Miracle-dz/UniFAR-FTUs huggingface dataset:
Disease stage determines the efficacy of treatment of a paediatric neurodegenerative disease

Lysosomal storage disorders are a large group of inherited metabolic conditions resulting from the deficiency of proteins involved in lysosomal catabolism, with resulting accumulation of substrates inside the cell. Two‐thirds of these disorders are associated with a neurodegenerative phenotype and, although few therapeutic options are available to patients at present, clinical trials of several treatments including lysosomal enzyme replacement are underway. Although animal studies indicate the efficacy of pre‐symptomatic treatment, it is largely unknown whether symptomatic disease‐related pathology and functional deficits are reversible. To begin to address this, we used a naturally‐occurring mouse model with Sanfilippo syndrome (mucopolysaccharidosis type IIIA) to examine the effectiveness of intracisternal cerebrospinal fluid enzyme replacement in early, mid‐ and symptomatic disease stage mice. We observed a disease‐stage‐dependent treatment effect, with the most significant reductions in primary and secondary substrate accumulation, astrogliosis and protein aggregate accumulation seen in mucopolysaccharidosis type IIIA mice treated very early in the disease course. Affected mice treated at a symptomatic age exhibited little change in these neuropathological markers in the time‐frame of the study. Microgliosis was refractory to treatment regardless of the age at which treatment was instigated. Although longer‐term studies are warranted, these findings indicate the importance of early intervention in this condition.
Sulfur and sulfur amino acids.

We may not be able to make you love reading, but sulfur and sulfur amino acids will lead you to love reading starting from now. Book is the window to open the new world. The world that you want is in the better stage and level. World will always guide you to even the prestige stage of the life. You know, this is some of how reading will give you the kindness. In this case, more books you read more knowledge you know, but it can mean also the bore is full.
Seasonality in six enterically transmitted diseases and ambient temperature

We propose an analytical and conceptual framework for a systematic and comprehensive assessment of disease seasonality to detect changes and to quantify and compare temporal patterns. To demonstrate the proposed technique, we examined seasonal patterns of six enterically transmitted reportable diseases (EDs) in Massachusetts collected over a 10-year period (1992–2001). We quantified the timing and intensity of seasonal peaks of ED incidence and examined the synchronization in timing of these peaks with respect to ambient temperature. All EDs, except hepatitis A, exhibited well-defined seasonal patterns which clustered into two groups. The peak in daily incidence of Campylobacter and Salmonella closely followed the peak in ambient temperature with the lag of 2–14 days. Cryptosporidium, Shigella, and Giardia exhibited significant delays relative to the peak in temperature (~40 days, P<0·02). The proposed approach provides a detailed quantification of seasonality that enabled us to detect significant differences in the seasonal peaks of enteric infections which would have been lost in an analysis using monthly or weekly cumulative information. This highly relevant to disease surveillance approach can be used to generate and test hypotheses related to disease seasonality and potential routes of transmission with respect to environmental factors.
Event‐related potentials reflect heterogeneity of developmental prosopagnosia

Event‐related potential (ERP) studies of developmental prosopagnosia (DP) are rare. Previous ERP investigations have reported smaller N170 amplitude differences between faces and objects in at least three prosopagnosics. The present study is based on a combination of behavioural and electrophysiological assessment of face processing. The aim was to investigate the face‐specifity of the N170 in both healthy subjects and a group of DP individuals (N = 4), using famous and nonfamous faces, caricatures and houses as stimuli. All prosopagnosic subjects showed impairments in recognition of famous faces, memory for faces and learning new faces in behavioural assessment. In healthy subjects the largest effects were found at parieto‐occipital electrode positions (PO7 and PO8), along with a familiarity effect at these electrode positions. Thus, parieto‐occipital areas appear to play an important role in face processing. Three prosopagnosics showed reliable N170 amplitude differences between faces and nonface stimuli, whereas one DP individual showed significantly reduced amplitude differences between faces and nonface objects. The behavioural and electrophysiological data support the idea that DP reflects a heterogeneous impairment and that face processing deficits are not necessarily correlated with a lack of face‐specific N170.
Sources and Amounts of Animal, Dairy, and Plant Protein Intake of US Adults in 2007–2010

