Miracle-dz/UniFAR-FTUs huggingface dataset:
Congenital Erythropoietic Porphyria: Characterization of Murine Models of the Severe Common (C73R/C73R) and Later-Onset Genotypes

Congenital erythropoietic porphyria (CEP) is an autosomal recessive disorder due to the deficient activity of uroporphyrinogen III synthase (UROS). Knock-in mouse models were generated for the common, hematologically severe human genotype, C73R/C73R, and milder genotypes (C73R/V99L and V99L/V99L). The specific activities of the UROS enzyme in the livers and erythrocytes of these mice averaged approximately 1.2%, 11% and 19% of normal, respectively. C73R/C73R mice that survived fetal life to weaning age (~12%) had a severe microcytic hypochromic anemia (hemoglobin 7.9 g/dL, mean cellular volume 26.6 fL, mean cellular hemoglobin content 27.4 g/dL, red cell distribution width 37.7%, reticulocytes 19%) and massively accumulated isomer I porphyrins (95, 183 and 44 µmol/L in erythrocytes, spleen and liver, respectively), but a nearly normal lifespan. In adult C73R/C73R mice, spleen and liver weights were 8.2- and 1.5-fold increased, respectively C73R/V99L mice were mildly anemic (hemoglobin was 14.0 g/dL and mean cellular hemoglobin was 13.3), with minimally accumulated porphyrins (0.10, 5.54 and 0.58 µmol/L in erythrocytes, spleen and liver, respectively), whereas adult V99L/V99L mice were normal. Of note, even the mildest genotype, V99L/V99L, exhibited porphyria in utero, which disappeared by 2 months of age. These severe and mild mouse models inform therapeutic interventions and permit further investigation of the porphyrin-induced hematopathology, which leads to photo-induced cutaneous lesions. Of significance for therapeutic intervention, these mouse models suggest that only 11% of wild-type activity might be needed to reverse the pathology in CEP patients.
Transplantation of Airway Epithelial Stem/Progenitor Cells: A Future for Cell‐Based Therapy

&NA; Cell therapy has the potential to cure disease through replacement of malfunctioning cells. Although the tissue stem cell (TSC) is thought to be the optimal therapeutic cell, transplantation of TSC/progenitor cell mixtures has saved lives. We previously purified the mouse tracheobronchial epithelial TSCs and reported that in vitro amplification generated numerous TSCs. However, these cultures also contained TSC‐derived progenitor cells and TSC repurification by flow cytometry compromised TSC self‐renewal. These limitations prompted us to determine if a TSC/progenitor cell mixture would repopulate the injured airway epithelium. We developed a cell transplantation protocol and demonstrate that transplanted mouse and human tracheobronchial epithelial TSC/progenitor cell mixtures are 20‐25% of airway epithelial cells, actively contribute to epithelial repair, and persist for at least 43 days. At 2 weeks after transplantation, TSCs/progenitor cells differentiated into the three major epithelial cell types: basal, secretory, and ciliated. We conclude that cell therapy that uses adult tracheobronchial TSCs/progenitor cells is an effective therapeutic option.
The Constitutional Dilemma of Economic Liberty

This paper studies the problem of self-enforcing constitutions, addressing the question, how do some constitutions provide incentives for political officials to abide by the constraints announced in the constitution? To understand the mechanisms underlying successful constitutions, the paper begins by exploring a simple society facing the dilemma of policing the government: a sovereign, who controls the government, and two citizens. It then moves to a discussion of how constitutions are often formed out of crises, with some more detailed discussion of two main examples: England's Glorious Revolution of 1688 and the U.S. Constitution.
The Two Sides of the Story: Network Investments and New Venture Creation

