Miracle-dz/UniFAR-FTUs huggingface dataset:
Defective iron homeostasis and hematological abnormalities in Niemann-Pick disease type C1

Background: Niemann-Pick disease type C1 (NPC1) is a neurodegenerative lysosomal storage disorder characterized by the accumulation of multiple lipids in the late endosome/lysosomal system and reduced acidic store calcium. The lysosomal system regulates key aspects of iron homeostasis, which prompted us to investigate whether there are hematological abnormalities and iron metabolism defects in NPC1. Methods: Iron-related hematological parameters, systemic and tissue metal ion and relevant hormonal and proteins levels, expression of specific pro-inflammatory mediators and erythrophagocytosis were evaluated in an authentic mouse model and in a large cohort of NPC patients. Results: Significant changes in mean corpuscular volume and corpuscular hemoglobin were detected in Npc1 -/- mice from an early age. Hematocrit, red cell distribution width and hemoglobin changes were observed in late-stage disease animals. Systemic iron deficiency, increased circulating hepcidin, decreased ferritin and abnormal pro-inflammatory cytokine levels were also found. Furthermore, there is evidence of defective erythrophagocytosis in Npc1 -/- mice and in an in vitro NPC1 cellular model. Comparable hematological changes, including low normal serum iron and transferrin saturation and low cerebrospinal fluid ferritin were confirmed in NPC1 patients. Conclusions: These data suggest loss of iron homeostasis and hematological abnormalities in NPC1 may contribute to the pathophysiology of this disease.
Predicting attitudes towards screening for neurodegenerative diseases using OCT and artificial intelligence: Findings from a literature review

Recent developments in artificial intelligence (AI) and machine learning raise the possibility of screening and early diagnosis for neurodegenerative diseases, using 3D scans of the retina. The eventual value of such screening will depend not only on scientific metrics such as specificity and sensitivity but, critically, also on public attitudes and uptake. Differential screening rates for various screening programmes in England indicate that multiple factors influence uptake. In this narrative literature review, some of these potential factors are explored in relation to predicting uptake of an early screening tool for neurodegenerative diseases using AI. These include: awareness of the disease, perceived risk, social influence, the use of AI, previous screening experience, socioeconomic status, health literacy, uncontrollable mortality risk, and demographic factors. The review finds the strongest and most consistent predictors to be ethnicity, social influence, the use of AI, and previous screening experience. Furthermore, it is likely that factors also interact to predict the uptake of such a tool. However, further experimental work is needed both to validate these predictions and explore interactions between the significant predictors.
Gauge fixed gluonic observables and neutral kaon mixing on the lattice

This thesis presents gauge fixed gluonic observable and neutral Kaon mixing matrix element measurements using nf=2+1 Domain Wall Fermion (DWF) configurations. These were generated with the Iwasaki gauge action by the RBC and UKQCD collaborations. Results from the first measurement of the QCD strong coupling with these ensembles using the triple gluon vertex are shown. We find that while a very accurate measurement of the coupling is possible using this technique, the systematic error from the perturbative matching at current lattice scales is large. We also discuss the utilisation of this method as a probe for possible Technicolor theories. The calculation of the QCD strong coupling constant from the triple gluon vertex required an implementation of a fast code to fix lattice gauge configurations. I provide details on my implementation of a parallel and optimised Fourieraccelerated algorithm for both Landau and Coulomb gauge fixing. I include the first calculation of the highly accurate W0-scale using these ensembles, allowing for percent-level scale setting. I show results from a wide variety of smearing methods and present the first gluonic measurement of different smearing radii. This thesis also details the first nf=2+1 measurement of the BSM neutral Kaon mixing renormalised matrix elements from lattice simulations with almost exact chiral symmetry in the valence sector and the sea.
Mass distribution in nearby Abell clusters

