Miracle-dz/UniFAR-FTUs huggingface dataset:
Characterisation of the Cullin-3 mutation that causes a severe form of familial hypertension and hyperkalaemia

Deletion of exon 9 from Cullin‐3 (CUL3, residues 403–459: CUL3Δ403–459) causes pseudohypoaldosteronism type IIE (PHA2E), a severe form of familial hyperkalaemia and hypertension (FHHt). CUL3 binds the RING protein RBX1 and various substrate adaptors to form Cullin‐RING‐ubiquitin‐ligase complexes. Bound to KLHL3, CUL3‐RBX1 ubiquitylates WNK kinases, promoting their ubiquitin‐mediated proteasomal degradation. Since WNK kinases activate Na/Cl co‐transporters to promote salt retention, CUL3 regulates blood pressure. Mutations in both KLHL3 and WNK kinases cause PHA2 by disrupting Cullin‐RING‐ligase formation. We report here that the PHA2E mutant, CUL3Δ403–459, is severely compromised in its ability to ubiquitylate WNKs, possibly due to altered structural flexibility. Instead, CUL3Δ403–459 auto‐ubiquitylates and loses interaction with two important Cullin regulators: the COP9‐signalosome and CAND1. A novel knock‐in mouse model of CUL3WT/Δ403–459 closely recapitulates the human PHA2E phenotype. These mice also show changes in the arterial pulse waveform, suggesting a vascular contribution to their hypertension not reported in previous FHHt models. These findings may explain the severity of the FHHt phenotype caused by CUL3 mutations compared to those reported in KLHL3 or WNK kinases.
Quantity over quality: light intensity, but not red/far‐red ratio, affects extrafloral nectar production in Senna mexicana var. chapmanii

Abstract Extrafloral nectar (EFN) mediates food‐for‐protection mutualisms between plants and insects and provides plants with a form of indirect defense against herbivory. Understanding sources of variation in EFN production is important because such variations affect the number and identity of insect visitors and the effectiveness of plant defense. Light represents a potentially crucial tool for regulating resource allocation to defense, as it not only contributes energy but may help plants to anticipate future conditions. Low red/far‐red (R/FR) light ratios can act as a signal of the proximity of competing plants. Exposure to such light ratios has been shown to promote competitive behaviors that coincide with reduced resource allocation to direct chemical defenses. Little is known, however, about how such informational light signals might affect indirect defenses such as EFN, and the interactions that they mediate. Through controlled glasshouse experiments, we investigated the effects of light intensity, and R/FR light ratios, on EFN production in Senna mexicana var. chapmanii. Plants in light‐limited conditions produced significantly less EFN, and leaf damage elicited increased EFN production regardless of light conditions. Ratios of R/FR light, however, did not appear to affect EFN production in either damaged or undamaged plants. Understanding the effects of light on indirect defenses is of particular importance for plants in the threatened pine rockland habitats of south Florida, where light conditions are changing in predictable ways following extensive fragmentation and subsequent mismanagement. Around 27% of species in these habitats produce EFN and may rely on insect communities for defense.
Variation-Tolerance of a 65-nm Error-Hardened Dual-Modular-Redundancy Flip-Flop Measured by Shift-Register-Based Monitor Structures

We show measurement results of variation-tolerance of an error-hardened dual-modular-redundancy flip-flop fabricated in a 65-nm process. The proposed error-hardened FF called BCDMR is very strong against soft errors and also robust to process variations. We propose a shift-register-based test structure to measure variations. The proposed test structure has features of constant pin count and fast measurement time. A 65nm chip was fabricated including 40k FFs to measure variations. The variations of the proposed BCDMR FF are 74% and 55% smaller than those of the conventional BISER FF on the twin-well and triple-well structures respectively.
A critical review of the current evidence examining whether resistance training improves time trial performance

