Miracle-dz/UniFAR-FTUs huggingface dataset:
Mutation R120G in alphaB-crystallin, which is linked to a desmin-related myopathy, results in an irregular structure and defective chaperone-like function.

alphaB-crystallin, a member of the small heat shock protein family, possesses chaperone-like function. Recently, it has been shown that a missense mutation in alphaB-crystallin, R120G, is genetically linked to a desmin-related myopathy as well as to cataracts [Vicart, P., Caron, A. , Guicheney, P., Li, A. , Prevost, M. -C. , Faure, A. , Chateau, D. , Chapon, F. , Tome, F. , Dupret, J.-M. , et al. (1998) Nat. Genet. 20, 92-95]. By using alpha-lactalbumin, alcohol dehydrogenase, and insulin as target proteins, in vitro assays indicated that R120G alphaB-crystallin had reduced or completely lost chaperone-like function. The addition of R120G alphaB-crystallin to unfolding alpha-lactalbumin enhanced the kinetics and extent of its aggregation. R120G alphaB-crystallin became entangled with unfolding alpha-lactalbumin and was a major portion of the resulting insoluble pellet. Similarly, incubation of R120G alphaB-crystallin with alcohol dehydrogenase and insulin also resulted in the presence of R120G alphaB-crystallin in the insoluble pellets. Far and near UV CD indicate that R120G alphaB-crystallin has decreased beta-sheet secondary structure and an altered aromatic residue environment compared with wild-type alphaB-crystallin. The apparent molecular mass of R120G alphaB-crystallin, as determined by gel filtration chromatography, is 1.4 MDa, which is more than twice the molecular mass of wild-type alphaB-crystallin (650 kDa). Images obtained from cryoelectron microscopy indicate that R120G alphaB-crystallin possesses an irregular quaternary structure with an absence of a clear central cavity. The results of this study show, through biochemical analysis, that an altered structure and defective chaperone-like function of alphaB-crystallin are associated with a point mutation that leads to a desmin-related myopathy and cataracts.
Assessing Credit Risk of Companies with Mean-Reverting Leverage Ratios

Empirical findings and theoretical studies suggest that firms adjust towards time-varying target leverage ratios. This paper studies the performances of the default probabilities generated from two stationaryleverage models with time-dependent and constant target ratios respectively. The time-dependent model consistently performs better in terms of discriminatory power of differentiating firms' default risk and capability for predicting default rates over the period 1996 to 2006. The model provides appropriate measures of credit risk of firms and evidence to support the existence of a time-varying target leverage ratio.
A Diagnostic Algorithm for the Detection of Clostridium difficile-Associated Diarrhea.

BACKGROUND Clostridium difficile is a common cause of hospital-acquired diarrhea, which is usually associated with previous antibiotic use. The clinical manifestations of C. difficile infection (CDI) may range from mild diarrhea to fulminant colitis. Clostridium difficile should be considered in diarrhea cases with a history of antibiotic use within the last 8 weeks (community-associated CDI) or with a hospital stay of at least 3 days, regardless of the duration of antibiotic use (hospital-acquired CDI). AIMS This study investigated the frequency of CDI in diarrheic patients and evaluated the efficacy of the triple diagnostic algorithm that is proposed here for C. difficile detection. STUDY DESIGN Cross-sectional study. METHODS In this study, we compared three methods currently employed for C. difficile detection using 95 patient stool samples: an enzyme immunoassay (EIA) for toxin A/B (C. diff Toxin A+B; Diagnostic Automation Inc.; Calabasas, CA, USA), an EIA for glutamate dehydrogenase (GDH) (C. DIFF CHEK-60TM, TechLab Inc.; Blacksburg, VA, USA), and a polymerase chain reaction (PCR)-based assay (GeneXpert(®) C. difficile; Cepheid, Sunnyvale, CA, USA) that detects C. difficile toxin genes and conventional methods as well. In this study, 50.5% of the patients were male, 50 patients were outpatients, 32 were from inpatient clinics and 13 patients were from the intensive care unit. RESULTS Of the 95 stool samples tested for GDH, 28 were positive. Six samples were positive by PCR, while nine samples were positive for toxin A/B. The hypervirulent strain NAP-1 and binary toxin was not detected. The rate of occurrence of toxigenic C. difficile was 5.1% in the samples. Cefaclor, ampicillin-sulbactam, ertapenem, and piperacillin-tazobactam were the most commonly used antibiotics by patients preceding the onset of diarrhea. Among the patients who were hospitalized in an intensive care unit for more than 7 days, 83.3% were positive for CDI by PCR screening. If the PCR test is accepted as the reference: C. difficile Toxin A/B ELISA sensitivity and specificity were 67% and 94%, respectively, and GDH sensitivity and specificity were 100% and 75%, respectively. CONCLUSION Tests targeting C. difficile toxins are frequently applied for the purpose of diagnosing CDI in a clinical setting. However, changes in the temperature and reductant composition of the feces may affect toxin stability, potentially yielding false-negative test results. Therefore, employment of a GDH EIA, which has high sensitivity, as a screening test for the detection of toxigenic strains, may prevent false-negative results, and its adoption as part of a multistep diagnostic algorithm may increase accuracy in the diagnosis of CDIs.
Serum hormones, follicular fluid steroids, insulin-like growth factors and their binding proteins, and ovarian IGF mRNA in sheep with different ovulation rates.