It is widely recognized that networks provide access to resources necessary for founding a new venture. However, they also come along with opportunity costs of time. We therefore argue that maintaining a set of network relationships is an investment that may not always pay off. More specifically, we develop detailed hypotheses on why the relationship between investing time in developing and maintaining a larger network and more intense network relationships and success in new venture creation may be best described by an inverted U. Testing our hypotheses on longitudinal data of 137 nascent entrepreneurs, we find broad support for our propositions.
A Calcium-Dependent Feedback Mechanism Participates in Shaping Single NMDA Miniature EPSCs

NMDA receptors (NMDARs) are highly calcium-permeable and are negatively regulated by intracellular calcium during prolonged exposure to agonist. We have investigated whether calcium-mediated feedback occurs during transient exposure to glutamate during single synaptic events. Examination of miniature EPSCs (mEPSCs) indicated that the decay kinetics of the NMDAR component was markedly slowed by the intracellular perfusion of exogenous calcium buffers (BAPTA or Fluo-3). In contrast, the AMPA receptor component of the miniature EPSC was unaffected. Slow on-rate calcium buffers, such as EGTA, did not alter kinetics of the NMDAR component of the mEPSC. Addition of exogenous fast calcium buffers did not slow the decay kinetics of glutamate-evoked currents mediated by NR1/NR2A heteromers expressed in HEK 293 cells, suggesting that the effect we observed in neurons may be specific to processes associated with synaptically activated receptors. Trial-to-trial amplitude variability of miniature calcium transients mediated by NMDARs increased with the injection of exogenous calcium buffers, suggesting that the amplitude of synaptic calcium transients are maintained at a rather constant level by a calcium-mediated feedback mechanism.
Twelve tips for teaching with ultrasound in the undergraduate curriculum

With ever increasing use of imaging as a diagnostic tool in medicine, medical schools are being urged to further integrate imaging into their curricula. Ultrasound is one such way of doing so-enabling students to bridge the gap between form and function, medical school and clinical practice. As a non-invasive imaging technique, with low risk when compared to other methods of imaging, ultrasound is ideal for integration into basic science and clinical teaching. The twelve tips given in this article offer advice on the practicalities of running a successful ultrasound imaging session in an appropriate environment, promoting safety and curriculum integration.
Changes in employees attitudes at work following an acquisition: a comparative study by acquisition type

The purpose of this paper is to examine how employee reaction varies in the eventof an acquisition, and ultimately, to show that it depends on the acquisition context;speciﬁcally: (1) the legitimacy of the purchasing ﬁrm’s identity, and (2) the extentof the organizational changes or discontinuity resulting from the acquisition. Theresearch hypotheses considered are tested using a single questionnaire administeredrepeatedly over a ﬁve-year period to the employees of 85 sites belonging initially tothree different ﬁrms: ABC (the acquiring ﬁrm), EFG (the ﬁrm taken over in afriendly acquisition) and XYZ (ﬁrm absorbed in a hostile acquisition). The resultsmainly show that employees working at sites belonging initially to EFG have higherorganizational identiﬁcation scores than those at sites belonging initially to XYZ.Insecurity scores increase at all sites after the acquisition period, even for employeeswho originally belonged to ABC. Finally, a temporal link is seen betweenorganizational identiﬁcation, insecurity and job satisfaction.
Financial reporting on the internet in the Middle East: the case of Jordanian industrial companies

In recent years, online disclosure of financial information has become a common practice in developed countries. Developing countries, however, face several obstacles that hinder such dissemination of financial information. These obstacles relate to technology, culture, cost and other factors. This study examines the extent of financial information dissemination on the internet by Jordanian industrial companies listed in the Amman Stock Exchange (ASE). The study also examines the advantages and obstacles of online disclosure from the point of view of investors and financial managers in the industrial sector in Jordan. The findings of this study indicate that 55% of industrial companies have websites and only 30% have utilised these websites to disseminate financial information. The study concludes that the cost of establishing and maintaining websites, in addition to the lack of regulations, has contributed to the infrequent use of online reporting by Jordanian companies.
Optimal operation of a district heating power plant with thermal energy storage

