Miracle-dz/UniFAR-FTUs huggingface dataset:
Retinal degeneration in mice and humans with neuronal ceroid lipofuscinosis type 8

Background Ceroid lipofuscinosis type 8 belongs to a heterogenous group of vision and life-threatening neurodegenerative diseases, neuronal ceroid lipofuscinosis (NCL). Effective therapy is limited to a single drug for treatment of ceroid lipofuscinosis type 2, necessitating animal disease models to facilitate further therapeutic development. Murine models are advantageous for therapeutic development due to easy genetic manipulation and rapid breeding, however appropriate genetic models need to be identified and characterized before being used for therapy testing. To date, murine models of ocular disease associated with ceroid lipofuscinosis type 8 have only been characterized in motor neuron degeneration mice. Methods Cln8−/− mice were produced by CRISPR/Cas9 genome editing through the International Mouse Phenotyping Consortium. Ophthalmic examination, optical coherence tomography, electroretinography, and ocular histology was performed on Cln8−/− mice and controls at 16 weeks of age. Quantification of all retinal layers, retinal pigmented epithelium, and the choriocapillaris was performed using images acquired with ocular coherence tomography and planimetry of histologic sections. Necropsy was performed to investigate concurrent systemic abnormalities. Clinical correlation with human patients with CLN8-associated retinopathy is provided. Results Retinal degeneration characterized by retinal pigment epithelium mottling, scattered drusen, and retinal vascular attenuation was noted in all Cln8−/− mice. Loss of inner and outer photoreceptor segment demarcation was noted on optical coherence tomography, with significant thinning of the whole retina (P=1e-9), outer nuclear layer (P=1e-9), and combined photoreceptor segments (P=1e-9). A global reduction in scotopic and photopic electroretinographic waveforms was noted in all Cln8−/− mice. Slight thickening of the inner plexiform layer (P=0.02) and inner nuclear layer (P=0.004), with significant thinning of the whole retina (P=0.03), outer nuclear layer (P=0.01), and outer photoreceptor segments (P=0.001) was appreciated on histologic sections. Scattered lipid vacuoles were noted in splenic red pulp of all Cln8−/− mice, though no gross systemic abnormalities were detected on necropsy. Retinal findings are consistent with those seen in patients with ceroid lipofuscinosis type 8. Conclusions This study provides detailed clinical characterization of retinopathy in adult Cln8−/− mice. Findings suggest that Cln8−/− mice may provide a useful murine model for development of novel therapeutics needed for treating ocular disease in patients with ceroid lipofuscinosis type 8.
Cybercrime is (often) Boring: Infrastructure and Alienation in a Deviant Subculture

The boredom and alienation produced by capitalist societies and countervailing forces of attraction and excitement are at the heart of the subcultural account of crime. The underground hacker subculture is no exception, commonly represented as based around exciting, technically skilled practices and high-profile deviance. However, the illicit economy associated with these practices has become industrialized, developing shared infrastructures that facilitate the sale of illicit services rather than skilled technical work. We explore how this shift in the nature of work has shaped the culture and experiences of this subculture. Developing a novel concept—the ‘illicit infrastructure’—and drawing on an extensive analysis of empirical data from interviews and novel data sources such as forums and chat channels, we argue that as they industrialize, deviant subcultures can begin to replicate the division of labour, cultural tensions and conditions of alienation present in mainstream capitalist economies.
Mediating the Strain-Bullying Perpetration Relationship with Impulsivity and Social Learning: Later Sexual Harassment and Teen Dating Violence among Midwestern Middle School Students

ABSTRACT Developmental trajectories of bullying perpetration were examined across 5 waves of longitudinal panel data among Midwestern middle school students. This study explained an unobserved heterogeneity of subgroups, each having the similar pattern of trajectories and investigated antecedents and consequences of class membership. Growth-mixture modeling yielded 4 groups: (1) Chronic, (1.2%), (2) High Declining (6.2%), (3) Middle School Peak (8.1%), and (4) Low (84.4%). The overall results revealed that strain variables were found to be significant in the Chronic, High Declining, and Middle School Peak groups. The probability of the Chronic and High Declining groups was high among students with higher impulsivity. Peer delinquency and anger were found to be significant in all three groups. Members of the Chronic group were at the highest risk of sexual harassment and teen dating violence.
Modeling energy requirements in planetary ball milling of rice grain