We study the mass distribution in six nearby (z < 0.06) relaxed Abell clusters of galaxies A0262, A0496, A1060, A2199, A3158 and A3558. Given the dominance of dark matter in galaxy clusters, we approximate their total density distribution by the Navarro, Frenk & White (NFW) formula characterized by virial mass and concentration. We also assume that the anisotropy of galactic orbits is reasonably well described by a constant and that galaxy distribution traces that of the total density. Using the velocity and position data for 120‐420 galaxies per cluster we calculate, after removal of interlopers, the profiles of the lowest order even velocity moments, dispersion and kurtosis. We then reproduce the velocity moments by jointly fitting the moments to the solutions of the Jeans equations. Including the kurtosis in the analysis allows us to break the degeneracy between the mass distribution and anisotropy and constrain the anisotropy as well as the virial mass and concentration. The method is tested in detail on mock data extracted from the N-body simulations of dark matter haloes. We find that the best-fitting Galactic orbits are remarkably close to isotropic in most clusters. Using the fitted pairs of mass and concentration parameters for the six clusters, we conclude that the trend of decreasing concentration for higher masses found in the cosmological N-body simulations is consistent with the data. By scaling the individual cluster data by mass, we combine them to create a composite cluster with 1465 galaxies and perform a similar analysis on such sample. The estimated concentration parameter then lies in the range 1.5 < c < 14 and the anisotropy parameter in the range −1.1
Adjuvant Systemic Treatment for Renal Cancer After Surgery: A Network Meta-Analysis

Background Approximately 15% to 20% of patients will experience disease recurrence following surgical removal of renal cell carcinoma. A range of pharmacological agents is prescribed for metastatic renal cell carcinoma, but there are trials testing whether these have an earlier role in the adjuvant setting. We aim to assess the efficacy of adjuvant systemic treatment following surgery in patients with renal cell carcinoma and to determine the most effective treatment. Methods The protocol for this review was published in PROSPERO (CRD42021281588). We searched multiple databases up to August 2021. We included only randomized trials of patients with renal cell carcinoma that had been completely resected. We included patients with locoregional nodal disease if it was surgically removed, and excluded all cases of metastatic disease. We included all adjuvant systemic therapies that were commenced within 90 days of renal surgery. A network meta-analysis was performed using a frequentist approach. Results A total of 13 studies with 8103 patients were included for analysis. Only pembrolizumab (HR 0.74; 95%CI 0.57 to 0.96) and pazopanib (HR 0.80; 95%CI 0.68 to 0.95) improved disease-free survival compared with observation. These 2 treatments were the 2 highest ranked comparisons with a P-score of 0.87 and 0.80. No agent improved overall survival. All agents increased the risk of severe adverse events compared with observation. Conclusions Pembrolizumab and pazopanib were the only 2 adjuvant agents that improved time to disease recurrence compared with observation, with the former likely being the more efficacious. None of the treatments improved overall survival and almost all increased severe adverse events. Introduction
Applying Novel Visitation Models using Diverse Social Media to Understand Recreation Change after Wildfire and Site Closure

Abstract Natural disturbances such as wildfires are increasing in severity and frequency. Although the ecological impacts of disturbance are well documented, we have limited understanding of how disturbances and associated management responses influence recreation use patterns. This reflects, in part, difficulty in quantifying recreation use across different land ownerships with inconsistent, or non-existent, recreation monitoring practices. In this study, we use visitation models based on social media to examine how recreation use changed after a wildfire and site closures in a large, mixed-ownership landscape. We find that wildfire and associated closures resulted in visitation loss to the recreation system as a whole and little site-to-site displacement within the system in the two years following the wildfire. Our study highlights the importance, when considering how wildfire and management may alter recreation use patterns, of considering the many factors that influence substitution behavior, including the relative locations of visitor origins, disturbances, and substitute sites.
Efficacy of agomelatine with cognitive behavioral therapy for delayed sleep-wake phase disorder in young adults: A randomized controlled study

