Miracle-dz/UniFAR-FTUs huggingface dataset:
Protein product of CLN6 gene responsible for variant late‐onset infantile neuronal ceroid lipofuscinosis interacts with CRMP‐2

Mutations in CLN6 cause variant late‐onset neuronal ceroid lipofuscinosis (vLINCL), a childhood neurodegenerative disorder resulting from aberrant neuronal cell loss and pathological accumulation of lysosomal autofluorescent storage material in the central nervous system. The direct function of the endoplasmic reticulum–resident protein CLN6 and how dysfunction of this protein results in vLINCL are unknown. We report that CLN6 interacts with collapsin response mediator protein‐2 (CRMP‐2). To further understand the significance and possible contribution to vLINCL of the CLN6–CRMP‐2 interaction, we utilized the nclf mouse, which harbors mutations in CLN6. Significantly, CRMP‐2 protein level was found to be reduced in the nclf mouse brain, particularly in the thalamus. Because CRMP‐2 functions in growth cone collapse and is an effector protein downstream of Sema3A signaling, this pathway was examined via a dorsal root ganglion (DRG) repulsion assay. However, there were no defects in the repulsion of DRGs derived from nclf mice, indicating that the loss of CLN6 does not affect Sema3A signaling. CRMP‐2 has also been implicated in controlling axon number and outgrowth, as observed in cultured hippocampal neurons. Therefore, we explored the formation and maturation of hippocampal neurons derived from nclf mice in a glial coculture system. The maturation of these neurons was reduced; by day in vitro (DIV) 8, more than 50% of nclf‐derived hippocampal neurons had died. Additionally, beginning around DIV4, nclf neurons were less mature than their WT counterparts, presumably because of an inability to form mature synaptic connections. We concluded that alterations in neurite maturation resulting from a loss of CLN6–CRMP‐2 interaction may contribute to neuronal dysfunction and pathology in vLINCL. © 2009 Wiley‐Liss, Inc.
Effect of particle deposition parameters on silica coating of zirconia using a chairside air-abrasion device.

PURPOSE To evaluate the effect of nozzle distance, nozzle angle, and deposition duration on the silica content attained on zirconia by air abrasion. MATERIALS AND METHODS Disk-shaped zirconia (LAVA, 3M ESPE) (diameter: 10 mm, thickness: 2 mm) specimens (N = 54) were obtained. They were wet-ground finished using 600-, 800-, and 1200-grit silicone carbide abrasive papers in sequence and ultrasonically cleaned. The specimens were mounted in a specially designed apparatus that allowed the chairside air-abrasion device to be operated under standard conditions. Alumina-coated silica particles (CoJet Sand, 3M ESPE) were deposited on the zirconia disk surfaces varying the following parameters: a) nozzle distance (2, 5, 10 mm), and b) deposition duration (5, 13, 20 s) at two nozzle angles (45 and 90 degrees) under 2.5 bar pressure at three locations on each ceramic disk. The specimen surfaces were then gently air dried for 20 s. Silica content in weight percentage (wt%) was measured from 3 surfaces on each disk using Energy Dispersive X-ray Spectroscopy (EDS) (150X) in an area of 0.8 mm x 0.6 mm (n = 3 per group). Surface topographies were evaluated using SEM. Data were analyzed using ANOVA and Tukey's tests (α = 0.05). RESULTS Nozzle angle (p = 0.003) and deposition duration (p = 0.03) significantly affected the results, but nozzle distance (p = 0.569) did not. A significantly higher amount of silica (wt%) was achieved when the nozzle angle was 45 degrees to the surface in all distance-duration combinations (16.7 to 28.2 wt%) compared to the 90-degree nozzle angle (10.7 to 18.6 wt%) (p < 0.001). The silica amount was significantly higher after 20-s deposition duration than after 13 s (p < 0.05). EDS analysis demonstrated not only Si but also Al, Zr, and O traces on the substrate. SEM images indicated that deposition at a nozzle distance of 2 mm often created cavitations in zirconia. CONCLUSION Effective silica deposition using a charside air-abrasion device can be achieved when the nozzle is held at 45 degrees to the surface with more than 2-mm nozzle distance for 20 s.
Phase 2 randomized trial of primary endocrine therapy versus chemotherapy in postmenopausal patients with estrogen receptor‐positive breast cancer

