Miracle-dz/UniFAR-FTUs huggingface dataset:
Substrate Deprivation Therapy to Reduce Glycosaminoglycan Synthesis Improves Aspects of Neurological and Skeletal Pathology in MPS I Mice

Mucopolysaccharidosis type I (MPS I) is the most common form of the MPS group of genetic diseases. MPS I results from a deficiency in the lysosomal enzyme α-l-iduronidase, leading to accumulation of undegraded heparan and dermatan sulphate glycosaminoglycan (GAG) chains in patient cells. MPS children suffer from multiple organ failure and die in their teens to early twenties. In particular, MPS I children also suffer from profound mental retardation and skeletal disease that restricts growth and movement. Neither brain nor skeletal disease is adequately treated by current therapy approaches. To overcome these barriers to effective therapy we have developed and tested a treatment called substrate deprivation therapy (SDT). MPS I knockout mice were treated with weekly intravenous injections of 1 mg/kg rhodamine B for six months to assess the efficacy of SDT. Mice were assessed using biochemistry, micro-CT and a battery of behaviour tests to determine the outcome of treatment. A reduction in female bodyweight gain was observed with the treatment as well as a decrease in lung GAG. Behavioural studies showed slight improvements in inverted grid and significant improvements in learning ability for female MPS I mice treated with rhodamine B. Skeletal disease also improved with a reduction in bone mineral volume observed. Overall, rhodamine B is safe to administer to MPS I knockout mice where it had an effect on improving aspects of neurological and skeletal disease symptoms and may therefore provide a potential therapy or adjunct therapy for MPS I patients.
Kiswahili, a Continental Language: How Possible is it? (Part One)

African leaders, officials of the Organisation of African Unity, African citizens of the continent and sympathizers of African problems have for many years now been calling for one or more African languages to be used as the continent's official lingua franca(s), for official as well as intra-national business, and other social interactions in place of colonial languages. Among the languages most favoured for this task is Kiswahili. What, it seems, the advocates of the use of Kiswahili as Africa's lingua franca have not taken into account is how far these hopes, aspirations and objectives can be attained satisfactorily. For example, how easy will it be to use Kiswahili for official national business or for intra-national transactions and communication? Another question which is equally relevant is for what purpose will Kiswahili as a continental lingua franca serve? We need to bear in mind that in terms of prestige and social status, education and social advancement, science, technology and international relations, European 'colonial' languages still predominate in Africa and in African affairs. It is undeniable that Kiswahili is a negro-african language of black peoples enriched by contacts with Oriental and European cultures for the expression of 'international knowledge' (c.f. Amidu 1995). Even so, we observe that on the African continent Arabic is predominant in the north of Africa as a lingua franca, Hausa is widely used in West Africa as another lingua franca, Lingala is used in Zaire, Congo-Brazzaville, and the Central African Republic as a lingua-franca, and Zulu is also widely used in South Africa. Apart from these, there are a few lingua francas peculiar to some African nation-states: for example, Akan 1
Smoothing the LPM Estimate of the Frequency Response Function Via an Impulse Response Truncation Technique

A statistical impulse response truncation technique is applied to the local polynomial method (LPM)-estimate of the frequency response function (FRF), resulting in an improved, smooth FRF. Formulated as a nonparametric linear-least-squares-estimate, the LPM is first applied to estimate the FRF from a full data record of a single-input-single-output system, systematically expressed in an output-error framework. The smooth characteristics of both the exact FRF and the leakage from transients allow for an optimal application of the local polynomial method, leading to the elimination of both the leakage and interpolation errors. The truncation method introduced in this paper makes it possible for the user to fine-tune the tradeoff between the uncertainty (variance) and the bias on the estimated instantaneous FRF.
Lessons from interdisciplinary qualitative research: learning to work against a single story

In this article, we consider the everyday practices and methodological and theoretical tensions of interdisciplinary, qualitative work. In particular, we discuss the varied interpretations of focus group data from Burundian men and women with refugee status and explore the consequences of representations that result in deficit-based understandings. We highlight how through our research process we learned that following participants, rather than leading with our disciplines, deepened our understanding and complicated our representations.
Performance Modeling of UML-Based Software Specifications : A Comparative Study