ABSTRACT Background Delayed sleep-wake phase disorder (DSWPD) is common and easily misdiagnosed in young people, and to date, there is no evidence-based treatment. Purpose A nonblinded randomized controlled study evaluated the effect of agomelatine therapy (AT) and cognitive behavior therapy (CBT) on DSWPD in young adults. Methods Sixty adolescents and young adults (range = 19–24 years, mean = 22 years, 52% female) diagnosed with DSWPD were randomized to receive 4 weeks of agomelatine therapy with or without cognitive behavior therapy. Sleep diaries, Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS), Insomnia Severity Index (ISI), and World Health Organization wellbeing questionnaire (WHO-5) were measured pre-treatment and post-treatment. Results Agomelatine therapy for 4 weeks shifted the sleep-wake rhythm (p < .001) forward in both groups at the week 4 assessment. There were no significant differences in sleep onset (p = .099) and sleep offset (p = .959) between the CBT group and the no treatment (NT) group at the follow-up visits. However, significant differences were found in sleep duration (p = .002), sleep quality (p=0.005), sleep difficulties (p < .001), daytime sleepiness (p = .001), and wellbeing (p = .007) between groups. Conclusions The improvements were received largely through the sleep-promoting effects of agomelatine therapy, and combining with cognitive behavior therapy on maintenance of altered sleep rhythms might be feasible.
A SURVEY FOR NEW MEMBERS OF THE TAURUS STAR-FORMING REGION WITH THE SLOAN DIGITAL SKY SURVEY

Previous studies have found that ∼1 deg2 fields surrounding the stellar aggregates in the Taurus star-forming region exhibit a surplus of solar-mass stars relative to denser clusters like IC 348 and the Orion Nebula Cluster. To test whether this difference reflects mass segregation in Taurus or a variation in the initial mass function, we have performed a survey for members of Taurus across a large field (∼40 deg2) that was imaged by the Sloan Digital Sky Survey (SDSS). We obtained optical and near-infrared spectra of candidate members identified with those images and the Two Micron All Sky Survey, as well as miscellaneous candidates that were selected with several other diagnostics of membership. We have classified 22 of the candidates as new members of Taurus, which includes one of the coolest known members (M9.75). Our updated census of members within the SDSS field shows a surplus of solar-mass stars relative to clusters, although it is less pronounced than in the smaller fields toward the stellar aggregates that were surveyed for previously measured mass functions in Taurus. In addition to spectra of our new members, we include in our study near-IR spectra of roughly half of the known members of Taurus, which are used to refine their spectral types and extinctions. We also present an updated set of near-IR standard spectra for classifying young stars and brown dwarfs at M and L types.
Finite-mass correction to 2D Black-hole evaporation rate

We numerically analyze the evolution of a two-dimensional dilatonic black hole, within the CGHS model. We focus our attention on the finite-mass corrections to the universal evaporation rate which applies at the large-mass limit. Our numerical results confirm a previous theoretical prediction for the first-order ($\propto 1/M$) correction. In addition, our results strongly suggest that the next-order ($\propto 1/M^{2}$) term vanishes, and provide a rough estimate for the third-order term.
Flight Testing GBAS for UAV Operations

In this study the concept of using GBAS corrections and integrity parameters for UAV operations is explored. The GBAS messages are received and decoded at the airport to overcome the issue of VDB coverage for alternative applications that do not have line-of-sight to the GBAS. A flight test at a distance of about 15 km from an operational GBAS ground station was conducted using a quad-rotor UAV as test vehicle. The recorded GBAS messages and recorded pseudoranges and carrier phases were subsequently used to evaluate the performance that can be obtained. One main issue that was identified is the number of satellites lost when maneuvering the drone. Loss of tracking requires a re-initialization of the smoothing filter and thus entails a wait time before a satellite can be used for positioning again. The GBAS corrections are generated based on 100 s smoothing, requiring a user to use the same smoothing time constant do avoid potential differential error buildups due to different filters. Hence, the time until a satellite can be used again after reacquisition may be substantial. When losing several satellites simultaneously or shortly after another this decreases the number of available satellites significantly and negatively impacts accuracy and protection level performance of this proposed mode of operation. The paper also studies the multipath effect at different altitudes and for different smoothing time constants compared to the standardized error models that were defined for large transport aircraft. When using 100 s smoothing the results indicate that the models are suitable also for the use case of UAV operations.
Teaching Team Science: The Key to Addressing 21st Century Global Challenges