Dietary guidelines suggest consuming a mixed-protein diet, consisting of high-quality animal, dairy, and plant-based foods. However, current data on the distribution and the food sources of protein intake in a free-living, representative sample of US adults are not available. Data from the National Health and Nutrition Examination Survey (NHANES), 2007–2010, were used in these analyses (n = 10,977, age ≥ 19 years). Several US Department of Agriculture (USDA) databases were used to partition the composition of foods consumed into animal, dairy, or plant components. Mean ± SE animal, dairy, and plant protein intakes were determined and deciles of usual intakes were estimated. The percentages of total protein intake derived from animal, dairy, and plant protein were 46%, 16%, and 30%, respectively; 8% of intake could not be classified. Chicken and beef were the primary food sources of animal protein intake. Cheese, reduced-fat milk, and ice cream/dairy desserts were primary sources of dairy protein intake. Yeast breads, rolls/buns, and nuts/seeds were primary sources of plant protein intake. This study provides baseline data for assessing the effectiveness of public health interventions designed to alter the composition of protein foods consumed by the American public.
Universality of local spectral statistics of random matrices

The Wigner-Gaudin-Mehta-Dyson conjecture asserts that the local eigenvalue statistics of large random matrices exhibit universal behavior depending only on the symmetry class of the matrix ensemble. For invariant matrix models, the eigenvalue distributions are given by a log-gas with inverse temperature $\beta = 1, 2, 4$, corresponding to the orthogonal, unitary and symplectic ensembles. For $\beta \not \in \{1, 2, 4\}$, there is no matrix model behind this model, but the statistical physics interpretation of the log-gas is still valid for all $\beta > 0$. The universality conjecture for invariant ensembles asserts that the local eigenvalue statistics are independent of $V$. In this article, we review our recent solution to the universality conjecture for both invariant and non-invariant ensembles. We will also demonstrate that the local ergodicity of the Dyson Brownian motion is the intrinsic mechanism behind the universality. Furthermore, we review the solution of Dyson's conjecture on the local relaxation time of the Dyson Brownian motion. Related questions such as delocalization of eigenvectors and local version of Wigner's semicircle law will also be discussed.
A bag-of-words approach for assessing activities of daily living using wrist accelerometer data

Most physical activity (PA) assessment studies use wearable accelerometers attached to the hip. However, there is significant and recent interest in understanding the usefulness of wrist based accelerometer data collection due to ease of use and higher compliance. This paper develops machine learning methods for identifying activity types and computing energy expenditures. Our approach converts the raw time series data into intermediate variables or features using standard statistical methods as well as bag-of-words (BoW) approach. We tested this approach to assess type of physical activities as well as estimate the required corresponding energy expenditure. The method was evaluated on 17 participants. Each of the participants wore an Actigraph GT3X+ accelerometer on the right wrist and performed 33 activities of daily living. Energy expenditure was measured in parallel by a portable indirect calorimetry system. Our results show that the BoW approach resulted in a more accurate model for PA identification (F1-score = 0.88 and 0.91 for sedentary and locomotion detection, respectively), compared with standard statistical summaries. The BoW approach preserved additional details about the accelerometer data, which resulted in distinguishing different activities that belonged to the same higher-level category (e.g., distinguishing leisure walk from stair ascent where both PAs belong to the locomotion class) and consequently yielding an accurate energy expenditure estimation model for PAs (rMSE = 0.93 and R2 = 0.69).
Combined Effect of Foot Arch Structure and an Orthotic Device on Stress Fractures