Abstract Modified rice flours were obtained by high-impact milling. A factorial design was performed in a planetary ball mill varying rotational speed (450–650 rpm) and milling time (10–40 min) while milling energy (0.3–4.3 kJ/g), particle size distribution (PSD) of rice flour, and changes in starch structure were recorded. The effect of rotational speed and time on milling energy was satisfactorily modeled by the Response Surface Methodology. Modified flours presented bimodal PSD unlike that of the control flour, which was monomodal with a lower size dispersion. A significant reduction of D[4,3] (mean diameter) was observed as the milling energy increased. However, the effect of milling energy on PSD was dependent on milling speed. Classical milling equations provided a satisfactory fit of energy-size records at low speed (450 rpm), but at higher speed, a poor adjustment was obtained due to the increasing relevance of heat dissipation. The empirical milling equation based on linear or exponential relationships between milling energy and size reduction ratio provided a good fit for experimental data. Significant correlations between milling energy and damaged starch, crystallinity, and gelatinization degree were found. The proposed mathematical models are a contribution to the milling process design for specific modifications of rice flour.
Observed Trends in the South Asian Monsoon Low‐Pressure Systems and Rainfall Extremes Since the Late 1970s

The core Indian monsoon region receives more than half of the rainfall extremes from low‐pressure systems (LPSs), which typically form over the Bay of Bengal and propagate upstream against the time‐mean low‐level westerlies. Yet, the relationship between the trends of LPSs and rainfall extremes remains uncertain. Using two tracking algorithms and reanalyses‐derived LPSs, we find that LPS activity and extreme rainfall exhibit coherent trends during the post‐1979 satellite era. Over time, the LPSs propagate preferentially into south‐central India rather than north‐central India, imparting a corresponding dipole footprint in rainfall extremes. Consistent with existing theories that the diabatic heating is instrumental in shifting the LPSs west‐southwestward, the LPSs traveling through south‐central India have stronger updrafts on their west‐southwestern flank than those passing through north‐central India. The increased frequency of LPSs propagating into south‐central India is likely due to a strengthened cross‐equatorial moisture transport, which favors stronger storm ascents.
Financial Inclusion’s Role in Economic Growth and Human Capital in South Asia: An Econometric Approach

Many of the 2030 sustainable development goals have targeted the strengthening of financial inclusion, which is currently a key policy priority on the agendas of most governments in developing nations. The process of facilitating banking and financial services for individuals is called financial inclusion, which supports growth and the broader development goals of an economy. Although economic growth and human capital development indices have been analyzed using different proxy variables, insufficient attention has been paid to constructing a composite index for measuring this to achieve greater sustainability in terms of the economy, communities, and the environment. This study sought to address this gap using secondary data from eight countries in South Asia from 2004 to 2018. A financial inclusion index was developed through principal component analysis using an econometric approach of panel data with vector error correction models and a Granger causality test. As per the results of the study, financial inclusion has a long-run impact on human capital development in South Asian countries, whereas it has a short-run positive impact on economic growth. Domestic credits to the private sector also impact the short-run growth and human capital development in the economy. This ensures the confidence of vulnerable communities in their economy, as well as information management, and allows for quality enhancements of transactions with fewer environmental impacts. Government intervention to improve access to financial services, including ATMs and commercial banking, is one policy allowing digital finance to accelerate the achievement of sustainable development goals in South Asian countries.
Traversing the infrastructures of digital life