ABSTRACT A number of reviews have concluded that resistance training is beneficial for improving sports performance despite the inclusion of studies which do not actually measure a performance outcome (i.e. a timed trial). The purpose of this review was to examine only those studies which would allow us to infer the benefits of resistance training on improving time trial performance. Of the nine studies meeting all inclusion criteria only three demonstrated an additive effect of adding resistance training to the current activity-specific training being performed. These three studies demonstrated improvements in either 5 or 10 km time trial among recreationally skilled athletes (i.e. non-elite level time). Previous reviews have included studies which did not include: (1) performance outcomes; (2) control groups; and/or (3) equal volumes of activity-specific exercise among the resistance training and control groups. Presently, there is little evidence that adding resistance exercise to a sport-specific training program will augment time trial performance. While it is difficult to perform such long-term studies assessing the effects of resistance training among time trial athletes, the statement that resistance training is efficacious for improving time trial performance should be tempered until sufficient evidence is presented to support such claims.
Forest Products Industry in a Digital Age: Factors Affecting Social Media Adoption

Abstract The use of social media as a marketing tool has increased significantly in recent years. However, limited information is available regarding social media use in the US forest products industry or social media adoption at the organizational level, especially within the business-to-business context. This study presents part two of a two-part series of articles that look at the forest products industry in the digital age. A mail survey was conducted in 2013 to examine factors affecting the use of social media in the US forest products industry. This article also looks at the perception of forest products companies regarding social media effectiveness and identifies challenges faced by the forest products companies regarding social media use. Results show that close to 58 percent of respondents currently use some form of social media. The most common social media tool implemented was Facebook. Respondents' adoption of social media was influenced by company age, net sales revenue, product type, Web si.
The Intricacies of the Relationship Between Pay and Performance for Teachers : Do teachers respond to Performance Related Pay schemes ?

Performance related pay (PRP) has been introduced for teachers in the UK, as part of a drive to improve the outcome of public service and teaching in particular. For the introduced individual PRP scheme to be effective, teachers must have an effect on pupil attainment, must respond to financial incentives, must respond to performance based pay and within this to individual PRP schemes. This review examines evidence on these issues, drawing in particular from the experience of the USA. We conclude that individual PRP schemes can result in small positive gains in pupil attainment, but that the current operation of the UK scheme is more akin to a general pay rise for eligible teachers than a PRP scheme. JEL Classification: I2, J4
Emerging Trends in Epidemiology of Hepatitis B Virus Infection

Abstract Although a vaccine against hepatitis B virus (HBV) has been available since 1982, the prevalence of adults with chronic HBV infection in sub-Saharan Africa and East Asia is still estimated at 5–10%. A high rate of chronic infections is also found in the Amazon and the southern parts of eastern and central Europe. In the Middle East and the Indian subcontinent, the prevalence is 2–5%. Less than 1% of the population of Western Europe and North America is chronically infected. Given the high prevalence of infections (such as hepatitis) among inmates, prison is considered a reservoir for facilitating such infections. Based on these premises, this current review examines and discusses emerging trends in the epidemiology of HBV infection, with particular attention to HBV infection in prison. The hepatitis B surface antigen (HBsAg) prevalence in prisoners in west and central Africa is very high (23.5%). The Centers for Disease Control and Prevention has highlighted the importance of HBV blood screening and subsequent anti-HBV vaccination in the prison population. The vaccination was recommended for all inmates, representing an opportunity to prevent HBV infection in a high-risk population. In these subjects, an accelerated hepatitis B immunisation schedule may result in rapid seroconversion for early short-term protection. Therefore, it is necessary to seek collaboration among public health officials, clinicians and correctional authorities to implement a vaccination programme.
Associations of size at birth and dual-energy X-ray absorptiometry measures of lean and fat mass at 9 to 10 y of age.