This paper presents an optimal control strategy for a district heating power plant with thermal energy storage. The main goal of the control strategy is to reduce the operation costs of the power plant, by scheduling the boilers, the operation of the thermal energy storage and the curtailment on the loads. The problem is stated as a constrained optimization in the form of a Mixed Integer Linear Program (MILP), embedded on an Model Predictive Control (MPC) framework. Particular attention is paid to modeling of boilers operating constraints, including the outlet water flow temperature, to the energy exchanged with the thermal energy storage and to the operating modes of the power plant layout, including the constraints related to the supply water temperature needed from the network. The results are performed using the data and the layout of the power plant located in the city of Ylivieska, in Finland. The cost analysis performed shows the advantages of using the predictive control strategy.
Reframing the Compositional Capability: A Resource-Based View on ‘A Composition-Based View of Firm Growth’

This commentary targets the core ideas of the composition-based view (CBV). First, we argue that the deployment of compositional capabilities (CCs) to combine ordinary resources fits the resource-based view (RBV) and that there is therefore no need for a CBV. Second, we argue that the CCs should be presented as a specific type of dynamic capability (DC). We show that even where ordinary resources are being combined, superior combinatory capabilities are needed as competitive advantage cannot otherwise be achieved. Third, we argue that Luo and Child (2015) focused too much on the emerging economy enterprises (EEEs) as the conceptual setting. We conclude with a future research agenda to prepare the ground for research on compositional capabilities within the composition-based view of the firm.
The Beta Regime Change Risk Premium

The number of statistically detectable Beta regime changes a portfolio experienced in the past is a natural proxy for ex ante "Beta regime change risk" of the portfolio. This study applies newly developed statistical tests of multiple structural breaks to investigate whether Beta regime change risk is a systematic risk priced in subsequent security returns. Our results show Beta regime change risk is rewarded by higher returns. In addition, we find evidence showing that the explanatory power of both size and BE/ME are subsumed by our proxy for Beta regime change. Portfolios with high Beta regime change risk earn substantially higher than average returns. Additional tests confirm that a risk-based explanation can account for the results.
Human resource management and firm performance in China: A critical review

Strategic human resource management is an emerging field of study in the transitional economy of China. The purpose of this study is to identify the current status of strategic human resource management research in the context of mainland China, and offer recommendations for future research. This study reviewed recently published articles (between 1992 and 2008) in major academic journals in both English and Chinese, focusing on the most important topic of strategic human resource management: the relations between human resource management and firm performance. This study finds that China-based strategic human resource management research has actively participated in major theoretical debates in the literature, and that China-based studies have extended previous models by identifying additional moderating and mediating variables. We recommend future research in this area be more responsive to emerging methodological concerns of the field, and pay more attention to China-specific issues that may have significant organizational consequences.
Pictorial and motion-based depth information during active control of self-motion: size-arrival effects on collision avoidance.

With computer simulations of self-motion, Ss controlled their altitude as they approached a floating object and, after getting as close as possible to the object, tried to "jump" over it without collision. Ss jumped significantly later for small objects, compared with larger objects that were approached from equal distances at equal speeds and were positioned at equal clearance heights. This occurred even when accretion-deletion information was present and when object width and length were varied independently. Results were consistent with studies in which Ss judged a large far approaching object to hit the viewpoint before a small near object that would have arrived sooner (P.R. DeLucia, 1991a, 1991b). Results suggest that pictorial information such as relative size contributes to active collision-avoidance tasks and must be considered in models of perceived distance and time-to-arrival.
Modal age at death: lifespan indicator in the era of longevity extension