In this chapter I turn my attention to the infrastructural qualities inherent to the experience living with contemporary digital technologies. Digital technologies from smartphones to bitcoin, rely on infrastructural networks from undersea cables, to the hundreds of communications satellites that orbit the earth, radio communications masts, fibre-optic cables, local Wi-Fi transmitters, and mobile data communications standards. Communications protocols and programming languages also infra-structure technological devices, making the inter-operability of particular platforms possible and creating the basis for contemporary ways of communicating and socialising. Moreover, not only do digital devices rely on communications infrastructures, but infrastructures of other kinds from energy grids to global logistics are undergoing their own processes of digitisation. Digital infrastructure includes then, not only the wires and cables that support mobile and computer communication but also the integration of sensors, databases of measurements, and real-time data analytics into buildings, motorways, ticketing services, fast food delivery, taxi services and more. Digital infrastructures in one, other or both of these senses are now an inherent part of contemporary life for most people in the world and their effects on the reorganisation of social life have been profound. These digital infrastructures have provided the grounds for structural transformations in social relations, for what it is possible to know, for communication, mobility, kinship, and access to resources.
Determinants of Social Commerce Adoption: An Empirical Study of Vietnamese Consumers

Abstract Social networking website usage has been rapidly expanding ever since their introduction in the early 2000s. This strategy of social commerce is paying off with increased revenues for many businesses. Despite the growth of social commerce websites, some consumers are still reluctant to use social commerce websites. This research intends to examine the antecedents of social commerce adoption in Vietnam. Four hundred and forty-seven (447) Vietnamese social networking website users participated in this study. The findings uncover the antecedents which are significant in influencing Vietnamese consumers’ intention to engage in social commerce. The results have both practical and research implications on understanding social commerce adoption in emerging economies.
Cyclonic eddies in the West Greenland Boundary Current System

The boundary current system in the Labrador Sea plays an integral role in modulating convection in the interior basin. Four years of mooring data from the eastern Labrador Sea reveal persistent mesoscale variability in the West Greenland boundary current. Between 2014 and 2018, 197 mid-depth intensified cyclones were identified that passed the array near the 2000 m isobath. In this study, we quantify these features and show that they are the downstream manifestation of Denmark Strait Overflow Water (DSOW) cyclones. A composite cyclone is constructed revealing an average radius of 9 km, maximum azimuthal speed of 24 cm/s, and a core propagation velocity of 27 cm/s. The core propagation velocity is significantly smaller than upstream near Denmark Strait, allowing them to trap more water. The cyclones transport a 200-m thick lens of dense water at the bottom of the water column, and increase the transport of DSOW in the West Greenland boundary current by 17% relative to the background flow. Only a portion of the features generated at Denmark Strait make it to the Labrador Sea, implying that the remainder are shed into the interior Irminger Sea, are retroflected at Cape Farewell, or dissipate. A synoptic shipboard survey east of Cape Farewell, conducted in summer 2020, captured two of these features which shed further light on their structure and timing. This is the first time DSOW cyclones have been observed in the Labrador Sea—a discovery that could have important implications for interior stratification.
The International Transmission of Local Economic Shocks Through Migrant Networks

Using newly validated data on geographic migration networks, we study how labor demand shocks in the United States propagate across the border with Mexico. We show that the large exogenous decline in US employment brought about by the Great Recession affected demographic and economic outcomes in Mexican communities that were highly connected to the most affected markets in the US. In the Mexican locations with strong initial ties to the hardest hit US migrant destinations, return migration increased, emigration decreased, and remittance receipt declined. These changes significantly increased local employment and hours worked, but wages were unaffected. Investment in durable goods and children's education also slowed in these communities. These findings document the effects in Mexico when potential migrants lose access to a strong US labor market, providing insight regarding the potential impacts of stricter US migration restrictions.
Particle Size Distributions of PCDD/Fs and PBDD/Fs in Ambient Air in a Suburban Area in Beijing, China