BACKGROUND Birth weight has been positively associated with risk of overweight in later life. However, little information exists on how weight and length at birth are associated with subsequent lean and total body fat. OBJECTIVE We investigated the association between weight and length at birth and body composition and fat distribution in childhood. DESIGN Body composition was measured by using dual-energy X-ray absorptiometry in 9-10-y-old subjects (n = 3006 boys and 3080 girls). Weight and length at birth were measured or taken from hospital records. RESULTS Birth weight was positively associated with both lean body mass (LBM) and total body fat at 9-10 y of age in both sexes. LBM rose by 320 g per 1-SD increase in birth weight (P < 0.001), and total body fat rose by 2.5% (P = 0.001), but birth weight was unassociated with the fat-to-lean mass ratio (FLR). Ponderal index (PI) at birth (ie, weight/length3) was positively associated with LBM, total body fat, and the FLR in both sexes; the FLR increased by 2.7% in boys (P = 0.021) and by 5.0% in girls per 1-SD increase in PI (P < 0.001). Weight and length at birth did not predict central adiposity; although trunk fat had a strong positive association with PI at birth, this association disappeared after adjustment for total body fat. CONCLUSIONS Higher PI at birth is associated with both higher fat and lean mass in childhood but also with an increase in the FLR. PI at birth is a better predictor of subsequent adiposity than is birth weight.
Genetic Disorders with Tau Pathology: A Review of the Literature and Report of Two Patients with Tauopathy and Positive Family Histories

Background: Tauopathies are a group of neurodegenerative disorders characterized by the pathological accumulation of hyperphosphorylated and insoluble tau protein within neurons and glia. Although most cases are sporadic, hereditary tauopathies have also been reported. Summary: In this article, we review genetic disorders in which tau pathology has been reported and present two novel families with primary tauopathies. Mutations in the microtubule-associated protein tau gene (MAPT) cause a small subset of primary tauopathies. Mutations in 21 other genes and an 18q deletion syndrome have also been reported to be associated with tau pathology reminiscent of Alzheimer's disease, corticobasal degeneration, progressive supranuclear palsy, argyrophilic grain disease or Pick's disease. In 8 of the 21 genes, tau pathology was only seen in cases with some ‘specific' mutations. In the remaining genes, tau pathology, often in the form of Alzheimer-type neurofibrillary lesions, was a common finding but was ‘not mutation specific'. The probands of the two families were diagnosed with progressive supranuclear palsy based on clinicopathological evaluation. Their family histories were relevant for parkinsonism in 3 siblings of family 1 and 1 brother and the father from family 2, but these were not autopsy-confirmed. DNA from the brains of the probands from these families was screened for MAPT and leucine-rich repeat kinase 2 gene mutations, but no mutations were identified. Key Messages:MAPT mutations are a cause of familial tauopathies, but other genes have also been associated with tau pathology. Novel genes still await discovery.
Severe Cutaneous Adverse Reactions

Severe cutaneous adverse reactions (SCARs) are generally induced by drugs and encompass the conditions of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), drug induced eosinophilia and systemic syndrome (DRESS) also known as drug induced hypersensitivity syndrome (DIHS), and acute generalized exanthematous pustulosis (AGEP). These conditions, although rare, cause significant morbidity and are potentially fatal. It is therefore important for the treating physician to promptly recognize SCARs through the identification of their characteristic clinical features so that the offending drug is promptly withdrawn and supportive and adjunctive therapies are administered. SCARs are accompanied by particular abnormalities on routine laboratory investigations and skin biopsy that enables confirmation of the diagnosis and provision of useful prognostic information. Data bases have been established, predominantly in Europe, since the 1980s to characterize the epidemiology of SCARs including the identification of drugs with the highest relative risk and the latency between the commencement of drug intake and the onset of clinical manifestations. The pathogenesis of the various SCARs involves delayed T cell-mediated inflammation in a genetically predisposed individual and in the case of DIHS, may involve viral factors. The emerging field of the genetic susceptibility to SCARs has raised the important issue of pharmacogenetic screening as a method of predicting an individual’s risk of developing SCAR to a certain drug.
Nonparametric Estimation of the Volatility Function in a High-Frequency Model corrupted by Noise

