Miracle-dz/UniFAR-FTUs huggingface dataset:
Skin globotriaosylceramide 3 deposits are specific to Fabry disease with classical mutations and associated with small fibre neuropathy

Background Fabry Disease (FD) is characterized by globotriaosylceramide-3 (Gb3) accumulation in several tissues and a small fibre neuropathy (SFN), however the underlying mechanisms are poorly known. This study aimed to: 1) ascertain the presence of Gb3 deposits in skin samples, by an immunofluorescence method collected from FD patients with classical GLA mutations or late-onset FD variants or GLA polymorphisms; 2) correlate skin GB3 deposits with skin innervation. Methods we studied 52 genetically-defined FD patients (32 with classical GLA mutations and 20 with late-onset variants or GLA polymorphisms), 15 patients with SFN associated with a specific cause and 22 healthy controls. Subjects underwent skin biopsy to evaluate Gb3 deposits and epi-dermal innervation. Results Skin Gb3 deposits were found in all FD patients with classical GLA mutations but never in FD patients with late-onset variants or GLA polymorphisms or in patients with SFN and healthy controls. Abnormal deposits were found inside different skin structures but never inside axons. FD patients with GB3 deposits showed lower skin innervation than FD patients with late-onset variants or polymorphisms. Conclusions 1) Skin Gb3 deposits are specific to FD patients with classical GLA mutations; 2) Gb3 deposits were associated with lower skin innervation but they were not found inside axons, suggesting an indirect damage on peripheral small fibre innervation.
Supervisor Support, Work-Family Conflict, and Satisfaction Outcomes: An Empirical Study in the Hotel Industry

ABSTRACT The current study developed and tested a model that investigated the relationship of supervisor support with work-family conflict and family-work conflict and the effects of both directions of conflict with family satisfaction, career satisfaction, and life satisfaction. The model also examined the impact of supervisor support on family and career satisfaction outcomes and the effects of these satisfaction variables on life satisfaction. The study hypotheses were tested based on data obtained from frontline employees in the Turkish hotel industry. According to the results of the path analysis, supervisor support lessened both work-family and family-work conflicts. As predicted, family-work conflict diminished family, career, and life satisfaction outcomes. Unexpectedly, work-family conflict was found to trigger career and life satisfaction. Results reveal that supervisor support increased both family and career satisfaction. The study findings also showed that family satisfaction was significantly and positively related to life satisfaction. The study findings, however, did not lend any support to the relationship between work-family conflict and family satisfaction and the effect of career satisfaction on life satisfaction. Implications for business practice and future research directions are discussed in the study.
Platonic and Archimedean geometries in multicomponent elastic membranes

Large crystalline molecular shells, such as some viruses and fullerenes, buckle spontaneously into icosahedra. Meanwhile multicomponent microscopic shells buckle into various polyhedra, as observed in many organelles. Although elastic theory explains one-component icosahedral faceting, the possibility of buckling into other polyhedra has not been explored. We show here that irregular and regular polyhedra, including some Archimedean and Platonic polyhedra, arise spontaneously in elastic shells formed by more than one component. By formulating a generalized elastic model for inhomogeneous shells, we demonstrate that coassembled shells with two elastic components buckle into polyhedra such as dodecahedra, octahedra, tetrahedra, and hosohedra shells via a mechanism that explains many observations, predicts a new family of polyhedral shells, and provides the principles for designing microcontainers with specific shapes and symmetries for numerous applications in materials and life sciences.
Techniques and outcomes of total upper and lower eyelid reconstruction.

OBJECTIVES To describe techniques used for reconstruction of the eyelids following total loss of the upper and lower eyelids and to describe visual and functional outcomes. METHODS Multicenter, retrospective, interventional case series of all of the patients requiring unilateral reconstruction of both the upper and lower eyelids. RESULTS Six cases were identified, 1 following trauma and 5 following tumor excision (4 with basal cell carcinoma and 1 with melanoma). The median age was 69 years (range, 18-90 years). Primary repair using preserved tissue was carried out in the case of traumatic avulsion. Following tumor excision, bilamellar repair was performed using composite grafts for the posterior lamella and skin-muscle flaps for the anterior lamella. Graft necrosis occurred in 3 cases (50%). In all of the cases, the reconstructed eyelids were stiff and immobile. Lagophthalmos (6 cases [100%]), ptosis (3 cases [50%]), lower eyelid retraction (3 cases [50%]), and ectropion (2 cases [33%]) were common. Useful vision was retained in all of the cases. CONCLUSIONS Total eyelid defects are rare and often unanticipated. Adequate corneal protection can be achieved using lamellar repair principles and local tissues; however, poor vascularity demands careful planning, with vascularized flaps favored over free grafts. Reconstructed eyelids have poor function in the setting of total upper and lower eyelid loss, and revision surgery is often required to improve eyelid structure and function.
Empirical study of a venture capital relationship