In a prospective study, quantitative measures of the structure of the longitudinal arch of the foot were established and related to the incidence of stress fractures in the bones of the lower limbs of military recruits. In addition, the role of a semirigid orthotic device (Langer military stress orthotic) in preventing stress fractures was evaluated as a function of the structure of the longitudinal arch. Femoral and tibial stress fractures were found to be more prevalent in the presence of feet with high arches, whereas the incidence of metatarsal fractures was higher in feet with low arches. The use of an orthotic device reduced the incidence of femoral stress fractures only in the presence of feet with high arches and the incidence of metatarsal fractures only among feet with low arches. The findings suggest that the normal foot with a low arch acts as a better shock absorber than the normal foot with a high arch, and that an orthotic device may improve the shock absorbing capacity of the arch.
Fintie-difference time-domain simulation of dispersive layered saw filters including electrode massloading

The finite-difference time-domain (FDTD) method is extended to analyze layered surface acoustic wave (SAW) filters. In this method, the partial derivatives of quasi-static Maxwell's equations and the equation of motion are discretized to centered finite-differences. In addition, the perfectly matched layer (PML) boundary condition is applied to reduce the spurious reflections. The proposed method is used to analyze a ZnO/Diamond/Si layered SAW filter with electrode massloading. The simulated results are in a good agreement with the existing experimental data, indicating that the FDTD method is an appropriate approach for modeling SAW devices.
High-performance partitioned-stator switched flux memory machines with hybrid magnets on external stator for traction applications

This paper proposes and investigates a new topology of switched flux memory machine, in which the hybrid magnets and armature windings are separately located on external and inner stators, respectively. Due to the effective online permanent magnet (PM) magnetization, the excitation copper loss can be eliminated. Hence, the high efficiency can be maintained across a wide range of speeds and loads, which is desirable for traction applications. Meanwhile, the partitioned stator (PS) design is beneficial to the alleviation of geometric conflicts between magnetic and electric loadings. In addition, the fact that armature windings are located in the inner stator leads to the increase of effective split ratio similar to an external-rotor machine design. Consequently, the torque capability can be further improved compared to its inner-PM counterpart. The machine topologies having PMs mounted on either external or internal stator are introduced first. The design considerations for the slot/pole combination are investigated. Based on the identical basic specifications, the major electromagnetic characteristics of the optimally designed external-PM machine are investigated in comparison to those of its internal-PM counterpart. The analysis results reveal that the EPM-PS-SFMM can provide wider flux regulation range, higher torque capability as well as lower torque ripple than its IPM counterpart, which confirms the validity for the proposed design.
Design and power production of small-scale wind turbines

Wind is one of the most used renewable energy sources for general applications due to its power availability. There are several studies that use wind energy in small-scale systems, however few analyse the lower then 10000 Reynolds number range and the usual approach consists in apply a commercial turbine, or adapt one from other system, to a commercial generator, providing low efficiency system. This paper uses Schmitz theory to design wind turbines in the centimetre scale based on three airfoils, BW3, E62 and S1223. A wind tunnel was used to determine the power coefficient and maximum power transfer, with a small generator. However, the problem with the use of wind energy in urban areas is the variability of the available power since most of the time the wind speed is very low. Therefore, this work includes a study on remote monitoring using a wind turbine to feed a wireless sensor node and a vertical axis wind turbine was used to compare the systems behaviour. The results demonstrate these systems are capable to feed the load and charge a battery even with very low average wind speed.
Chebyshev polynomial approximation to approximate partial differential equations

This pa per suggests a simple method based on Chebyshev approximation at Chebyshev nodes to approximate partial differential equations. The methodology simply consists in determining the value function by using a set of nodes and basis functions. We provide two examples. Pricing an European option and determining the best policy for chatting down a machinery. The suggested method is flexible, easy to program and efficient. It is also applicable in other fields, providing efficient solutions to complex systems of partial differential equations.
Elements in the immunoglobulin heavy-chain enhancer directly regulate simian virus 40 ori-dependent DNA replication