This paper examines and demonstrates the importance of the adult modal age at death (M) in longevity research. Unlike life expectancy at birth (e0) and median age at death, M is determined solely by old-age mortality as far as mortality follows a bathtub curve. It represents the location of old-age death heap in the age distribution of deaths, and captures mortality shifts more accurately than conditional life expectancies such as e65. Although M may not be directly determined from erratic mortality data, a recently developed method for deriving M from the P-spline-smoothed mortality curve based on penalised Poisson likelihood is highly effective in estimating M. Patterns of trends and differentials in M can be noticeably different from those in other lifespan measures, as indicated in some examples. In addition, major mathematical models of adult mortality such as the Gompertz, logistic and Weibull models can be reformulated using M, which plays a critical role as the mortality level parameter in those models.
Privatisation and sustainable competitive advantage in the emerging economies of Central Europe

Extant research on the impact of privatization in the Central Europe (CE) region has focused on improvements in efficiency and the nature of cost-based advantages. This study argues that the development of a vibrant privatized sector requires attention to the broader resource configurations of domestic enterprises. Empirical research was conducted on a large sample of firms in Poland, Hungary and Slovenia. Foreign investment was found to significantly impact on resource accumulation with implications for the development of strategic capabilities and competitive advantage. Foreign direct investment is an effective vehicle for the transfer of financial resources, reputation and new brands but not organizational capabilities. In terms of practice, this study demonstrates the important role of outside investment in the development of a firm's resource base (Frydman et al. 1999). Companies can gain a competitive advantage in their domestic markets through gaining access to the resources of foreign investors.
Propensity and comprehensiveness of corporate internet reporting in Egypt: Do board composition and ownership structure matter?

Purpose - The purpose of this study is to examine the impact of corporate governance attributes of listed Egyptian companies on the propensity (adoption) and comprehensiveness (quality) of corporate internet reporting (CIR) practices. Design/methodology/approach - This study uses archival data from the largest (top) 100 listed companies on the Egyptian Stock Exchange (EGX 100). Corporate governance attributes are captured by ownership structure (free float, managerial ownership, government ownership) and board of directors' structure (board size, board independence, CEO-chair duality). Empirical models are used to estimate the effects of these attributes on the propensity, content, presentation, and overall comprehensiveness of CIR. Findings - The results of this study indicate mixed effects of governance attributes on the choice to adopt CIR and its quality. The results from the Binary Logistic Regression suggest that Egyptian companies with greater (less) ownership dispersion, managerial ownership, governmental ownership, and (board independence) are more likely to adopt CIR. On the other hand – and as revealed by the seemingly unrelated regressions – among CIR companies those with greater (less) ownership dispersion, board size (governmental ownership), and (board independence) have more comprehensive CIR. Originality/value - This study extends the relatively limited research on the effects of corporate governance and CIR in emerging markets. The study contributes to this literature by demonstrating how corporate governance attributes affects the choice to adopt CIR disclosure practices and the level of its quality in an emerging market such as Egypt.
Peripheral CD4 T Cells Rapidly Accumulate at the Host:Parasite Interface during an Inflammatory Th2 Memory Response1

Memory peripheral Th2 immune responses to infectious pathogens are not well studied due to the lack of suitable models and the difficulty of assessing Th2 cytokine expression at sites of inflammation. We have examined the localized immune response to a nematode parasite that encysts in the small intestine. An unexpected architecture was observed on day 4 of the memory response, with granulocytes and macrophages infiltrating the cyst and CD4+, TCR-αβ+ T cells surrounding the cyst. Laser capture microdissection analysis showed a pronounced CD4-dependent Th2 cytokine pattern at the cyst region only during the memory response, demonstrating that the Th2 memory response is readily distinguished from the primary response by the rapid accumulation of Th2 effector cells at the host:parasite interface.
An Argument for Knowledge Variety in Evidence-Based Management