Few studies have compared primary neoadjuvant endocrine therapy with neoadjuvant chemotherapy in breast cancer patients. The need for preoperative chemotherapy with doxorubicin or taxanes may be reduced in postmenopausal patients with estrogen receptor (ER)‐positive and/or progesterone receptor (PgR)‐positive tumors. This randomized, controlled, phase 2 study evaluated the efficacy of neoadjuvant chemotherapy compared with endocrine treatment with aromatase inhibitors in postmenopausal women with ER‐positive and/or PgR‐positive breast cancer.
Investigating Relationship between Corporate Social Responsibility and Financial Performance using Structural Equation Modelling

The objective of present study is to investigate the relationship between corporate social responsibility (CSR) and financial performance (FP) with the help of a stakeholder approach in the context of small and medium enterprises (SME) industry in Delhi NCR. A sample of 382 SMEs was analysed through a structured questionnaire having 22 statements out of which two statements were removed due to low factor loading. The goodness of fit was measured by AMOS–SEM and relationship between variables was examined by path analysis. The overall model was fit and finding indicated a weak positive relationship between CSR and FP. The results were in conformance with previous research works by Jain, Vyas and Durga (2016) and Weber (2008). It was also observed that SMEs are socially responsible towards their stakeholders, but it is more in informal nature rather than adopting it as a strategy. Further, the results revealed that CSR of SMEs is associated with religious spirit.
The association of self-reported sleep, weight status, and academic performance in fifth-grade students.

BACKGROUND To improve support and justification for health promotion efforts in schools, it is helpful to understand how students' health behaviors affect academic performance. METHODS Fifth-grade students completed an online school-administered health survey with questions regarding their eating behavior, physical activity, academic performance, and sleep patterns. Differences in health behaviors were examined by sex, self-reported weight status, and sufficient (≥9 hours) versus insufficient sleep. Logistic regression was used to determine the relationship between academic performance and the health behaviors. RESULTS One third of the sample did not get the recommended amount of physical activity and more than half of the students watched television ≥ 2 hours/day. Self-reported overweight status was related to lower self-reported academic performance, fewer lunch and breakfast occasions, less physical activity, not meeting the recommendations for vegetable and soda consumption as well as hours of television watching. Sufficient sleep (≥9 hours/night) was associated with better grades, meeting the recommended hours of daily television watching and video game playing, being more physically active and increased breakfast and lunch frequency. Percentage of serving free/reduced lunch, soda consumption, breakfast frequency, amount of physical activity, and television watching were associated with academic performance. CONCLUSION More positive health behaviors generally were associated with better academic performance. Promoting healthy behaviors in schools might improve not only students' health academic performance as well.
The Volume of Experimental Design Cerebellum: Stereological Microanatomic Study

Abstract Cerebellum is responsible to higher cognitive functions and delicate motor activity. Because of its significance, cerebellum may play a major role in cognitive, behavioral, and psychiatric diseases. Therefore, the investigations on cerebellum may be contributed to the future researches so as to solve reasons of the diseases. The rabbit brain relatively larger than the rat and mouse brain and the individual evaluation of its subdivisions are rather easy. There are no data on cerebellar volume of rabbits in literature. Thus, the study presents microscopic volume calculation of rabbits’ cerebellum using the stereological method. The mean (SD) calculated volume of the cerebellum in the rabbit brain was found to be 0.69 (0.03) cm3. The individual microscopic volume estimation of an animal subject can be achieved by Cavalier method. Researchers believed that the findings and the applied method in this study may be useful for the scientists.
Pengaruh Rasio Keuangan Dan Ukuran Perusahaan Terhadap Kondisi Kesulitan Keuangan (Financial Distress) Pada Perusahaan Sub-Sektor Transportasi Yang Terdaftar Di Bursa Efek Indonesia (Bei) Periode 2013-2016

