Miracle-dz/UniFAR-FTUs huggingface dataset:
The electrodiagnostic characteristics of Glycogen Storage Disease Type III

Purpose: Glycogen Storage Disease Type III, also known as debrancher deficiency or Cori disease, is an autosomal recessive disorder recognized for both its hepatic and muscle manifestations. The neuromuscular manifestations of Glycogen Storage Disease Type III are not well characterized. In this study, we attempt to better define the disorder.Methods: The medical records of 40 patients with Glycogen Storage Disease Type III seen at Duke University during 1990–2009 were reviewed. The medical records of all patients with nerve conduction studies and/or electromyography were examined.Results: Twelve patients with Glycogen Storage Disease Type III (aged 5–55 years) had undergone nerve conduction studies ± electromyography. Three of these cases are presented in detail. Nine patients had Glycogen Storage Disease Type IIIa, two patients had Glycogen Storage Disease Type IIIb, and the clinical subtype of one patient was unknown. All had nerve conduction studies and of those nerves tested, abnormalities in the median motor response were most common, corresponding to previously described, intrinsic hand muscle weakness. Electromyography was performed in eight patients and myopathic findings were present in six individuals. Abnormal electrodiagnostic findings were more common in older patients. The two patients with Glycogen Storage Disease Type IIIb had electrodiagnostic evidence of nerve involvement with minor myopathic findings.Conclusions: The neuromuscular manifestations of Glycogen Storage Disease Type III include myopathy and neuropathy and are more likely to occur with increasing age, even in those diagnosed with Glycogen Storage Disease Type IIIb. Intrinsic hand muscle weakness is likely due to a combination of nerve and muscle dysfunction, a finding that may have implications for treatment.
Tourism destination zoning in mountain regions: a consumer-based approach

This paper puts into question the conventional way of delineating tourism destination borders in terms of taken-for-granted administrative boundaries. Despite the fact that the literature on destination boundaries advocates for conceptual frameworks where customers’ consumption patterns play a more fundamental role, instances of actual attempts of structuring tourism geographies into ‘new tourism areas’ are scant, and instances of zoning on the basis of visitors’ consumption patterns are absent. A method for identifying alternative and more effective consumption-based tourism zones that combines geographical information system and hierarchical cluster analysis techniques, and that relies on time distances between attractions, is thus proposed, and implemented in the case of the Pyrenees mountain region. As a result the region is restructured into nine new tourism zones, which, compared to the original destinations, are more uniform in size and have a higher correlation index between attractiveness and accommodation intensity; they also have different levels of cross-border intensity and are very similar to historical regions; and the more they differ from the original destinations the higher their attractiveness, which supports the effectiveness of the new zoning technique. Four types of tourism zones ranging from higher to lower tourism intensity are also identified.
The Complementary Effects of Market Orientation and Entrepreneurial Orientation on Profitability in Small Businesses**

Market orientation (MO) and entrepreneurial orientation (EO) are correlated, but distinct constructs. MO reflects the degree to which firms' strategic market planning is driven by customer and competitor intelligence. Entrepreneurial orientation reflects the degree to which firms' growth objectives are driven by the identification and exploitation of untapped market opportunities. When modeled separately, research has reported direct effects of both constructs on firm profitability. When modeled simultaneously, however, the direct effect of EO has disappeared. This has led some scholars to postulate that EO is an antecedent of MO. The results of this study contradict this presumption and suggest that EO and MO complement one another, at least in small businesses, to boost profitability. The major difference between this and previous studies is the inclusion of innovation success, which captures an indirect effect of EO on profitability. At least in small firms, the results suggest that EO complements MO by instilling an opportunistic culture that impacts the quality and quantity of firms' innovations.
Using Local Cuisines when Promoting Small Caribbean Island Destinations