Author(s): Martelli, Peter Fabrizio | Advisor(s): Shortell, Stephen M | Abstract: Evidence-based management has been proposed as a method to impose rationality on decision-making, and thereby link research more closely with practice. Yet, knowledge, not evidence per se, is the means by which organizations map the uncertainty of their environments and develop strategies accordingly. This essay develops a theoretical argument to support the construct of knowledge variety, and argues that knowledge variety is a preferable operationalization of evidence-based management for organizations facing the epistemic uncertainty of complex environments. The first section sets a foundation drawing upon diversity in socio-behavioral regulation, requisite variety, the social structure of knowledge, epistemic knowledge categories, rhetorical persuasion, sensemaking, and organizational attention. The second section describes a mixed-methods project to examine evidence-based management in terms of knowledge variety across 42 hospitals associated with a common knowledge intermediary. An original proposition intending to establish a relationship between the maintenance of variety and an organization's ability to customize diffusing best practices was unable to confirm or disconfirm a relationship. Nevertheless, a series of qualitative and quantitative findings on evidence-based management and best practices are presented, including a relationship between knowledge variety and the number of years since last academic degree, suggesting the importance of revisiting the Aristotelian notion of practical wisdom (or, phronesis). Reflections, limitations, and suggestions for future research are described throughout.
Clinical evaluation of endoscopic ultrasonography-guided drainage using a novel flared-type biflanged metal stent for pancreatic fluid collection

Background and Objectives: Endoscopic ultrasonography (EUS)-guided transluminal drainage for pancreatic fluid collections (PFCs) has become the standard therapy worldwide as a minimally invasive therapy compared with surgical drainage. Recently, a novel flared-type biflanged metal stent (BFMS) designed specifically for the treatment of PFCs has been developed. The aim of this study was to retrospectively assess the feasibility and safety of EUS-guided drainage and direct endoscopic necrosectomy (DEN) for PFCs using the novel flared-type BFMS. Patients and Methods: Twenty-one patients were treated by EUS-guided drainage using a flared-type BFMS for PFCs (pancreatic pseudocyst, 2 patients; walled-off necrosis, 19 patients). Results: The present study showed a technical success rate of 100%, a final clinical success rate of 100%, a procedure-related adverse event (AE) rate of 0%, an early AE rate of 28.6% (moderate and severe AE rate of 9.5%), a mortality rate of 0%, and a recurrence rate of 9.5%. DEN (mean, 2.3 sessions) was required in 38% of the patients. Conclusion: The present study clarified that the EUS-guided drainage using the flared-type BFMS is an effective and safe treatment approach for PFCs. Further studies using randomized controlled multicenter trials are warranted.
Essays on Economics of Network Industries: Mobile Telephony

This thesis addresses theoretical and empirical questions related to network economics. In particular, it analyze network effects and switching costs in the mobile telecommunications industry. The first chapter analyzes competition in a two-period differentiated-products duopoly in the presence of both switching costs and network effects. They are shown to have opposite implications on the demand side, specially in the first period. While the former reduces demand elasticities, the latter increases them. Increases in marginal network benefits imply lower prices in both periods while the effects of switching costs are ambiguous. However, when network effects are strong, and switching costs are moderate, prices in both periods may be lower than those in a market without network effects and switching costs. This property may be exploited to increase aggregate consumer surplus by instituting a switching tax. The second essay analyzes demand for mobile telecommunications services in Germany in the period from January 1998 to June 2003. During this time, subscriber base grew exponentially by about 700% while prices declined only moderately by about 41%. The estimation results indicate that network effects played a significant role in the diffusion of mobile services in Germany. The third chapter employs multinominal and mixed logit on panel data of British households between the years 1999-2001 to estimate the magnitude of switching costs in mobile telephony. According to the estimation results, consumers of mobile services in the UK face significant switching costs which vary in the magnitude across network operators. The choices of network operators are also explained by observed and unobserved heterogeneity in consumers' tastes. The final essay analyzes the impact of regulation on the development and competitiveness of mobile telecommunications industry across the European Union. Using cross-country panel data, a reduced-form and a structural model are estimated. The estimation results indicate that prices are significantly influenced by the regulatory policy.