Purpose – The paper aims to examine five assumptions of agency theory: that both investor and investee make rational decisions; future outcomes are predictable; both act in their own best interests; the investee has an information advantage over the investor; and the investee is work‐ and risk‐averse.Design/methodology/approach – An agency framework is used to analyse the relationship between a venture capital investment house and one of its investees. Empirical evidence is provided on the nature of this relationship. Additional evidence of the post‐investment performance of both parties is provided, to examine whether the contractual arrangements are conducive to good performance.Findings – There is general support for the assumptions of agency theory, and the framework is found to provide a useful basis for analysing the relationship between a venture capital investor and investee.Research limitations/implications – The paper uses case studies of one investor and one investee, and so is limited by data,.
The effect of intralesional injection of bone marrow derived mesenchymal stem cells and bone marrow supernatant on collagen fibril size in a surgical model of equine superficial digital flexor tendonitis.

REASONS FOR PERFORMING STUDY Collagen fibril size is decreased in repair tissue following tendon injury compared to normal tendon matrix in horses. Mesenchymal stem cells have been suggested to promote regeneration of tendon matrix rather than fibrotic repair following injury, although this concept remains unproven. OBJECTIVES To explore the hypothesis that implantation of autologous mesenchymal stem cells derived from bone marrow into a surgically created central core defect in the superficial digital flexor tendon (SDFT) of horses would induce the formation of a matrix with greater ultrastructural similarities to tendon matrix than the fibrotic scar tissue formed in control defects. METHODS Tissue was collected 16 weeks after induction of injury and 12 weeks after treatment from normal and injured regions of control and treated limbs of 6 horses and examined using transmission electron microscopy. Collagen fibril diameters were measured manually with image analysis software and surface areas calculated. Three parameters assessed for normal and injured tissue were mass average diameter (MAD), collagen fibril index (CFI) and the area dependent diameter (ADD). RESULTS Normal regions from both treated and control limbs displayed higher MAD and CFI values, as well as a characteristic bimodal distribution in fibril size. Injured regions from both treated and control limbs displayed significantly lower MAD and CFI values, as well as a unimodal distribution in fibril size. There were no significant differences between treated and control limbs for any of the parameters assessed. CONCLUSIONS Intralesional injection of autologous bone marrow derived mesenchymal stem cells had no measurable effect on the fibril diameter of collagen in healing tissue in the SDFT of this experimental model 16 weeks after injury. POTENTIAL RELEVANCE Favouring matrix regeneration over fibrotic repair may not be the mechanism by which autologous mesenchymal stem cells assist healing of tendon injury.
Copper Ions Stimulate Polyphosphate Degradation and Phosphate Efflux in Acidithiobacillus ferrooxidans

ABSTRACT For some bacteria and algae, it has been proposed that inorganic polyphosphates and transport of metal-phosphate complexes could participate in heavy metal tolerance. To test for this possibility in Acidithiobacillus ferrooxidans, a microorganism with a high level of resistance to heavy metals, the polyphosphate levels were determined when the bacterium was grown in or shifted to the presence of a high copper concentration (100 mM). Under these conditions, cells showed a rapid decrease in polyphosphate levels with a concomitant increase in exopolyphosphatase activity and a stimulation of phosphate efflux. Copper in the range of 1 to 2 μM greatly stimulated exopolyphosphatase activity in cell extracts from A. ferrooxidans. The same was seen to a lesser extent with cadmium and zinc. Bioinformatic analysis of the available A. ferrooxidans ATCC 23270 genomic sequence did not show a putative pit gene for phosphate efflux but rather an open reading frame similar in primary and secondary structure to that of the Saccharomyces cerevisiae phosphate transporter that is functional at acidic pH (Pho84). Our results support a model for metal detoxification in which heavy metals stimulate polyphosphate hydrolysis and the metal-phosphate complexes formed are transported out of the cell as part of a possibly functional heavy metal tolerance mechanism in A. ferrooxidans.
The data measurement process for UK GNP: stochastic trends, long memory, and unit roots

Much published data is subject to a process of revision due, for example, to additional source data, which generates multiple vintages of data on the same generic variable, a process termed the data measurement process or DMP. This article is concerned with several interrelated aspects of the DMP for UK Gross National Product. Relevant questions include the following. Is the DMP well behaved in the sense of providing a single stochastic trend in the vector time series of vintages? Is one of the vintages of data, for example the "final", the sole vintage generating the long-memory component? Does the multivariate framework proposed here add to the debate on the existence of a unit root in GNP? The likely implicit assumptions of users (that the DMP is well behaved and the final vintage is "best") can be cast in terms of testable hypotheses; and we show that these "standard" assumptions have not always been empirically founded. Copyright © 2002 by John Wiley & Sons, Ltd.
Flexography‐Printed In2O3 Semiconductor Layers for High‐Mobility Thin‐Film Transistors on Flexible Plastic Substrate