ABSTRACT This study compares how four Caribbean small islands—Aruba, the Dominican Republic, Jamaica, and Martinique—use their authentic cuisines to promote their destinations. Brochures, catalogs, websites, and other promotional materials for each destination were content analyzed. Although all four destinations seem to use their authentic cuisines for tourism promotion, key differences exist among these islands in their marketing and promotional strategies. Martinique appears to use its local cuisine most aggressively, using a combination of locally prepared foods, cocktails, rum, fruits, and vegetables to visually portray this aspect of the country's heritage. Jamaica, in contrast, uses mainly fruit and vegetable imagery. The study findings suggest a need for these four Caribbean island destinations to develop expertise in culinary tourism, followed by promotion through brochures, catalogs, websites, and other marketing materials.
The Sarbanes-Oxley Act and Cross-Listed Foreign Private Issuers

I examine the short- and long-term impact of the 2002 Sarbanes–Oxley Act (SOX) on cross-listed foreign private issuers. Both short- and long-term test results suggest that the costs of SOX compliance significantly exceed its benefits and reduce the net benefits of cross-listings.
Aging-Related Increased Expression of Inducible Nitric Oxide Synthase and Cytotoxicity Markers in Rat Hypothalamic Regions Associated with Male Reproductive Function

We have previously demonstrated that the inducible nitric oxide synthase (iNOS) protein and total NOS activity increase in the hypothalamus and other regions of the male rat brain during aging. We have now tested the hypothesis that increased iNOS results in excessive nitric oxide (NO) and peroxynitrite production, and leads to increased apoptosis in CNS cells, including the GnRH and oxytocin hypothalamic neurons involved in the control of male reproductive function. Young (3-month-old) and old (24-month-old) male Brown Norway rats (n = 6) were perfused with 4% formalin. Adjacent coronal paraffin-embedded sections (5 µm) of preoptic area (POA), supraoptic nucleus (SON), paraventricular nucleus (PVN), and arcuate nucleus (ARC) of the hypothalamus were immunostained with antibodies for iNOS, neuronal NOS (nNOS), and nitrotyrosine (a marker of peroxynitrite formation). The intensity of immunostaining was measured using a densitometric image analysis system. Apoptosis was determined by the TUNEL assay. Double immunofluorescence staining with confocal laser scanning microscopy was used for co-localization studies. A significant increase in the iNOS immunostaining measured as optical density (OD) was found in the old compared to the young animals (SON: 0.32 ± 0.02 vs. 0.23 ± 0.03, p < 0.05; PVN: 0.34 ± 0.03 vs. 0.07 ± 0.05, p < 0.001; POA: 0.18 ± 0.02 vs. 0.01 ± 0.02, p < 0.001). Aging did not affect nNOS expression. Nitrotyrosine was elevated in the hypothalamic regions of old compared to young rats (SON: 0.32 ± 0.05 vs. 0.10 ± 0.04, p < 0.05; PVN: 0.32 ± 0.04 vs. 0.13 ± 0.03, p < 0.01; POA: 0.72 ± 0.06 vs. 0.03 ± 0.003, p < 0.001). Increased nitrotyrosine was accompanied by an elevation of the apoptotic index in the old rats (SON: 11.01 ± 3.33 vs. 0.57 ± 0.50, p < 0.001; PVN: 3.08 ± 1.12 vs. 0.42 ± 0.32; POA: 6.60 ± 1.93 vs. 0.18 ± 0.17, p < 0.01; ARC: 0.001 ± 0.0001 vs. 4.33 ± 2.33). iNOS staining co-localized with GnRH and oxytocin staining. In conclusion: The aging-related iNOS increased expression in the hypothalamus of the male rat affects regions known to control the synthesis and release of GnRH (POA, ARC) and oxytocin (PVN, SON), and the factors regulating penile erection (POA, and PVN). These observations suggest that iNOS may play a role in the reduction in GnRH and oxytocin neuronal secretion resulting in reproductive dysfunctions such as lowered serum testosterone, hypospermatogenesis, and diminished copulatory function in the aging male animal.
Theses on Nonprofit Organizational Effectiveness