ABSTRAK Tujuan dari penelitian ini adalah untuk menganalisis pengaruh rasio keuangan (rasio likuiditas, rasio leverage, rasio aktivitas, rasio profitabilitas) dan ukuran perusahaan terhadap kondisi kesulitan keuangan (financial distress). Populasi dalam penelitian ini adalah perusahaan sub-sektor transportasi yang terdaftar di Bursa Efek Indonesia periode 2013-2016 yang terdiri dari 35 perusahaan. Teknik pengambilan sampel adalah metode purposive sampling yang menghasilkan sampel sebanyak 20 perusahaan dengan 80 data. Metode analisis data menggunakan analisis statistik deskriptif dan analisis regresi logistik. Pada penelitian ini kombinasi antara rasio likuiditas, rasio leverage, rasio aktivitas, rasio profitabilitas, ) dan ukuran perusahaan berpengaruh secara simultan dan mampu menjelaskan variasi dari variabel dependen yaitu financial distress sebesar 71,6% dan sisanya 28,4% dijelaskan oleh variabel-variabel lain yang tidak dilibatkan dalam penelitian ini. Hasil penelitian ini menunjukkan variabel rasio leverage, rasio profitabilias dan ukuran perusahaan secara simultan berpengaruh negatif terhadap financial distress. Dan variabel rasio aktivitas secara parsial berpengaruh positif terhadap financial distress, sedangkan variabel rasio likuiditas secara parsial tidak berpengaruh terhadap financial distress. Kata Kunci : Financial Distress, Rasio Likuiditas, Rasio Leverage, Rasio Aktivitas, Rasio Profitabilitas, Dan Ukuran Perusahaan. ABSTRACT The purpose of this study is to analyze the influence of financial ratios (liquidity ratio, leverage ratio, activity ratio, profitability ratio) and firm size toward financial distress conditions. The population in this study is a subsector transportation companies listed on the Indonesia Stock Exchange period 2013-2016 whch consist of 35 companies. The sampling technique is a purposive sampling method that results for 20 companies with 80 data. The data analysis method uses descriptive statistical analysis and logistic regression analysis. In this study, the combination of liquidity ratio, leverage ratio, activity ratio, profitability ratio, and firm size can explain and have an influence to the dependent variable financial distress equal to 71,6% and while the remaining 28,4% explained by other variables not included in the study. The result of this study shows that leverage ratio, profitabilias ratio and company size simultaneously have negative effect on the financial distress. And variable of activity ratio partially has positive effect to the financial distress, However variable of liquidity ratio partially has no effect to financial distress. Keywords: Financial Distress, Liquidity Ratio, Leverage Ratio, Activity Ratio, Profitability Ratio, Dan Firm Size
Moderating Effect of Environmental Dynamism on the Relationship between a Firm’s Entrepreneurial Orientation and Financial Performance

Abstract The relationship between entrepreneurial orientation and firm performance has been the focus of numerous empirical studies over the past decade. The conclusions and findings reported are diverse and often conflicting. One possible explanation for mixed findings is that past studies do not take into account the dynamic nature of the industry environment. Using a sample of 192 Swiss firms from several different industries, this study examines the direct effect of entrepreneurial orientation on financial firm performance along with the moderating effect of a dynamic environment on the relationship between entrepreneurial orientation and performance. Results of this study suggest that for firms competing in a highly dynamic environment, the positive effect of an entrepreneurial orientation on financial performance is enhanced.
Distinctive features of tumor-infiltrating γδ T lymphocytes in human colorectal cancer

ABSTRACT γδ T cells usually infiltrate many different types of cancer, but it is unclear whether they inhibit or promote tumor progression. Moreover, properties of tumor-infiltrating γδ T cells and those in the corresponding normal tissue remain largely unknown. Here we have studied features of γδ T cells in colorectal cancer, normal colon tissue and peripheral blood, and correlated their levels with clinicopathologic hallmarks. Flow cytometry and transcriptome analyses showed that the tumor comprised a highly variable rate of TILs (5–90%) and 4% γδ T cells on average, with the majority expressing Vδ1. Most Vδ1 and Vδ2 T cells showed a predominant effector memory phenotype and had reduced production of IFN- γ which was likely due to yet unidentified inhibitory molecules present in cancer stem cell secretome. Transcriptome analyses revealed that patients containing abundant γδ T cells had significantly longer 5-year disease free survival rate, suggesting their efficacy in controlling tumor at very early stage.
Serum cholesterol levels do not influence outcome or recovery in acute ischemic stroke