Assessing software quality attributes is one of the most sought-after topics in recent software engineering research literature. Performance characteristics of the software systems, responsiveness and scalability in particular, are of the most important attributes determining the software quality. The ability to assess the software performance as early as possible in the software life cycle is highly desirable. It would be less costly to detect and rectify performance problems in the software architectural design. In the recent years, the Unified Modeling Language (UML) is becoming the de-facto standard for software development. This success is due to its capability to provide different views of the software, resulting in a clearer understanding. Performance analysis of software specifications modeled in UML is drawing much attention in both industry and research communities. A wide-array of approaches has been introduced over the last few years to address this problem. In this paper we present the major trends in performance modeling of UML-based software specifications over the last two years in particular. Throughout this paper, we compare these approaches in terms of their applicability, limitations, generality and deficiencies. Our objective is to highlight future research areas and to compile a set of recommended strategies to better enhance current methodologies.
Structural Characterizations of Palladium Clusters Prepared by Polyol Reduction of [PdCl4]2− Ions

Palladium nanoparticles are of great interest in many industrial fields, ranging from catalysis and hydrogen technology to microelectronics, thanks to their unique physical and chemical properties. In this work, palladium clusters have been prepared by reduction of [PdCl4]2− ions with ethylene glycol, in the presence of poly(N-vinyl-2-pyrrolidone) (PVP) as stabilizer. The stabilizer performs the important role of nucleating agent for the Pd atoms with a fast phase separation, since palladium atoms coordinated to the polymer side-groups are forced at short distances during nucleation. Quasispherical palladium clusters with a diameter of ca. 2.6 nm were obtained by reaction in air at 90°C for 2 hours. An extensive materials characterization by transmission electron microscopy (TEM), X-ray diffraction (XRD), X-ray photoelectron spectroscopy (XPS), and other characterizations (TGA, SEM, EDS-SEM, and UV-Vis) has been performed in order to evaluate the structure and oxidation state of nanopalladium.
Estimation of the Electrode-Fiber Bioelectrical Coupling From Extracellularly Recorded Single Fiber Action Potentials

Selective peripheral neural interfaces are currently capable of detecting minute electrical signals from nearby nerve fibers as single fiber action potential (SFAP) waveforms. Each detected single unit has a distinct shape originating from the unique bioelectrical coupling that exists between the neuroprosthetic electrode, the nerve fiber and the extracellular milieu. The bioelectrical coupling manifests itself as a series of low-pass Bessel filters acting on the action currents along the nerve fiber. Here, we present a method to estimate the electrode-fiber bioelectrical coupling through a quantitative analysis of the spectral distribution of the single units extracellularly recorded with the thin-film longitudinal intrafascicular electrode (tfLIFE) in an in vivo mammalian peripheral nerve animal model. The bioelectrical coupling estimate is an estimate of the electrode sensitivity function traversed by the nerve fiber, suggesting that it is as a means to directly measure the spatial relationship between the nerve fiber and electrode. It not only reflects a shape change to the SFAP, but has implications for in situ nerve fiber location tracking, in situ diagnostics of nerves and neuroproshetic electrodes, and assessment of the biocompatibility of neural interfaces and the health of the reporting nerve fibers.
Work status and productivity costs due to ankylosing spondylitis: comparison of three European countries

Objective: To compare work disability, sick leave, and productivity costs due to ankylosing spondylitis (AS) of three European countries. Methods: 216 patients with AS from the Netherlands, France, and Belgium participated in a two year observational study. Employment and work disability rates at baseline were adjusted for age and sex. Productivity costs were calculated by both the friction cost method and the human capital approach. The adjusted contributions of country to employment, work disability, and having an episode of sick leave were assessed by logistic regression and the contribution of the country to days of sick leave and costs by Cox proportional hazard analysis. Results: 209 patients completed the two years' follow up with sufficient data for cost analysis. Adjusted employment was 55% in the Netherlands as compared with 72% in both other countries and only in the Netherlands was it lower than expected in the general population. Adjusted work disability was 41%, 23%, and 9% in the Netherlands, France, and Belgium and in all countries was higher than expected in the general population. In those with a paid job, the mean number of days of sick leave per patient per year because of AS was 19 (range 0–130), six (range 0–77), and nine (range 0–60 ) in the Netherlands, France, and Belgium respectively. Applying the friction cost method to those with a paid job resulted in mean costs per patient per year of 1257 euros (range 0–7356), 428 euros (range 0–5979), and 476 euros (range 0–2354) in the Netherlands, France, and Belgium. Applying the human capital approach to the whole group resulted in mean costs per patient per year of 8862 euros (range 0–46 818), 3188 euros (range 0–43 550), and 3609 euros (range 0–34 320) in the three countries, respectively. After adjusting for sociodemographic and disease characteristics, living in the Netherlands, as compared with both other countries, was associated with a higher chance of being work disabled (odds ratio (OR)=3.82; 95% confidence interval (CI) 1.33 to 11.01), but not with the risk of having an episode of sick leave. Similarly, living in the Netherlands contributed independently to the number of days sick leave (OR=0.65; 95% CI 0.43 to 0.97), a higher amount of friction costs (OR=0.63; 95% CI 0.42 to 0.96), and a higher amount of human capital costs (OR=0.46; 95% CI 0.32 to 0.68). Conclusion: There are remarkable differences in work status and productivity costs between the three European countries. This has implications for the generalisability of health economic studies.
Polymerization and mechanical properties of single RecA-DNA filaments.