This article draws from the general literature on organizational effectiveness and the specialized literature on nonprofit organizational effectiveness to advance six theses about the effectiveness of public benefit charitable nonprofit organizations (NPOs). (a) Non-profit organizational effectiveness is always a matter of comparison. (b) Nonprofit organizational effectiveness is multidimensional and will never be reducible to a single measure. (c) Boards of directors make a difference in the effectiveness of NPOs, but how they do this is not clear. (d) More effective NPOs are more likely to use correct management practices. (e) Nonprofit organizational effectiveness is a social construction. (f) Program outcome indicators as measures of NPO effectiveness are limited and can be dangerous. The article concludes by considering three possible futures for NPO effectiveness research.
Knowledge Management as the Basis of Sustained High Performance

By deconstructing some of the key elements of the resource-based view and the knowledge-based view of the firm, we suggest that there are three components to knowledge management systems that influence firm performance: the firm's ability to produce new knowledge, its ability to build on that knowledge, and its effectiveness in capturing a high proportion of the subsequent spin-offs. Using regression analysis to analyse data from 30,022 patent records from 42 firms, we find that a firm's growth rate is positively associated with its ability to generate rare and valuable knowledge, and to build on that knowledge. Copyright Blackwell Publishing Ltd 2007.
The TIPS-treasury bond puzzle

We show that the price of a Treasury bond and an inflation-swapped Treasury Inflation-Protected Securities (TIPS) issue exactly replicating the cash flows of the Treasury bond can differ by more than $20 per $100 notional. Treasury bonds are almost always overvalued relative to TIPS. Total TIPS-Treasury mispricing has exceeded $56 billion, representing nearly 8% of the total amount of TIPS outstanding. We find direct evidence that the mispricing narrows as additional capital flows into the markets. This provides strong support for the slow-moving-capital explanation of arbitrage persistence.
How to improve the estimation of power curves for wind turbines

We introduce a dynamical approach for the determination of power curves for wind turbines and compare it with two common methods—among them the standard procedure due to IEC 61400-12-1, i.e. the international standard prepared and published by the International Electrotechnical Commission. The main idea of the new method is to separate the dynamics of a wind turbine’s power output into a deterministic and a stochastic part, corresponding to the actual behaviour of the wind turbine and external influences such as the turbulence of the wind, respectively. In particular, the governing coefficients are reconstructed from the data, and the power characteristic is extracted as the stationary states of the deterministic behaviour. Our results prove that a dynamical approach enables one to grasp the actual conversion dynamics of a wind turbine and to gain most accurate results for the power curve, independent of site-specific influences.
Responsibility for and performance of corporate real estate functions

Purpose – The purpose of this paper is to identify different organizational models concerning both the functions and responsibilities assigned to corporate real estate (CRE) professionals in European and North American companies, as well as to determine the factors that influence the occurrence of these different management models.Design/methodology/approach – An empirical survey between 74 major European and 38 North American companies from the banking, energy, telecommunication, and transportation and logistics industries is conducted.Findings – Five typical models describing the allocation of responsibility of real estate functions within a company and the performance of those responsibilities are identified. Only weak statistical associations are found between these models and certain contextual factors that may influence the choice for a specific model, as well as between the models and certain achievements in CRE. From this, the paper infers that there does not exist one “best practice” CRE manageme.
Health‐related quality of life (HRQOL) among pediatric leukemia patients in Malaysia

Health‐Related Quality of Life (HRQOL) in pediatric leukemia patients in Malaysia has not been studied before. This was mainly due to a lack of databases on patients in the past. Many patients abandoned treatment or were lost to follow up. With more children now fully compliant and completing treatment nowadays, with higher cure rate, HRQOL has become important for our patients. The purpose of the current study was to determine the HRQOL scores in children with acute leukemia and to compare the scores for those on maintenance chemotherapy with those off‐treatment as well as to determine factors which might affect HRQOL.
THE CONTAGIOUS POWER OF INNOVATIVENESS: HOW DIFFERENT CORPORATE PARTNERS CONTRIBUTE TO A FIRM'S KNOWLEDGE