Industrially scalable and roll-to-roll-compatible fabrication methods are utilized to fabricate high-mobility (≈8 cm(2) V(-1) s(-1) ) nanocrystalline In2 O3 thin-film transistors (TFTs) on an flexible plastic substrate. Flexographic printing of multiple thin In2 O3 semiconductor layers from precursor-solution is performed on a Al2 O3 gate dielectric obtained via atomic layer deposition. A low-temperature post-contact-annealing step allows control of the TFT device turn-on voltage to ≈0 V for enhancement-mode operation.
The role of management strategies in business performance: men and women entrepreneurs managing creativity and innovation

The aim of this study is to determine the relationships between management strategies and business performance among male and female entrepreneurs. Focusing on Creativity and Innovation (CI and that the C&I management strategies used by men and women entrepreneurs have a stronger impact on business performance than gender. C&I management relates to how the entrepreneur navigates processes relating to valuable internal assets, and how business performance is assessed by changes – improvement or deterioration – in some performance characteristic over time. Findings based on the responses of 233 entrepreneurs revealed that men and women used most managerial strategies similarly, with the exception of one strategy associated with encouraging people involved in the business to utilise C&I. Furthermore, gender per se emerged as insignificant in affecting business performance, while C&I management strategies had a significant impact on business performance. The interaction effects of gender and certain C&I management strategies revealed how men and women emphasise these strategies to advance business performance. Academic and practical implications are discussed.
State estimates of Medicaid expenditures attributable to cigarette smoking, fiscal year 1993.

OBJECTIVE To develop estimates of state Medicaid expenditures attributable to smoking for fiscal year 1993. METHODS The smoking-attributable fractions (SAFs) of state Medicaid expenditures were estimated using a national model that describes the relationship between smoking and medical expenditures, controlling for a variety of sociodemographic, economic, and behavioral factors. RESULTS In fiscal year 1993, the SAF for all states (all types of expenditures) was 14.4%, with a range from 8.6% in Washington DC to 19.2% in Nevada. On average, SAFs ranged from a low of 7.9% for home health services expenditures to 21.7% for hospital expenditures. An estimated total of $12.9 billion of fiscal year 1993 Medicaid expenditures was attributable to smoking. The relative error of this estimate was 40.3%. CONCLUSIONS Cigarette smoking accounts for a substantial portion of annual state Medicaid expenditures, with considerable variation among states. The range in expenditures among the states is due to differences in smoking prevalence, health status, other socioeconomic variables used in the model, and the level and scope of the Medicaid program.
Linear Regression Estimation of Discrete Choice Models with Nonparametric Distributions of Random Coefficients

Random coefficient discrete choice models are a popular method for estimating demand in differentiated product markets. We introduce a computationally simple estimator that uses linear regression to estimate the distribution of random coefficients. The estimator is nonparametric for the distribution of the random coefficients. We compare our estimator to several alternatives in a Monte Carlo exercise, and find the estimator predicts out-of-sample market shares well. We discuss extensions to panel data and dynamic programming. ∗Bajari: Department of Economics, University of Minnesota, Twin Cities and NBER, 1035 Heller Hall, 271 19th Ave South, Minneapolis, MN 55455, email: bajari@econ.umn. edu; Fox: Department of Economics, University of Chicago, 1126 E. 59th St., Chicago, IL 60637, email: fox@uchicago.edu; Ryan: Massachusetts Institute of Technology and NBER, 50 Memorial Drive, E52-262C, Cambridge, MA 02142, email: sryan@mit.edu.
Syndecan-1 regulates FGF8b responses in S115 mammary carcinoma cells

In murine mammary carcinoma cells Shionogi 115 (S115) testosterone induces phenotypical transformation which is largely due to expression of fibroblast growth factor (FGF) 8b. Concomitantly, the expression of the cell surface heparan sulfate proteoglycan syndecan-1 is down-regulated. However, if syndecan-1 expression is maintained by transfection with a testosterone-driven syndecan-1 construct, transformation does not occur. Here we have investigated how the down-regulation of syndecan-1 expression in testosterone-treated S115 cells and the high level of expression in syndecan-1 transfected cells influence the cellular responses toward FGF8b. Our results show that high level of syndecan-1 is associated with a decreased magnitude and duration of the FGF8b induced Erk phosphorylation. This effect was observed regardless whether the cells were stimulated directly with exogenous FGF8b or with testosterone to induce autocrine FGF8b production. Moreover, syndecan-1 transfected cells did not respond to FGF8b stimulation by increase in the intracellular free calcium, whereas untransfected cells displayed a rapid (10 s) induction. These data suggest that, in S115 cells, syndecan-1 acts as a modulator of FGF8b signaling that can limit cellular responses to FGF receptor activation. The decreased levels of syndecan-1 expression and upregulation of the FGF signaling system seen in many cancers may contribute to the proliferation of the malignant cells in vivo.
Interaction of Legionella pneumophila with Acanthamoeba castellanii: uptake by coiling phagocytosis and inhibition of phagosome-lysosome fusion