To solve complex 21st-century global challenges, universities must prepare students to be competent team members. This article presents results from analysis of data collected at a university in four types of undergraduate sociology classrooms using mixed-methods, including social network analysis, student reflections, and an alumni survey. Results showed that learning is a social process. Compared with traditional lecture, fixed teams, and interacting teams, opportunistic collaboration is the most effective structure in teaching team learning through fostering communication, support, and learning networks. Post-secondary education should endorse opportunistic collaboration learning practices to prepare students for workplace success in a global economy.
BULK FLOWS FROM GALAXY LUMINOSITIES: APPLICATION TO 2MASS REDSHIFT SURVEY AND FORECAST FOR NEXT-GENERATION DATA SETS

We present a simple method for measuring cosmological bulk flows from large redshift surveys, based on the apparent dimming or brightening of galaxies due to their peculiar motion. It is aimed at estimating bulk flows of cosmological volumes containing large numbers of galaxies. Constraints on the bulk flow are obtained by minimizing systematic variations in galaxy luminosities with respect to a reference luminosity function measured from the whole survey. This method offers two advantages over more popular bulk flow estimators: it is independent of error-prone distance indicators and of the poorly known galaxy bias. We apply the method to the Two Micron All Sky Survey redshift survey to measure the local bulk flows of spherical shells centered on the Milky Way (MW). The result is consistent with that obtained by Nusser & Davis using the SFI++ catalogue of Tully–Fisher distance indicators. We also make an assessment of the ability of the method to constrain bulk flows at larger redshifts (z = 0.1–0.5) from next-generation data sets. As a case study we consider the planned EUCLID survey. Using this method we will be able to measure a bulk motion of ∼200 km s−1 of 106 galaxies with photometric redshifts, at the 3σ level for both z ∼ 0.15 and z ∼ 0.5. Thus, the method will allow us to put strong constraints on dark energy models as well as alternative theories for structure formation.
Horizon thermodynamics and gravitational field equations in Horava-Lifshitz gravity

We explore the relationship between the first law of thermodynamics and gravitational field equation at a static, spherically symmetric black hole horizon in Horava-Lifshitz theory with/without detailed balance. It turns out that as in the cases of Einstein gravity and Lovelock gravity, the gravitational field equation can be cast to a form of the first law of thermodynamics at the black hole horizon. This way we obtain the expressions for entropy and mass in terms of black hole horizon, consistent with those from other approaches. We also define a generalized Misner-Sharp energy for static, spherically symmetric spacetimes in Horava-Lifshitz theory. The generalized Misner-Sharp energy is conserved in the case without matter field, and its variation gives the first law of black hole thermodynamics at the black hole horizon.
The effects of baryons on the halo mass function