As innovation is often created in networks, a company's business partners influence its innovative outcome. Although many studies investigate innovation networks as a means to explore and exploit external knowledge, virtually no research has focused on the characteristics of external partners — in particular their innovativeness. This study considers axial and lateral corporate partners and investigates the differential impact of the partners' innovativeness on a company's knowledge stocks (market, technological, organisational, process, and product knowledge). Drawing on the literature on inter-organisational networks and using survey data from the manufacturing industry, the results extend theory by revealing that not only relationships with partners but also their properties — partner innovativeness — are important for a company's knowledge stock. In essence our results suggest that the innovativeness of a firm's axial and lateral partners positively yet diversely influences its various knowledge stocks.
The contribution of vowel coarticulation and prosodic weakening in initial and final fricatives to sound change

This paper is concerned with the influence of vocalic context (/ɪ, ʊ/) and prosodic weakening on the production of the German fricatives /s, ʃ/ in two syllable positions and its relation to the frequent sound change from alveolar to post-alveolar fricatives. Previous studies reported coarticulatory influences of vowel context on fricatives and more coarticulation in prosodically weak positions. However, the influence of syllable position is unclear, even though sound change affects more often segments in coda than in onset position. In order to test these factors, acoustic and articulatory data from six German speakers were analysed. They produced lexical words with fricatives in onsets and codas of stressed syllables in accented and deaccented words. The results show a small influence of syllable position on the analysed acoustic and articulatory measurements, with higher spectral centers of gravity and more retracted mean tongue trajectories in coda position.
Can the Consumption Capital Asset Pricing Model Account for Traders' Expected Currency Returns?

This study uses survey data on traders' exchange rate forecasts to test whether their expected excess returns are related to the covariance between the exchange rate and consumption; as predicted by the consumption capital asset pricing model (CCAPM). The covariance is measured through the novel use of rolling windows of the realized covariance (both forward and backward looking) and testing is conducted with the cointegrated VAR. The model is able to account for expected returns with more plausible degrees of risk aversion, but only when using sufficiently long, backward-looking measures of the covariance. This suggests that market participants assess risk, in part, based upon the pro-cyclicality of returns, and infer it from experience in the recent past. There is also evidence that inclusion of the real exchange rate improves the plausibility of the estimates and the model fit.
Entrepreneurship and Competitive Strategy: An Integrative Approach

The two fields of strategic management and entrepreneurship have been viewed as inseparable twins and the relationship has been coined strategic entrepreneurship by a number of scholars. Though the integrative studies of the two areas continue, there seems to be a dearth in the study of the two areas at business (competitive) strategy level. This is a conceptual article which aims to show how business strategy can be integrated with entrepreneurship to enhance firm competitiveness. Whether one argues that strategy subsumes entrepreneurship or that entrepreneurship subsumes strategy, an apparent intersection exists. The integrated strategy is termed entrepreneurial competitive strategy and the article presents a framework for this integration. As with any other literature based model, this model can still be improved and needs to be tested for practical application.
Social sustainability in healthcare facilities: a rating tool for analysing and improving social aspects in environments of care.

INTRODUCTION Nowadays several rating systems exist for the evaluation of the sustainability of buildings, but often their focus is limited to environmental and efficiency aspects. Hospitals are complex constructions in which many variables affect hospital processes. Therefore, a research group has developed a tool for the evaluation of sustainability in healthcare facilities. METHODOLOGY The paper analyses social sustainability issues through a tool which evaluates users' perception from a the quality and well-being perspective. It presents a hierarchical structure composed of a criteria and indicators system which is organised through a weighing system calculated by using the Analytic Network Process. RESULTS AND DISCUSSION The output is the definition of a tool which evaluates how Humanisation, Comfort and Distribution criteria can affect the social sustainability of a building. CONCLUSION Starting from its application, it is evident that the instrument enables the improvement of healthcare facilities through several design and organisational suggestions for achieving healing and sustainable architectures.
In Situ Proteomic Analysis of Human Breast Cancer Epithelial Cells Using Laser Capture Microdissection: Annotation by Protein Set Enrichment Analysis and Gene Ontology*