In a previous study, we showed that the immunoglobulin heavy-chain (IgH) enhancer (IgHe) is near or in an initiation zone of chromosomal DNA replication, which is preferentially active in B cells (K. Ariizumi, Z. Wang, and P. W. Tucker, Proc. Natl. Acad. Sci. USA 90:3695-3699, 1993). This suggests the existence of a functional relationship between IgHe-mediated transcription and DNA replication. To test this theory, we utilized simian virus 40 (SV40) DNA replication as a model of chromosomal replication. IgHe or its operationally divisible domains (5'-En, core, and 3'-En) were introduced into SV40 minichromosomes (IgHe-SV40). Results of replication assays with IgHe-SV40 replicons indicated that the 5'-En and 3'-En activated or suppressed SV40 DNA replication regardless of the presence of SV40 enhancers or promoters in these replicons. The activity did not reside in IgHe core sequences. The results suggested that the 5'- and 3'-En regulated SV40 replication through direct interaction with the origin, not through suppression at the SV40 enhancer and/or promoter. In an effort to identify elements within the 5'-En motif that contributed to this effect, we found that the E site, but not microE5 and microE2 boxes, upregulated DNA replication. Our results provide another possible regulatory function for the 5'-En and 3'-En domains besides transcriptional suppression of IgHe.
Amplification of RNA by NASBA allows direct detection of viable cells of Ralstonia solanacearum in potato

Aims: The objective of this study was to develop a Nucleic Acid Sequence Based Amplification (NASBA) assay, targeting 16S rRNA sequences, for direct detection of viable cells of Ralstonia solanacearum, the causal organism of bacterial wilt. The presence of intact 16S rRNA is considered to be a useful indicator for viability, as a rapid degradation of this target molecule is found upon cell death. Methods and Results: It was demonstrated by RNase treatment of extracted nucleic acids from R. solanacearum cell suspensions that NASBA exclusively detected RNA and not DNA. The ability of NASBA to assess viability was demonstrated in two sets of experiments. In the first experiment, viable and chlorine‐killed cells of R. solanacearum were added to a potato tuber extract and tested in NASBA and PCR. In NASBA, only extracts spiked with viable cells resulted in a specific signal after Northern blot analysis, whereas in PCR, targeting 16S rDNA sequences, both extracts with viable and killed cells resulted in specific signals. In the second experiment, the survival of R. solanacearum on metal strips was studied using NASBA, PCR‐amplification and dilution plating on the semiselective medium SMSA. A positive correlation was found between NASBA and dilution plating detecting culturable cells, whereas PCR‐amplification resulted in positive reactions also long after cells were dead. The detection level of NASBA for R. solanacearum added to potato tuber extracts was determined at 104 cfu per ml of extract, equivalent to 100 cfu per reaction. With purified RNA a detection level of 104 rRNA molecules was found. This corresponds with less than one bacterial cell, assuming that a metabolically active cell contains ca 105 copies of rRNA. Preliminary experiments demonstrated the potential of NASBA to detect R. solanacearum in naturally infected potato tuber extracts. Conclusions: NASBA specifically amplifies RNA from viable cells of R. solanacearum even present in complex substrates at a level of 100 cfu per reaction. Significance and Impact of the Study: The novel NASBA assay will be particularly valuable for detection of R. solanacearum in ecological studies in which specifically viable cells should be determined.
Measuring thermomechanical displacements of solar cells in laminates using digital image correlation

Solar modules are commercially fabricated for more than 30 years but there is little knowledge about its thermomechanical properties. Solid materials shrink or expand when subjected to temperature changes. The coefficients of thermal expansion (CTE) for silicon and glass are small compared to the CTE of the polymer sheets. This CTE-mismatch leads to mechanical stress and strain which can be critical for the solar cells, the interconnects and the polymers. We demonstrate that the digital image correlation technique (DIC) is capable of measuring displacements in modules that are within a climate chamber by an optical measurement through a transparent back sheet and one layer of encapsulation material. As a first demonstration the change of the gaps between adjacent cells is determined at various temperatures for three test laminates with three different interconnection techniques. We find the gap between two solar cells to deform about 0.4-0.6 ¿m/°C in the temperature range of 0°C to 80°C and slightly less in the temperature region below 0°C. The results are verified by a simplified calculation of the gap deformation.
Conceptual framework for a value-based corporative management in the sustainable development society