Ovulation rate, serum hormone concentrations, follicular fluid (FFL) concentrations of steroids and IGF, IGF binding protein (IGFBP) activity in FFL, and follicular IGF-I and -II mRNA were compared during the follicular phase among five genotypes of ewes: Finn (F), Composite III (C), 1/2 Booroola Merino (B) x 1/2 F (B x F), 1/2 F x 1/2 C (F x C), 1/2 B x 1/2 C (B x C). Composite III ewes were a Columbia x Suffolk x Hampshire crossbred. Ovulation rates for F (n = 7), C (n = 5), B x F (n = 6), F x C (n = 3), and B x C (n = 8) ewes were 3.1, 1.6, 3.8, 2.9, and 2.9 (Pooled SEM = .5), respectively. Concentrations of IGF-I in FFL were 53% greater (P < .05) in large (> or = 4.1 mm) than in small (< 4.1 mm) follicles but did not differ (P > .10) among genotypes. In contrast, FFL IGF-II concentrations were greater (P < .05) in B x C and B x F ewes than in C or F x C ewes but did not differ between small and large follicles. Ligand blotting revealed that IGFBP activity of three species (34, 27 to 29, and 24 kDa) were lower (P < .05) in FFL of large than in FFL of small follicles but did not differ (P < .10) among genotypes. Follicular wall IGF-I mRNA and IGF-II mRNA was detected in 5 and 32% of the samples from preovulatory follicles, respectively, using reverse transcriptase-PCR and ethidiumbromide staining. Ovarian IGF-I mRNA levels, assessed by Northern analysis, in B x F and B x C ewes were greater (P < .05) than those in C ewes; ovarian IGF-I mRNA levels in F and F x C ewes were intermediate and did not differ (P > .10) from those in C ewes. Small follicles from B x C and B x F ewes had severalfold greater (P < .05) estradiol concentrations than those from F or C ewes, whereas large follicles from B x F ewes had twice (P < .05) the estradiol concentrations of follicles from F or C ewes. Progesterone in FFL did not differ among genotypes. Serum LH, FSH, inhibin, IGF-I, and progesterone did not differ (P > .10) among genotypes. (ABSTRACT TRUNCATED AT 400 WORDS)
Efficacy and safety of intracavernosal alprostadil in men with erectile dysfunction. The Alprostadil Study Group.

BACKGROUND Erectile dysfunction is a common medical problem affecting many men. Although several intracavernosal therapies are available, their efficacy and safety have not been studied systematically. METHODS We investigated the efficacy and safety of alprostadil formulated for intracavernosal treatment in three separate multi-institutional, prospective studies in men with erectile dysfunction of vasculogenic, neurogenic, psychogenic, and mixed causes. Clinical and laboratory evaluations of erection and the feasibility of satisfactoriness of sexual activity as assessed both by the men and by their partners were the primary measures of efficacy. RESULTS In a dose-response study of 296 men, all doses of alprostadil were superior to placebo and there was a significant dose-response relation (P < / = 0.001), resulting in higher response rates with increasing doses of alprostadil (from 2.5 to 20 microg). In a dose-finding study of 201 men, the minimal effective dose was < / = 2 microg in 23, 20, 38 and 23 percent of men with erectile dysfunction of neurogenic, vasculogenic, psychogenic, or mixed causes, respectively. In a six-month self-injection study in 683 men, the participants reported being able to have sexual activity after 94 percent of the injections. The men and their partners rated the sexual activity as satisfactory after 87 and 86 percent of the injections, respectively. Penile pain, usually mild, occurred in 50 percent of the men at some time but after only 11 percent of the injections. Prolonged erections occurred in 5 percent of the men, priapism in 1 percent, penile fibrotic complications in 2 percent, and hematoma or ecchymosis in 8 percent. CONCLUSIONS In men with erectile dysfunction, intracavernosal injection of alprostadil is an effective therapy with tolerable side effects.
Making or letting managers manage what it means to manage A longitudinal Investigation of the changing role of central government in the Institutionalization of public management