Identification of molecular signatures that allow detection of the transition from normal breast epithelial cells to malignant invasive cells is a critical component in the development of diagnostic, therapeutic, and preventative strategies for human breast cancer. Substantial efforts have been devoted to deciphering breast cancer etiology at the genome level, but only a limited number of studies have appeared at the proteome level. In this work, we compared individual in situ proteome profiles of nonpatient matched nine noncancerous, normal breast epithelial (NBE) samples with nine estrogen receptor (ER)-positive (luminal subtype), invasive malignant breast epithelial (MBE) samples by combining laser capture microdissection (LCM) and quantitative shotgun proteomics. A total of 12,970 unique peptides were identified from the 18 samples, and 1623 proteins were selected for quantitative analysis using spectral index (SpI) as a measure of protein abundance. A total of 298 proteins were differentially expressed between NBE and MBE at 95% confidence level, and this differential expression correlated well with immunohistochemistry (IHC) results reported in the Human Protein Atlas (HPA) database. To assess pathway level patterns in the observed expression changes, we developed protein set enrichment analysis (PSEA), a modification of a well-known approach in gene expression analysis, Gene Set Enrichment Analysis (GSEA). Unlike single gene-based functional term enrichment analyses that only examines pathway overrepresentation of proteins above a given significance threshold, PSEA applies a weighted running sum statistic to the entire expression data to discover significantly enriched protein groups. Application of PSEA to the expression data in this study revealed not only well-known ER-dependent and cellular morphology-dependent protein abundance changes, but also significant alterations of downstream targets for multiple transcription factors (TFs), suggesting a role for specific gene regulatory pathways in breast tumorigenesis. A parallel GOMiner analysis revealed both confirmatory and complementary data to PSEA. The combination of the two annotation approaches yielded extensive biological feature mapping for in depth analysis of the quantitative proteomic data.
Unexplored aspects of bureaucratic autonomy: a state of the field and ways forward

This article first provides a selective overview of the literature on bureaucratic autonomy and identifies different approaches to this topic. The second section discusses three major sets of open questions, which will be tackled in the contributions to this special issue: the subjective, dynamic and relational nature of autonomy; the complex linkages between tasks, organizational forms, and national path dependencies on the one hand and autonomy and performance on the other hand; and the interplay between autonomy, accountability and democratic legitimacy.
Phage T4 DNA [N 6 -Adenine] Methyltransferase: Kinetic Studies Using Oligonucleotides Containing Native or Modified Recognition Sites

The DNA-[N6-adenine] methyltransferase of T4 phage (T4 Dam MTase) catalyzes methyl group transfer from S-adenosyl-L-methionine (AdoMet) to the N6-position of adenine in the palindromic sequence, GATC. We have investigated the effect of eliminating different structural components of the recognition site on the ability of a substrate to be bound and methylated by T4 Dam. For this purpose, steady state binding (by gel shift assays) and kinetic parameters of methylation (using the methyl donor, [3H-CH3]-AdoMet, at 25 degrees C) were studied using various synthetic duplex oligonucleotides containing some defect in the DNA-target site; e.g., the absence of an internucleotide phosphate or a nucleotide(s) within the recognition site, or a single stranded region. The salient results are summarized as follows: (1) Addition of T4 Dam to a complete reaction mixture (with a 20-mer duplex as substrate) resulted in a 'burst' of 3H-methylated product, followed by a constant rate of product formation that reflected establishment of steady-state conditions. This suggests that the rate-limiting step is release of product methylated DNA from the enzyme [and not the transfer of the methyl group]. (2) A number of the defects in duplex structure had only a weak influence on the binding and Km values, but strongly reduced the kcat. At the same time, several poorly bound duplexes retained good substrate characteristics, especially duplexes having uninterrupted GAT-sequences in both strands. Whereas having only one half of the recognition site element intact was sufficient for stable complex formation, the catalytic turnover process had a strict requirement for an uninterrupted GAT-sequence on both strands. (3) There was no correlation between Km and binding capability; the apparent Kd for some duplexes was 5-70 times higher than Km. This indicates that the T4 Dam methylation reaction can not be explained by a simple Michaelian scheme.