For the sustainable development of our society, simultaneous achievement of economic efficiency, environmental protection and social responsibilities in each corporation should be attained. These targets, however, have some strong interdependency. It is difficult to find the optimal solution that satisfies these targets. In this paper, the authors propose a sustainability oriented value chain combining corporate activities with product lifecycles, and define two types of value chain; horizontal value chain and vertical value chain. Based on these value chains and the objective functions, the optimal value chains can be selected as a compromised solution or the Pareto-optimum solution throughout the corporate activities by a proposed multi-objective dynamic programming method.
Synthesis and Preliminary Antimicrobial Evaluation of New 7-(N-pyrrolyl) Derivatives of Cephalosporins

Summary A series of seven new cephalosporins was prepared for preliminary microbiological evaluation by N-acylation of 7-aminoce-phalosporanic acid with substituted N-pyrrolylcarboxylic acids via mixed anhydrides. The chemical structure of the compounds were confirmed by IR, 1H-NMR and mass spectral data. The 7-(N-pyrrolyl) cephalosporin derivatives were tested in vitro by the disc diffusion method upon 3 strains and subsequent determination of the minimal inhibitory concentration (MIC) of the most active ones upon 29 strains. The products of the series exhibited antibacterial activity. They showed selective potency against Gram-positive and were practically inactive against Gram-negative microorganisms. The compound 3-[(acetyloxy)methyl]-7-({2-[3-(ethoxycarbonyl)-2-methyl-5-phenyl-1H-1-pyrrolyl] acetyl} amino) -6-oxo-7,7a-dihydro-2H,6H-aceto[2,1b][1,3]thiazine-4-carboxylic acid (4a) was outlined as more active than the reference cefazolin (CAS 23325-78-2) in regard to S. pyogenes and some strains of S. aureus, the MIC of 4a against S. pyogenes were at least 4-fold lower. The toxicological evaluations of the starting N-pyrrolylcarboxylic acids showed no acute toxicity.
ON TRYING TO DO ORAL HISTORY IN ACCOUNTING RESEARCH

A number of studies have emerged calling for the use of more interpretative methods to be employed in accounting research (Collins and Bloom, 1991; Carnegie and Napier, 1996; Hammond and Sikka, 1996; Llewellyn, 1999). It is claimed that oral history as an interpretative method, has enormous potentialities in the accounting field (Hammond and Sikka, 1996). In particular, it is claimed that it can prioritise the marginalised and those previously silenced by providing a platform for the ‘voice’ to be heard through the generation of rich experiences (Kyriacou, 2000). In recent years, an increasing number of studies utilising this methodology have emerged which have provided access to accounting experiences [McKeen and Richardson (1998); Emery et al., (2002); Hammond and Streeter (1994); Matthews and Pirie (2001); Hammond (2002); and Kim (2004)]. Although, oral history is a valuable method which can enrich accounting research, it is however fraught with difficulties, including those of an ethical and practical nature which can seriously affect the eventual outcome of the research (Kim, 2008). The aim of this paper, in part, seeks to explore some of the issues inherent in oral history research through the findings of a research study conducted into the experiences of minority ethnic women in U.K. accounting (Kyriacou, 2000). Full paper citation: Kyriacou, O. (2009). On trying to do oral history in accounting research, Accountancy Business and the Public Interest, 8(2), pp. 44-64.
Biomimetic dehydrogenative Diels-Alder cycloadditions: total syntheses of brosimones A and B.

Passing (H2) Gas Concise syntheses of the natural products brosimones A and B using sequential dehydrogenative Diels-Alder (DHDA) cycloadditions are reported. The syntheses employ either Pt/C-cyclopentene or DDQ to effect dehydrogenation of prenylchalcone substrates in combination with silver nanoparticles (AgNP’s) to promote subsequent Diels-Alder cycloadditions.