The particle size distributions of polychlorinated and polybrominated dibenzo-p-dioxins and dibenzofurans (PCDD/Fs and PBDD/Fs, respectively; together labeled PXDD/Fs) in ambient air in a suburban area in Beijing, China, were determined. The sums of the concentrations of the 17 2,3,7,8-PCDD/Fs and the sums of the concentrations of the 13 2,3,7,8-PBDD/Fs that were analyzed were 1499-2799 fg m(-3) (95.4-175.4 fg I-TEQ m(-3)) and 1171-2424 fg m(-3) (42.2-109.3 fg TEQ m(-3)), respectively. The PXDD/Fs were mainly (similar to 90%) in the particulate phase. Significant linear correlations were found between the gas/particle partition coefficients (K-p) and subcooled liquid vapor pressures (P-L(0)) of the PXDD/Fs. The regression coefficients indicated that the PCDD/Fs were mainly adsorbed to the particles and that the PBDD/Fs were mainly absorbed by the particles. The concentrations of the PXDD/Fs increased as the particle size decreased. The highest PXDD/F concentrations were found in the d(ae) < 1.0 mu m particles and more than 80% of the PXDD/Fs were found to be in the dae < 2.5 mu m particles. Similar regression coefficients were found for the K-P against P-L(0) for the different particle size fractions in the air. The PXDD/F distribution profiles in particles of different sizes were also studied. The lower chlorinated PCDD/Fs were found at higher concentrations in the coarser particles, and the higher chlorinated PCDD/Fs were mainly found in the finer particles. Polybrominated dibenzofurans, particularly the higher brominated dibenzofurans, were the dominant PBDD/F congeners. The contributions of the higher brominated dibenzofurans to the total PBDD/F concentrations decreased as the particle size increased, but that was not the case for the polybrominated dibenzo-p-dioxins.
SARS-CoV-2 Nonstructural Protein 1 Inhibits the Interferon Response by Causing Depletion of Key Host Signaling Factors

SARS-CoV-2 is the causative agent of COVID-19, a serious disease that can have a myriad of symptoms from loss of taste and smell to pneumonia and hypercoagulation. The rapid spread of SARS-CoV-2 can be attributed in part to asymptomatic transmission, where infected individuals shed large amounts of virus before the onset of disease. ABSTRACT Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the causative agent of the ongoing coronavirus disease 2019 (COVID-19) pandemic. While previous studies have shown that several SARS-CoV-2 proteins can antagonize the interferon (IFN) response, some of the mechanisms by which they do so are not well understood. In this study, we describe two novel mechanisms by which SARS-CoV-2 blocks the IFN pathway. Type I IFNs and IFN-stimulated genes (ISGs) were poorly induced during SARS-CoV-2 infection, and once infection was established, cells were highly resistant to ectopic induction of IFNs and ISGs. Levels of two key IFN signaling pathway components, Tyk2 and STAT2, were significantly lower in SARS-CoV-2-infected cells. Expression of nonstructural protein 1 (NSP1) or nucleocapsid in the absence of other viral proteins was sufficient to block IFN induction, but only NSP1 was able to inhibit IFN signaling. Mapping studies suggest that NSP1 prevents IFN induction in part by blocking IRF3 phosphorylation. In addition, NSP1-induced depletion of Tyk2 and STAT2 dampened ISG induction. Together, our data provide new insights into how SARS-CoV-2 successfully evades the IFN system to establish infection. IMPORTANCE SARS-CoV-2 is the causative agent of COVID-19, a serious disease that can have a myriad of symptoms from loss of taste and smell to pneumonia and hypercoagulation. The rapid spread of SARS-CoV-2 can be attributed in part to asymptomatic transmission, where infected individuals shed large amounts of virus before the onset of disease. This is likely due to the ability of SARS-CoV-2 to effectively suppress the innate immune system, including the IFN response. Indeed, we show that the IFN response is efficiently blocked during SARS-CoV-2 infection, a process that is mediated in large part by nonstructural protein 1 and nucleocapsid. Our study provides new insights on how SARS-CoV-2 evades the IFN response to successfully establish infection. These findings should be considered for the development and administration of therapeutics against SARS-CoV-2.
Analysis of Outdoor Thermal Discomfort Over the Kingdom of Saudi Arabia