This paper examines the institutionalization of public management in Denmark over a period of thirty years. Based upon a longitudinal design lens we demonstrate how the central Government played a crucial role in both legitimating and facilitating the institutionalization of public management as a profession and a ready-to-wear concept. In addition we demonstrate how the role of central Government shifted over time from being an agenda setter in the 1980ies and core driver in the 00ies to now becoming a more subtle facilitator. While Government in the wake of the financial crisis obviously is taking a more prominent role in many sectors, this study illustrates another development characteristic in which central government control-efforts are diminishing or taking on a role of the facilitating state. Subtheme 06 – B.W. Rennison, J. A. Nielsen, H. H. Salomonsen – bwr@socsci.aau. dk
High Suppressor of Cytokine Signaling-3 Expression Impairs STAT3-dependent Protective Effects of Interleukin-22 in Ulcerative Colitis in Remission

Background:High SOCS3 expression in intestinal epithelial cells (IECs) of patients with ulcerative colitis (UC) in remission reflects the shorter time to relapse. We investigated whether high SOCS3 increased risk for relapse through violating STAT3-dependent protective effects of interleukin (IL)-22 during UC remission. Methods:Expression of IL-22 and c-Myc in UC remission mucosa was analyzed by immunohistochemistry. Effects of IL-22 on migration and proliferation of IEC cell lines with enforced SOCS3 expression were assessed with wounding assay and CCK-8 assay, respectively. Influence of STAT3 interference and SOCS3 overexpression on IL-22–regulated expression of antimicrobial peptide and proliferation-related molecules, including DMBT1, c-Myc, Survivin, Bcl-2, and Bcl-xL, were performed with quantitative real-time polymerase chain reaction or Western blot. Results:Patients with UC in remission showed significantly more IL-22–positive immune cells, but no difference of epithelial c-Myc levels, in mucosa compared with healthy controls. Overexpression of SOCS3 nearly abolished IL-22–induced activation of STAT3. By inhibiting STAT3 signaling, SOCS3 influenced IL-22–induced expression of DMBT1, c-Myc, Survivin, and Bcl-2 as well as proliferation and migration processes in cultured IEC cell line. Conclusions:SOCS3 overexpression impairs IL-22–mediated epithelial homeostasis and mucosal wound healing, which could be the mechanism for high SOCS3 IEC expression contributed early relapse of mucosal inflammation. Prevention of SOCS3 expression or enhancement of IL-22/STAT3 signaling in IEC seems to be rational therapeutic strategies for UC remission maintenance.
Preaxial polydactyly in an infant with Down’s syndrome

To the Editor: Although Down’s syndrome (DS) is associated with distal limb abnormalities, these are not generally thought to include polydactyly. Here we report a female infant with DS and striking unilateral preaxial polydactyly (PPD). The fourth child of healthy, unrelated Bangladeshi parents, she had no family history of polydactyly or other congenital malformations. Although her mother was aged 45, her parents had declined routine antenatal screening for DS. Following an uncomplicated pregnancy, she was born by normal vaginal delivery at 42 weeks gestation. Birth weight (2.6 kg) and head circumference (29.8 cm) were both below the third percentile. Typical features of DS were noted, including hypotonia, brachycephaly, upslanting palpebral fissures, flat nasal bridge, low-set ears, a single transverse palmar crease in the right hand, bilateral fifth finger clinodactyly and bilateral wide gaps between the first and second toes. Echocardiography showed a restrictive ventricular septal defect. In addition, there was unilateral PPD. An extra biphalangeal thumb, complete with thumbnail, was present in the left hand (Fig. 1). Radiological examination confirmed that the distal and proximal phalanges of the left thumb were duplicated, although only a single first metacarpal was present. The right thumb was clinically and radiologically normal. Cytogenetic analysis showed a 47,XX,+21 karyotype with no evidence of mosaicism. Common distal limb abnormalities in DS include a wide gap between the first and second toes (68%); short, broad hands (64%); short fifth fingers (58%); fifth finger clinodactyly (57%); a single transverse palmar crease (53%); and characteristic dermatoglyphic anomalies (1). Two large-scale surveys, which compared the prevalence of malformations in infants with and without DS, have recently shown that other limb abnormalities are significantly more common in DS, including reduction defects, clubfoot, syndactyly and polydactyly (2, 3). Interestingly, the first study also found that, although there was no increase in the risk of postaxial, central or unspecified polydactyly, infants with DS had a ten-fold higher risk of PPD than infants without DS (2). PPD occurs in several rare, multiple-malformation syndromes, including Greig cephalopolysyndactyly (GCPS) and Townes–Brocks syndrome (TBS). As an isolated abnormality, it is often dominantly inherited and it may manifest as biphalangeal thumb polydactyly (type I), triphalangeal thumb polydactyly (type II), index finger polydactyly (type III) or polysyndactyly (type IV). A condition known as triphalangeal thumb polysyndactyly syndrome, which is similar to PPD type II, maps to 7q36 (4, 5); while PPD type IV may be allelic to GCPS (6), which is caused by haploin-
Optimal power adaption for NAF relaying with channel side information