Abstract Objective: To examine the influence of admission serum cholesterol levels (SCL) on severity of initial neurological deficit, neurological outcome at month 3 and neurological recovery in patients with acute first-ever ischemic stroke. Methods: Prospectively collected data from 889 consecutive patients with first-ever acute ischemic stroke were retrospectively analysed. Patients who suffered a recurrent ischemic stroke (n=22) or died (n=30) during the follow-up period were excluded from this study. Age, gender, arterial hypertension, diabetes mellitus, smoking, stroke etiology, SCL and severity of neurological deficit, using the National Institute of Health Stroke Scale (NIHSS), at presentation (NIHSS0) and after 3 months (NIHSS1), were assessed. Neurological recovery was defined as difference in NIHSS score (Δ NIHSS), according to Δ NIHSS=NIHSS0 − NIHSS1. Results: Data from 837 patients (66% men, age: 62 ± 14 years) were analysed. NIHSS1 was 2.3 ± 1.8 and Δ NIHSS was 3.4 ± 3. Clinically insignificant correlations between SCL and NIHSS0 (r=−0.13, p=0.0002), NIHSS1 (r=−0.09, p=0.001) and Δ NIHSS (r=−0.1, p=0.03) were evident. Multivariate binary logistic regression analysis revealed smoking (p=0.008), stroke etiology (p=0.023) and NIHSS0 (p<0.001) but not age, gender, arterial hypertension, diabetes mellitus or SCL as predictors for Δ NIHSS. Conclusion: Our data suggest that SCL in patients with acute ischemic stroke are not associated with neurological deficit on admission, outcome or neurological recovery.
Pregnancy and fetal outcomes after interferon-β exposure in multiple sclerosis

Objective: To assess pregnancy and fetal outcomes after in utero exposure to interferon-β (IFNβ) in all pregnancies occurring in women with multiple sclerosis (MS) during the study period, with a specific focus on the risk of spontaneous abortion. Methods: In this cohort study, data were gathered through a standardized, semi-structured interview. Patients who discontinued IFNβ less than 4 weeks from conception (exposed) were compared with those who had discontinued the drug at least 4 weeks from conception or who were never treated (not exposed). Possible confounders were handled through multivariate analyses adjusted for propensity score (PS). Results: We collected data on 396 pregnancies in 388 women, 88 classified as exposed (mean exposure 4.6 ± 5.8 weeks). IFNβ exposure was not associated with an increased risk of spontaneous abortion (PS-adjusted odds ratio [OR] 1.08, 95% confidence interval [CI] 0.4 to 2.9, p = 0.88), although it was associated with both lower baby weight (PS-adjusted β −113.8, p < 0.0001) and length (PS-adjusted β −1.102, p < 0.0001). Proportion of spontaneous abortion in exposed patients fell within the range expected for the Italian population in the same period. IFNβ exposure (PS-adjusted OR 2.11, 95% CI 1.18 to 3.78, p = 0.012) and cesarean delivery were the only predictors of preterm delivery. In the exposed group, we did not observe any significant fetal complications, malformations, or developmental abnormalities over a median follow-up of 2.1 years. Conclusions: Our findings point to the relative safety of IFNβ exposure times of up to 4 weeks and can assist neurologists facing therapeutic decisions in women with MS with a pregnancy plan.
The Impact of Quality of Institutions on Sectoral FDI

This study addresses the issue whether institutional quality affects the sectoral FDI both in short run and long run in Pakistan. By employing ARDL co-integration technique, we analyse the impact of institutional quality on primary, manufacturing and services sectors FDI in Pakistan. The findings suggest that institutional quality matters in attracting FDI in manufacturing and services sectors in the long run while institutional quality does not have a significant impact on FDI in the primary sector. Moreover, results show that the impact of institutional quality on these sectors is not apparent in short run. The main findings from this research are that in long run institutional quality matters to attract substantial FDI in manufacturing and services sector of Pakistan. Hence, policies aimed at strengthening the institutional quality should be the priority for government. JEL: F21, O43, C22
Super-critical withdrawal from a two-layer fluid through a line sink if the lower layer is of finite depth