The polymerization of individual RecA-DNA filaments, containing either single-stranded or double-stranded DNA, was followed in real time, and their mechanical properties were characterized with force-measuring laser tweezers. It was found that the stretch modulus of a filament is dominated by its (central) DNA component, while its bending rigidity is controlled by its (eccentric) protein component. The longitudinal stiffness of DNA increases 6- to 12-fold when the DNA is contained in the protein helix. Both the stretch modulus and the bending rigidity of a fiber change in the presence of various nucleotide cofactors-e.g., [gamma-thio]ATP, ATP, and ADP-indicating a substantial re-arrangement of spatial relationships between the nucleic acid and the protein scaffold. In particular, when complexed with ATP, a fiber becomes twice as extensible as a [gamma-thio]ATP fiber, suggesting that 32% of the DNA-binding sites have been released in its core. Such release may enable easy rotation of the DNA within the protein helix or slippage of the DNA through the center of the protein helix.
Perceptions of skill development in a living-learning first-year experience program

The purpose of this study was to explore the perceptions of students and faculty involved in a living-learning first-year experience program at a small, liberal arts institution about developing skills for life-long learning including critical thinking, written communication, and reflection and engagement across disciplines. The researcher conducted a qualitative case study including focus groups with students and interviews with faculty involved with the first-year experience program. Three focus groups with a total of 19 student participants and individual interviews with 11 faculty participants were conducted. Interviews and focus groups were digitally recorded and then the researcher listened to each session multiple times to discern common themes and direct quotes. The findings demonstrated that: (a) Students felt that their critical thinking and written communication skills were positively influenced through the program. (b) Students felt that the attempt to enhance reflection and engagement across disciplines through co-curricular programming was not as successful. (c) Relationships between peers and faculty were two of the most important aspects of the living-learning first-year experience program for students. (d) Faculty similarly felt that critical thinking and written communication skills were positively influenced. (e) Faculty differed from the student opinion on the program’s impact on reflection and engagement across disciplines and felt that the co-curricular programming helped to positively influence the development of multi-disciplinary perspectives. Recommendations for improvements included a stronger focus on the relationship between student affairs staff and academic affairs, more intentionality for co-curricular programming, increased uniformity among first-year seminars, and improvements in marketing of the program to internal and external stakeholders.
A coupled reaction-diffusion field model for perception-action cycle with applications to robot navigation

A generalised reaction-diffusion field model for robot navigation is proposed. It utilises two mutually antagonistic neural fields counteracting in patterns similar to that of flexor/extensor muscles controlling the movements in major joints in the human body. Combining local activation and generalised inhibition represented by Amari's neural field equations and extended by the Fitzhugh-Nagumo and Wilson-Cowan activator-inhibitor systems, results in the type of neural attractor dynamics that may lead to spontaneous oscillatory pattern formation. Preliminary simulation data suggest that this approach has utility in enabling a team of autonomous vehicles to navigate in a crowded pedestrian crossing.
Disentangling Ethnicity and Context as Predictors of Parenting Within Rural African American Families

This study will address the initial question: Are there ethnic differences in parenting that remain when contextual variables are controlled and are related to culture, focusing on two samples of rural African American families. This study is part of a series of coordinated studies presented in this special issue (Le et al., 2008). Specific attention was given to ascertaining whether these differences were explained by contextual factors, such as socioeconomic factors (i.e., parents' educational level, homeownership, family structure, and number of children in the household). Finally, this study examined whether any differences in parenting (warmth, monitoring, communication) were attributed to cultural factors, after controlling for the contributions of contextual factors. Results indicate that parental education, family size, negative life events, racial discrimination, neighborhood characteristics, and religiosity were significantly associated with various domains of parenting among rural African Americans.
Model-Driven Design of Ensemble-Based Component Systems

In this research abstract we describe our approach towards the design of ensemble-based component systems. Our motivation lies in the fact that, in these systems, tracing the behavior of individual constituents to system-level goals and requirements is challenging. Our approach is based on a novel invariant-based model that achieves the desired traceability. Along with using the model in a method that allows for systematic contractual design, we employ the model at runtime to achieve dynamic adaptation on the basis of requirements reflection.
Behavioral Interpretations of Intrinsic Connectivity Networks