We consider the models Yi;n = R i=n 0 (s)dWs + (i=n) i;n, and ~ Yi;n = (i=n)Wi=n + (i=n) i;n, i = 1;:::;n, where (Wt) t2[0;1] denotes a standard Brownian motion and i;n are centered i.i. d. random variables with E 2 = 1 and nite fourth moment. Furthermore, and are unknown deterministic functions and (Wt) t2[0;1] and ( 1;n;:::; n;n) are assumed to be independent processes. Based on a spectral decomposition of the covariance structures we derive series estimators for 2 and 2 and investigate their rate of convergence of the MISE in dependence of their smoothness. To this end specic basis functions and their corresponding Sobolev ellipsoids are introduced and we show that our estimators are optimal in minimax sense. Our work is motivated by microstructure noise models. A major nding is that the microstructure noise i;n introduces an additionally degree of ill-posedness of 1=2; irrespectively of the tail behavior of i;n. The performance of the estimates is illustrated by a small numerical study.
Correlation between atomic-scale structure and mobility anisotropy in superlattices

We have performed detailed characterization of atomic-scale interface structure in InAs/Ga12xInxSb superlattices using cross-sectional scanning tunneling microscopy ~STM! and established a semiquantitative correlation between interface structure and transport properties in these structures. Quantitative analysis of STM images of both ~110! and (11̄0) cross-sectional planes of the superlattice indicates that interfaces in the (11̄0) plane exhibit a higher degree of interface roughness than those in the ~110! plane and that the Ga12xInxSb-on-InAs interfaces are rougher than the InAs-on-Ga12xInxSb interfaces. The roughness data are consistent with anisotropy in interface structure arising from anisotropic island formation during growth and, in addition, a growth-sequence-dependent interface structure arising from differences in interfacial bond structure between the two interfaces. Low-temperature Hall measurements performed on these samples demonstrate the existence of a substantial lateral anisotropy in mobility that is in semiquantitative agreement with modeling of interface roughness scattering that incorporates our quantitative measurements of interface roughness using STM. @S0163-1829~98! 03911-3#
Opisthorchiasis and viral hepatitis b: clinical cases.

OBJECTIVE Introduction: Among the cases of biohelminthosis, which are registered in Ukraine, opisthorchiasis is the most common and constitutes the second largest centre in Europe. Due to the growth of migration processes and global tourism, opisthorchiasis is becoming increasingly relevant for the countries of the European Union and the United States. Under modern conditions, the clinical course of many infectious and parasitic diseases has changed. The aim: The present paper discusses and analyzes the cases of opisthorchiasis and hepatitis B virus which are challenging in terms of diagnostics and choice of treatment tactics. CONCLUSION Conclusion: The course of acute infections, the formation of results and the effectiveness of treatment are influenced by a number of factors, among which mixed infections are of particular interest.
High folate intake is associated with lower breast cancer incidence in postmenopausal women in the Malmö Diet and Cancer cohort.

BACKGROUND Epidemiologic studies of associations between folate intake and breast cancer are inconclusive, but folate and other plant food nutrients appear protective in women at elevated risk. OBJECTIVE The objective was to examine the association between folate intake and the incidence of postmenopausal breast cancer. DESIGN This prospective study included all women aged >or=50 y (n = 11699) from the Malmö Diet and Cancer cohort. The mean follow-up time was 9.5 y. We used a modified diet-history method to collect nutrient intake data. At the end of follow-up, 392 incident invasive breast cancer cases were verified. We used proportional hazard regression to calculate hazard ratios (HRs). RESULTS Compared with the lowest quintile, the incidence of invasive breast cancer was reduced in the highest quintile of dietary folate intake (HR: 0.56; 95% CI: 0.35, 0.90; P for trend = 0.02); total folate intake, including supplements (HR: 0.56; 95% CI: 0.34, 0.91; P for trend = 0.006); and dietary folate equivalents (HR: 0.59; 95% CI: 0.36, 0.97; P for trend = 0.01). CONCLUSION A high folate intake was associated with a lower incidence of postmenopausal breast cancer in this cohort.
Automatic recognition of Turkish fingerspelling