Legionella pneumophila is a facultative intracellular parasite able to survive within both human monocytes and amoebae. We have demonstrated that processing of L. pneumophila by the free-living amoeba Acanthamoeba castellanii shows many similarities to the processing of L. pneumophila by monocytes. These similarities include uptake of L. pneumophila by coiling phagocytosis and the subsequent confinement of L. pneumophila in a ribosome-studded phagosome. In addition, as in monocytes, inhibition of lysosomal fusion with phagosomes containing L. pneumophila was detected in amoebae. With all clinical isolates, inhibition of phagosomes-lysosome fusion correlated with virulence. However, with one of the environmental isolates tested, no significant difference in phagosome-lysosome fusion was observed between the virulent and avirulent forms. These results indicate that the avirulent form of this isolate differed from the virulent form in some other respect critical to intracellular survival. Therefore, intracellular multiplication of L. pneumophila within A. castellanii may not be solely dependent upon the inhibition of lysosomal fusion.
Foreign Direct Investment in Central Europe Since 1990: An Econometric Study

It is widely recognised that foreign direct investment (FDI) may have an important role to play in the transformation of the formerly centrally planned economies of Central and Eastern Europe. FDI provides a vital source of investment for modernising the industrial structure of these countries and for improving the quality and reliability of infrastructure. In addition new investments may also bring badly needed skills and technologies into the host economy. Evidence from joint ventures in Hungary (Lane, 1994) shows that such firms had a higher propensity to trade and invest than purely indigenous firms. Total FDI inflows into Hungary between 1991–93 were equivalent to 25 per cent of total fixed domestic capital formation (UINDTCI, 1995).
Remittance Responses to Temporary Discounts: A Field Experiment Among Central American Migrants

We study the impacts on remittances of offering migrants temporary discounts on remittance transaction fees. We randomly assigned migrants from El Salvador and Guatemala 10-week remittance transaction fee discounts, and assess impacts using administrative transaction data and a post-experiment survey. Temporary discounts lead to substantial increases in the number of transactions and total amount remitted during the discount period. Surprisingly, these increases persist up to 20 weeks after expiration of the discount. We find no evidence that the discounts cause migrants to shift remittances from other remittance channels, or to send remittances on behalf of other migrants. These findings are consistent with naivete on the part of migrants regarding remittance recipients' reference-dependent preferences.
Disillusionment in Cohabiting and Married Couples

Using a national sample of married (N = 752) and cohabiting (N = 323) couples, we examined the association between disillusionment and self-perceived breakup likelihood. Because disillusionment had not previously been studied in cohabiting couples, its extent and consequences for them were not known. We found considerable disillusionment in cohabiters, their mean level exceeding that of married couples. Based on a conceptual model of relationship change, we tested further whether disillusionment would predict self-perceived breakup likelihood, controlling for relationship satisfaction, commitment, and length. Furthermore, based on assumptions about barriers to leaving different types of relationships, we examined whether disillusionment’s association with breakup likelihood would be stronger in cohabiting than married couples. Results supported disillusionment’s ability to predict perceived breakup likelihood, even with rigorous controls, and the greater strength of this association in cohabiters. In addition, we found a significantly positive partner effect: Male partners’ disillusionment predicted female partners’ breakup likelihood.
Downregulation of the Petunia hybrida α-Expansin Gene PhEXP1 Reduces the Amount of Crystalline Cellulose in Cell Walls and Leads to Phenotypic Changes in Petal Limbs

The expansins comprise a family of proteins that appear to be involved in the disruption of the noncovalent bonds between cellulose microfibrils and cross-linking glycans, thereby promoting wall creep. To understand better the expansion process in Petunia hybrida (petunia) flowers, we isolated a cDNA corresponding to the PhEXP1 α-expansin gene of P. hybrida. Evaluation of the tissue specificity and temporal expression pattern demonstrated that PhEXP1 is preferentially expressed in petal limbs during development. To determine the function of PhEXP1, we used a transgenic antisense approach, which was found to cause a decrease in petal limb size, a reduction in the epidermal cell area, and alterations in cell wall morphology and composition. The diminished cell wall thickness accompanied by a reduction in crystalline cellulose indicates that the activity of PhEXP1 is associated with cellulose metabolism. Our results suggest that expansins play a role in the assembly of the cell wall by affecting either cellulose synthesis or deposition.