased elongation of the hypocotyl and the leaf petioles as well as with an acceleration of flowering time. The repressor of light signaling, CONSTITUTIVELY PHOTOMORPHOGENIC1 (COP1), has been shown previously to be essential for the shade-avoidance response in seedlings. Here, we have investigated the roles of COP1 and the COP1-interacting SUPPRESSOR OF PHYA-105 (SPA) proteins in seedling and adult facets of the shade-avoidance response. We show that COP1 and the four SPA genes are essential for hypocotyl and leaf petiole elongation in response to low R:FR, in a fashion that involves the COP1/SPA ubiquitination target LONG HYPOCOTYL IN FR LIGHT1 but not ELONGATED HYPOCOTYL5. In contrast, the acceleration of flowering in response to a low R:FR was normal in cop1 and spa mutants, thus demonstrating that the COP1/ SPA complex is only required for elongation responses to vegetative shade and not for shade-induced early flowering. We further show that spa mutant seedlings fail to exhibit an increase in the transcript levels of the auxin biosynthesis genes YUCCA2 (YUC2), YUC8, and YUC9 in response to low R:FR, suggesting that an increase in auxin biosynthesis in vegetative shade requires SPA function. Consistent with this finding, expression of the auxin-response marker gene DR5::GUS did not increase in spa mutant seedlings exposed to low R:FR. We propose that COP1/SPA activity, via LONGHYPOCOTYL IN FR LIGHT1, is required for shadeinduced modulation of the auxin biosynthesis pathway and thereby enhances cell elongation in low R:FR.
Interlocal Agreements as Overlapping Social Networks: Picket–Fence Regionalism in Metropolitan Kansas City

Public policies addressing complex issues require transjurisdictional solutions, challenging hierarchical modes of public–service delivery. Interlocal agreements (ILAs) are long–established service–delivery instruments for local governments, and research suggests they are plentiful, with a majority of cities and counties involved in at least one ILA. Although ILAs are an established feature of local government operations, previous research is atheoretical, largely descriptive, and unsystematic. This article explores ILAs as social network phenomena, identifying the rationales and underlying values for various ILAs, central and peripheral actors, and brokering roles. In particular, we explore the utility of incorporating network exchange theory into public management network models to identify the relative power of actors in network exchange relationships. We find that a “norm of reciprocity” culture predominates an economizing value as the rationale for an abundance of service–oriented policy networks that produce a picket–fence regionalism of ILA participation in the Kansas City metropolitan area.
Statin Induced Regression of Cardiomyopathy Trial: A Randomized, Placebo-controlled Double-blind Trial

Background: Hypertrophic cardiomyopathy (HCM), characterized by a thickened, fibrotic myocardium, remains the most common cause of sudden cardiac death in young adults. Based on animal and clinical data, we hypothesized that atorvastatin would induce left ventricular (LV) mass regression. Methods: Statin Induced Regression of Cardiomyopathy Trial (SIRCAT) was a randomized, placebo-controlled study. The primary endpoint was change in LV mass measured by cardiac magnetic resonance imaging 12 months after treatment with once-daily atorvastatin 80 mg or placebo. A key secondary endpoint was diastolic dysfunction measured echocardiographically by transmitral flow velocities. SIRCAT is registered with www.clinicaltrials. gov (NCT00317967). Results: Of 222 screened patients, 22 were randomized evenly to atorvastatin and placebo. The mean age was 47 ± 10 years, and 15 (68%) were male. All subjects completed the protocol. At baseline, LV masses were 197 ± 76 g and 205 ± 82 g in the placebo and atorvastatin groups, respectively. After 12 months treatment, the LV masses in the placebo and atorvastatin groups were 196 ± 80 versus 206 ± 92 g (P = 0.80), respectively. Echocardiographic indices were not different in the two groups at baseline. After 12 months, diastolic dysfunction as assessed using transmitral flow velocities E/E', A/A', and peak systolic mitral velocity showed no benefit from atorvastatin. Conclusions: In patients with HCM, atorvastatin did not cause LV mass regression or improvements in LV diastolic function.
The Role of Trust in the Affective Evaluation of Novel Risks: The Case of CO2 Storage

There is general recognition that trust and affect are closely connected concepts. Usually, affect is modeled as an antecedent of trust. In the present research, we will argue that, particularly in new situations, trust can also evoke affect toward a risky object. Using structural equation modeling, support was found for the hypothesis that trust influences attitudes through this process. In the present study, we analyzed attitudes toward (carbon dioxide) CO2 storage. The role of affect appears to be moderated by the level of self‐relevance. In the case of high self‐relevance (storage nearby), people's attitudes appeared to be merely based on affective reactions and trust. This effect is much weaker under low self‐relevance (CO2 storage in general). In such a case, cognitive factors, more particularly beliefs concerning perceived benefits, were also taken into account in attitude formation.
Managing quality inside a high-technology project organization

This action research addresses the deployment of Total Quality Management (TQM) principles in a high-technology new product development organisation. During the period of study, the organisation grew fast. High-technology product development and hypergrowth provided a unique combination of extreme conditions for the study. The existing concepts of TQM are presented as an organised map enabling strategic analysis for an implementation plan. The history of TQM dates back to the manufacturing industry. The key differences between product development as an operating environment and the industrial manufacturing environment are described. The deployment of TQM is described from the perspective of learning theories, leadership theories, studies of organisational culture and studies of teamwork. Based on the learning, a psychodynamic model of organisation is presented for better understanding the challenges of implementation. The results show that, in these specific conditions, organisational culture and leadership are of essential importance for the implementation of TQM. The study also shows that, in the given conditions, TQM is specifically a learning challenge. The results of this study are presented as a framework supporting the selection of TQM implementation objectives and the planning of a strategy for organisational learning.
Bridging Social Capital on Facebook as a Platform: A Case Study of Malaysian College Students

Bridging social capital is a significant predictor in national solidarity. Although Malaysia is known as a fairlysuccessful multi-ethnic country, it still divides along ethnic and religious lines. The underlying question iswhether Social networking sites like Facebook, carry out contributory agent reinforcing bridging social capitalamong different ethnics. The current research will be functional in filling up these gaps by defining the role ofFacebook in individuals’ bridging social capital among Malaysian college students. An online questionnairesurvey used in this study aimed to evaluate the association between the intensity of using Facebook, bridgingsocial capital and different ethnics and age groups. In conclusion, the amount of use of Facebook is aninsignificant factor affecting online bridging social capital among Malaysian undergraduates.