We present an analysis of the effects of baryon physics on the halo mass function. The analysis is based on simulations of a cosmological volume having a comoving size of 410 h−1 Mpc, which have been carried out with the tree-pm/smoothed particle hydrodynamics code gadget-3, for a Wilkinson Microwave Anisotropy Probe-7 Λ cold dark matter cosmological model. Besides a dark matter (DM)-only simulation, we also carry out two hydrodynamical simulations: the first one includes non-radiative physics, with gas heated only by gravitational processes; the second one includes radiative cooling, star formation and kinetic feedback in the form of galactic ejecta triggered by supernova explosions. All simulations follow the evolution of two populations of 10243 particles each, with mass ratio such that to reproduce the assumed baryon density parameter, with the population of lighter particles assumed to be collisional in the hydrodynamical runs. We identified haloes using a spherical overdensity algorithm and their masses are computed at three different overdensities (with respect to the critical one), Δc= 200, 500 and 1500. We find the fractional difference between halo masses in the hydrodynamical and in the DM simulations to be almost constant, at least for haloes more massive than . In this range, mass increase in the hydrodynamical simulations is of about 4–5 per cent at Δc= 500 and ∼1–2 per cent at Δc= 200. Quite interestingly, these differences are nearly the same for both radiative and non-radiative simulations. Mass variations depend on halo mass and physics included for higher overdensity, Δc= 1500, and smaller masses. Such variations of halo masses induce corresponding variations of the halo mass function (HMF). At z= 0, the HMFs for gravitational heating and cooling and star formation simulations are close to the DM one, with differences of ≲3 per cent at Δc= 200, and ≃7 per cent at Δc= 500, with ∼10–20 per cent differences reached at Δc= 1500. At this higher overdensity, the increase of the HMF for the radiative case is larger by about a factor of 2 with respect to the non-radiative case. Assuming a constant mass shift to rescale the HMF from the hydrodynamic to the DM simulations, brings the HMF difference with respect to the DM case to be consistent with zero, with a scatter of ≲3 per cent at Δc= 500 and ≲2 per cent at Δc= 200. Our results have interesting implications for assessing uncertainties in the mass function calibration associated with the uncertain baryon physics, in view of cosmological applications of future large surveys of galaxy clusters.
Staggered-Level Designs for Response Surface Modeling

In industrial experiments, there are often restrictions in randomization caused by equipment and resource constraints, as well as budget and time constraints. Next to the split-plot and the split-split-plot design, the staggered-level design is an interesting design option for experiments involving two hard-to-change factors. The staggered-level design allows both hard-to-change factors to be reset at different points in time, resulting in a typical staggering pattern of factor-level resettings. It has been shown that, for two-level designs, this staggering pattern leads to statistical benefits in comparison to the split-plot and the split-split-plot design. In this paper, we investigate whether the benefits of the staggered-level design carry over to situations where the objective is to optimize a response and where a second-order response surface model is in place. To this end, we study several examples of D- and I-optimal staggered-level response surface designs.
THE PROPAGATION OF RELATIVISTIC JETS IN EXTERNAL MEDIA

Relativistic jets are ubiquitous in astrophysical systems that contain compact objects. They transport large amounts of energy to large distances from the source and their interaction with the ambient medium has a crucial effect on the evolution of the system. The propagation of the jet is characterized by the formation of a shocked “head” at the front of the jet which dissipates the jet's energy and a cocoon that surrounds the jet and potentially collimates it. We present here a self-consistent, analytic model that follows the evolution of the jet and its cocoon, and describes their interaction. We show that the critical parameter that determines the properties of the jet–cocoon system is the dimensionless ratio between the jet's energy density and the rest-mass energy density of the ambient medium. This parameter, together with the jet's injection angle, also determines whether the jet is collimated by the cocoon or not. The model is applicable to relativistic, unmagnetized jets on all scales and may be used to determine the conditions in active galactic nucleus (AGN) jets as well as in gamma-ray bursts (GRBs) or microquasars. It shows that AGN and microquasar jets are hydrodynamically collimated due to the interaction with the ambient medium, while GRB jets can be collimated only inside a star and become uncollimated once they break out.
Spectral energy distributions of 6.7 GHz methanol masers

Context. The 6.7 GHz maser transition of methanol has been found exclusively towards massive star forming regions. A majority of the masers have been found to lack the presence of any associated radio continuum. This could be due to the maser emission originating prior to the formation of an H ii region around the central star, or from the central object being too cool to produce a H ii region. Aims. One way to distinguish between the two scenarios is to determine and model the spectral energy distributions (SEDs) of the masers. Methods. We observed a sample of 20 6.7 GHz methanol masers selected from the blind Arecibo survey, from centimeter to submillimeter wavelengths. We combined our observations with existing data from various Galactic plane surveys to determine SEDs from centimeter to near-infrared wavelengths. Results. We find that 70% of the masers do not have any associated radio continuum, with the rest of the sources being associated with hypercompact and ultracompact H ii regions. Modeling the SEDs shows them to be consistent with rapidly accreting massive