In this study, the variability and trends of the outdoor thermal discomfort index (DI) in the Kingdom of Saudi Arabia (KSA) were analyzed over the 39‐year period of 1980–2018. The hourly DI was estimated based on air temperature and relative humidity data obtained from the next‐generation global reanalysis from the European Center for Medium‐Range Weather Forecasts and in‐house high‐resolution regional reanalysis generated using an assimilative Weather Research Forecast (WRF) model. The DI exceeds 28°C, that is, the threshold for human discomfort, in all summer months (June to September) over most parts of the KSA due to a combination of consistently high temperatures and relative humidity. The DI is greater than 28°C for 8–16 h over the western parts of KSA and north of the central Red Sea. A DI of >28°C persistes for 7–9 h over the Red Sea and western KSA for 90% of summer days. The spatial extent and number of days with DI > 30°C, that is, the threshold for severe human discomfort, are significantly lower than those with DI > 28°C. Long‐term trends in the number of days with DI > 28°C indicate a reduced rate of increase or even a decrease over some parts of the southwestern KSA in recent decades (1999–2018). Areas with DI > 30°C, in particular the northwestern regions of the Arabian Gulf and its adjoining regions, also showed improved comfort levels during recent decades. Significant increases in population and urbanization have been reported throughout the KSA during the study period. Analysis of five‐years clinical data suggests a positive correlation between higher temperatures and humidity with heat‐related deaths during the Hajj pilgrimage. The information provided herein is expected to aid national authorities and policymakers in developing necessary strategies to mitigate the exposure of humans to high levels of thermal discomfort in the KSA.
The Cyclic Self: Menstrual Cycle Tracking as Body Politics

Tracking in/fertility—through ovulation biosensing, menstrual and perimenopausal apps, and ovarian reserve testing—is becoming increasingly commonplace amongst relatively privileged women in the Global North. Taking place on and through platforms comprised of devices, bodies, and discourses, such self-tracking articulates forms of in/fertility and reproductive futures that are, we argue, closely entwined with emerging forms of biomedical capitalization. While reproductive medicine focused on the creation of children has been entwined with corporate interests since the development of in vitro fertilization in the 1980s, fertility as an asset, or future value, is increasingly targeted by the new innovation sectors as a specific capacity, separable from reproduction per se, in which women should invest if they are not to fall prey to incipient infertility. Synthesizing our separate empirical work in this field, this paper theorizes the connections between the emergence of self-tracking logics and cultures, the burgeoning of consumer-oriented, clinical services, and contemporary social anxieties around fertility decline. Even in countries such as Australia and the United Kingdom, where birth rates are stable, (some) women’s fertility is being refigured as precious and vulnerable, something to be tracked, documented, and attended to in the name of individual future happiness and fulfilment. Women with enough financial and cultural capital are encouraged to monitor their periods, come to know their ovulation patterns, and become aware of their ovarian reserve, and, importantly, to act prudently on such knowledge to safeguard their reproductive futures.
Learning styles in counseling: a scoping review of the empirical evidence

ABSTRACT The concept of learning styles is popular among educators and the general public; however, many have highlighted the lack of empirical evidence supporting its relevance. We conducted a systematic literature search to identify the scope of empirical studies on learning styles applied to counseling and counseling education. Only 17 empirical studies were identified, indicating a relative lack of research. Four studies reported on the application of learning styles in treatments (of either the client, the counselor, and/or the treatment), suggesting that matching learning styles of intervention and client might be beneficial. Thirteen studies reported on learning styles in training settings (of students and their supervisor/teacher or field instructor). Although students’ learning styles appeared flexible, social work students frequently reported an active experiential learning style and counseling students as well as more experienced clinicians appeared more abstract and reflective. The majority of studies were cross-sectional survey studies and many reported minimal psychometric data on the used self-report measures of learning styles, which appeared to conflate the concepts of learning preference and learning ability. At present, there is no adequate evidence to justify incorporating learning styles assessments into counseling practice or training.