Fingerspelling is a manual representation of alphabet letters and used in sign language to spell out words, especially private names. In Turkish Sign Language it is a challenging task due to the ambiguity of the fingerspelling representations of the letters with diacritic marks and complex hand configurations. In this paper we propose a Turkish fingerspelling recognition system based on extraction of the most effective features that help disambiguation of the signs. Our approach is fundamentally based on feature extraction by Histograms of Oriented Gradients (HOG) and dimension reduction by Principal Component Analysis (PCA). Use of internal features instead of outer projection of the image enhances the performance on disambiguation. The test dataset consists of 493 fingerspelling images created by 4 different signers and the test results indicate an average success rate of classification of 99.39%.
Association of sirtuins with clinicopathological parameters and overall survival in gastric cancer

To evaluate the associations of sirtuins (SIRT1-7) with clinicopathological parameters in gastric cancer, sirtuins expression profile in NCBI GEO datasets, GSE62254 and GSE15459, was integrated and analyzed. The results suggested that SIRT4, SIRT6, and SIRT7 were associated with Lauren classification and SIRT3-5 were associated with pStage in gastric cancer. Then an online database derived from 1,065 gastric cancer cases, Kaplan-Meier plotter, was used to explore the associations of the mRNA levels of sirtuins with overall survival in gastric cancer. Survival curves generated from Kaplan-Meier plotter suggested that high expression of SIRT1 mRNA was favorable for overall survival in gastric cancer (SIRT1: HR = 0.64, 95% CI = 0.54–0.76, P = 2.2E-07), high expressions of SIRT2-4 and SIRT6-7 were poor for overall survival (SIRT2: HR = 2.31, 95% CI = 1.87–2.87, P = 3.6E-15; SIRT3: HR = 1.99, 95% CI = 1.62–2.45, P = 2.6E-11; SIRT4: HR = 1.41, 95% CI = 1.19–1.68, P = 6.6E-05; SIRT6: HR = 2.02, 95% CI = 1.66–2.47, P = 1.7E-12; SIRT7: HR = 1.96, 95% CI = 1.63–2.35, P = 2.7E-13), whereas no significant association existed between SIRT5 mRNA expression and overall survival. Further analyses stratified by gender, stages, Lauren classification, differentiation, treatment, and HER2 status were also performed. In summary, high SIRT1 mRNA level was associated with better overall survival, SIRT2-4 and 6–7 were associated with poor overall survival, whereas SIRT5 did not show significant association with overall survival in gastric cancer.
The Validation of the Interactive Metronome: A Pilot Study Prior to Implementation for Post Deployment Service Members

The purpose of this study was to validate the protocols created for the Interactive Metronome TM (IM) used in conjunction with the TRX® System for returning post deployment service members diagnosed with mild traumatic brain and/or Post-Traumatic Stress Disorder. This was done using a pilot population similar in age and physical status as active duty military, but within a college setting. The pre-established protocols were pilot tested on two male and two female participants. The data collected was from the IM Long Form, Nine Hole Peg Test, Canadian Occupational Performance Model and the Test of Everyday Attention. In a comparative analysis of pre- and post- status it was determined that the IM along with TRX® system would potentially benefit returning post deployment service members using this series of treatment interventions.
Microfiber and Macrofiber Hybrid Fiber-Reinforced Concrete

To realize the potential of micro- and macrofiber blends in a concrete matrix, a concrete containing polyvinyl alcohol (PVA) or steel microfibers and steel macrofibers was designed using a mixture proportioning method that provides good workability in concretes containing microfibers. This procedure is based on an optimum paste volume fraction determined from the relationship between flow and paste content. The mechanical performance, water permeability of the cracked material, and shrinkage crack resistance of cast concrete were evaluated. In the hybrid concrete, the microfibers delayed the development of macrocracks and so the composite demonstrated greater strength and crack resistance than a similar matrix reinforced with macrofibers only. This influence was less pronounced than was observed with a mortar matrix in a previous study. This is explained by differences in the failure mechanism of the fibers: A stronger fiber-matrix bond resulting from a lower water-to-binder ratio caused the microfibers t.