An increasingly large number of neuroimaging studies have investigated functionally connected networks during rest, providing insight into human brain architecture. Assessment of the functional qualities of resting state networks has been limited by the task-independent state, which results in an inability to relate these networks to specific mental functions. However, it was recently demonstrated that similar brain networks can be extracted from resting state data and data extracted from thousands of task-based neuroimaging experiments archived in the BrainMap database. Here, we present a full functional explication of these intrinsic connectivity networks at a standard low order decomposition using a neuroinformatics approach based on the BrainMap behavioral taxonomy as well as a stratified, data-driven ordering of cognitive processes. Our results serve as a resource for functional interpretations of brain networks in resting state studies and future investigations into mental operations and the tasks that drive them.
Onix: A Distributed Control Platform for Large-scale Production Networks

Computer networks lack a general control paradigm, as traditional networks do not provide any network-wide management abstractions. As a result, each new function (such as routing) must provide its own state distribution, element discovery, and failure recovery mechanisms. We believe this lack of a common control platform has significantly hindered the development of flexible, reliable and feature-rich network control planes. To address this, we present Onix, a platform on top of which a network control plane can be implemented as a distributed system. Control planes written within Onix operate on a global view of the network, and use basic state distribution primitives provided by the platform. Thus Onix provides a general API for control plane implementations, while allowing them to make their own trade-offs among consistency, durability, and scalability.
Decreased Energy Capacity and Increased Autophagic Activity in Optic Nerve Axons With Defective Anterograde Transport.

PURPOSE Autophagy is a critical process, compromised in neurodegenerative disease, by which terminally differentiated cells like neurons manage cytoskeletal and organelle turnover. How autophagy relates to associated neurodegenerative pathologies remain unclear. We examined autophagy in optic neuropathy by investigating cytoskeletal degradation, mitochondria, and autophagic vesicles in the DBA2/J mouse model of glaucoma exhibiting differing levels of axon transport functionality. METHODS DBA/2J and DBA/2J(wt-gpnmb) control mice 11 to 14 months of age were injected with cholera toxin-B (CTB) to assay anterograde axonal transport. Axonal mitochondria and autophagic vesicles were analyzed with respect to transport integrity in proximal and distal optic nerve using serial block face scanning electron microscopy (3D EM). RESULTS Several indices varied significantly between the DBA/2J and DBA/2J(wt-gpnmb) mice, including mitochondrial volume, average number of autophagic vesicles per axon, and mitochondrial cristae. However, there were no differences in mitochondrial cristae for axons with functional versus dysfunctional CTB transport, suggesting that mitochondrial dysfunction precedes overt transport blockade. Anterograde transport failure was accompanied by a dissociation of the relationship between mitochondrial and axon volumes. Autophagic vesicle profiles were significantly increased in optic nerve with transport deficit, consistent with greater autophagic activity. Mitochondria within autophagosomes, indicative of mitophagy, were observed in both proximal and distal axons. CONCLUSIONS Loss of anterograde transport in DBA/2J optic nerve is concomitant with diminished mitochondrial volume, increased cytoskeletal breakdown and autophagic activity, and accumulation of autophagic profiles, including signs of mitophagy, in proximal optic nerve. Axons with transport deficit are metabolically underserved, though not necessarily from mitophagy.
EXPLORER: an interactive floorplanner for design space exploration

An interactive floor planner based on the genetic algorithm is presented. Layout area, aspect ratio, routing congestion and maximum path delay are optimized simultaneously. The design requirements are refined interactively as knowledge of the obtainable cost tradeoffs is gained and a set of feasible solutions representing alternative, good tradeoffs is generated. Experimental results illustrate the special features of the approach.
Numerical Experiments on Solute Transport in Ground Water Flow Systems

This paper proposes groundwater flow simulation models based on explicit finite difference method (FDFLOW) and Galerkin finite element method (FEFLOW) and solute transport simulation models based on Galerkin finite element method (FESOLUTE), random walk method (RWSOLUTE) and modified method of characteristic (MMOCSOLUTE). These coupled flow and transport models are aimed at simulation of solute transport in two-dimensional, transient, unconfined groundwater flow systems. Proposed models are validated for chosen test cases. Sensitivity of model solutions to various flow and transport parameters viz. transmissivity, pumping rate, longitudinal and transverse dispersivity and effective porosity is analyzed. Further best-performing coupled flow and solute transport model amongst the proposed ones using the criteria of numerical oscillations and average global mass balance error is decided. It is found that the best-performing model is FEFLOW-MMOCSOLUTE model whose adequacy is ascertained by applying it to a Mahi Right Bank Canal (MRBC) command area aquifer in Gujarat state of India. Results showed that the computed head and TDS concentration distributions are in close agreement with the available field observations.