The steady response of a fluid consisting of two regions of different density, the lower of which is of finite depth, is considered during withdrawal. Super-critical flows are considered in which water from both layers is being withdrawn, meaning that the interface is drawn down directly into the sink. The results indicate that if the flow rate is above some minimum, the angle of entry of the interface depends more strongly on the relative depth of the sink than on the flow rate. This has quite dramatic consequences for the understanding of selective withdrawal from layered fluids.
Agency, Aspirations and Decision-Making of Marginalised Young People in Social Enterprises

This thesis examines the contribution that changes in young people’s agency, aspirations and decision-making during participation in social enterprises make in improving engagement and outcomes in employment, education and training. The focus is contextualised within the growing number of young people who are marginalised by not engaging in employment, education and training in Australia and the majority of countries in the Organisation for Economic Co-operation and Development (2012). Consequences of young people’s marginalisation are experienced at the personal, community and Government levels and include long-term marginalisation and heavy social and financial costs. The focus of this study is social enterprises that operate as intermediate labour market interventions with a social purpose. These enterprises address the vocational and non-vocational barriers of marginalised groups in an effort to assist them to obtain employment, education and training outcomes. While the literature on social enterprises is increasing, a gap persists regarding the possible changes in cognitive-motivational variables such as agency, aspirations and decision-making processes and their roles in promoting positive outcomes. In particular, the lack of research and theorisation addressing the predictive potential of change in such cognitive-motivational variables as young people participate in social enterprises has restricted evidence-based service delivery to capitalise on any such relation.
Limiter-Free Third Order Logarithmic Reconstruction

A third order conservative reconstruction, in the context of finite volume schemes for hyperbolic conservation laws, is constructed based on logarithmic functions. This logarithmic method reconstructs without the use of a limiter, any preprocessing of input data, special treatments for local extrema, or shock solutions. Also the method is local in the sense that data from only the nearest neighbors are required. We test the new reconstruction method in several numerical experiments, including nonlinear systems in one and two space dimensions.
Enhancing Tourism and Cultural Experience Through Gamification

We all travel to different historical places but rarely notice the cultural significance of the place: the history behind the various things and locations. The primary aim of this chapter is to devise and develop means to enhance tourism and provide culturally important knowledge to people. Moreover, internet is booming, and the number of smartphones is increasing day by day. The same is happening in many developing countries. Therefore, in this chapter, the authors propose a criterion that uses technology to promote historical significance and enhances tourism industry as well. The proposed app uses the concept of gamification that acts as a platform to fuel the process of branding in a manner that engages the tourists and provide them with an experience that can make them feel more connected to the tourism destinations.
[Otoacoustic emissions (TEOAE and DPOAE) after middle ear operation].

BACKGROUND The behavior of transitory evoked otoacoustic emissions (TEOAE) and distortion products (DPOAE) in patients after middle ear surgery is unknown. METHODS We examined TEOAEs and DPOAEs in 37 ears following stapes surgery, 16 ears following tympanoplasty type I, and nine ears after reconstruction of the ossicular chain. In all patients, the postoperative conductive hearing loss was less than 15 dB HL. In some cases, there was additional sensorineural hearing loss in the high frequencies. RESULTS Surprisingly, TEOAEs were present only in 10 of 62 ears postoperatively compared to all 22 control subjects. TEOAEs were detected in four of 37 ears after stapes surgery, in six of 16 ears after tympanoplasty type I, and in none of the nine ears who had undergone reconstruction of the ossicular chain. DPOAEs were detected in only four of 31 ears postoperatively compared to all 22 control ears. CONCLUSIONS These results and their relation to the retrograde OAE transmission in the middle ear are discussed. Changes enhancing the stiffness of the middle ear seem to have a greater effect than those enhancing the mass.