In this paper, we develop optimal power adaptation schemes by means of power amplification coefficients at the relay for the half-duplex single-relay non-orthogonal amplify-and-forward (NAF) system with channel side information (CSI) available at the relay. In particular, assuming that the relay has full knowledge of the channel gains, optimal power adaptation schemes are established in closed-form using both the mutual information (MI) and pairwise error probability (PEP) criteria with Gaussian inputs at the source. The proposed solutions can be understood as a multi-dimensional (multi-D) distributed water-filling in time and space. Numerical results show that the proposed power amplification methods provide a significant improvement over conventional schemes using either channel distribution information (CDI) or channel inversion (CI). The gain is observed with regard to both the MI with Gaussian inputs and the bit error rate (BER) performance using finite constellations such as quadrature amplitude modulation (QAM).
Exercise self-efficacy intervention in overweight and obese women

This study investigated the effects of a brief tailored intervention on self-efficacy beliefs and exercise energy expenditure in active and inactive overweight and obese women. Participants were randomly assigned to either control (N = 50) or intervention (N = 47) conditions, and their exercise self-efficacy was assessed three times over a 12-week period. Results showed that the intervention increased schedule, physical, exercise-worries efficacy, and energy expenditure in the previously inactive group. The results suggest that self-efficacy interventions are effective at increasing exercise energy expenditure in inactive overweight and obese women.
Key strategies for the successful involvement of customers in the co‐creation of new technology‐based services

Purpose – The aim is to propose a conceptual framework consisting of research propositions concerning the key strategies required for the successful involvement of customers in the co‐creation of new technology‐based services.Design/methodology/approach – The methodology involves a single case study from which data are derived and analyzed using the grounded theory methodology of “constant comparative analysis. ” User‐generated ideas for future mobile phone services are collected from four user involvement projects and analyzed at several workshops attended by senior managers from telecommunications firms.Findings – Seven key strategies are identified as being essential for successful user involvement in new product development. Each strategy is described and illustrated in relation to existing theory and presented as a research proposition.Research limitations/implications – The exploratory nature of the research means that the findings are tentative and need to be confirmed in other settings by other res.
[Antibiotic resistance of Gardnerella vaginalis isolated from cases of bacterial vaginosis].

INTRODUCTION Gardnerella vaginalis is one of the dominant etiological factors related to bacterial vaginosis. Literature offers a growing number of reports revealing there appear Gardnerella vaginalis strains increasingly resistant to metronidazole. OBJECTIVES The aim of this study was to determine the susceptibility of Gardnerella vaginalis strains isolated from women with bacterial vaginosis to metronidazole, clindamycin and amoxicillin/clavulanic acid. MATERIAL AND METHODS The investigation was performed on collection of 67 Gardnerella vaginalis strains isolated from the group of 604 women participating in the study Antibiotic sensitivity of strains was verified by E-test method (BioMerieux). Interpretation of results was performed in accordance with EUCAST criteria. RESULTS All tested strains, apart from one, were sensitive to clindamycin and amoxicillin/clavulanic acid. The results of susceptibility test to metronidazole indicated that 68.7% (46 out of 67 strains) were resistant to this antibiotic, while all of them were sensitive to both clindamycin and amoxicillin/clavulanic acid. CONCLUSIONS Near future may bring the need to change the treatment regimen of bacterial vaginosis.
Obstacles to investments in mobile payments: The perspective of merchants : Work in progress

The main objective of the ongoing research is to investigate obstacles and driving forces affecting merchants’ decision to invest in mobile payment solutions. The core aim of this paper is to present initial results of a desktop analysis revealing the current market situation, identifying main players, and estimating the structure of investment costs. In order to do that available secondary information was analyzed.The conducted analysis served to identify market fragmentation existing in the Swedish market due to existence of a number of competing mobile payment solutions. Another finding allows making conclusion, that retailers select to adopt already existing solutions, rather than developing own. Finally, the size of an organization and financial resources are important when making a decision to invest in a new payment solution.
The Hankel Determinant of Exponential Polynomials: A Very Short Proof and a New Result Concerning Euler Numbers

Me'moires de l'Acade'mie royale des Sciences et Belles-Lettres de Berlin 24 (1770) 251-326; also in cEuvres de Lagrange, vol. 3, Gauthier-Villars, Paris, 1869, pp. 5-73. 8. John Riordan, Combinatorial Identities, John Wiley & Sons, New York, 1968. 9. James Joseph Sylvester, Note on Burman's Law for the inversion of the independent variable, Philosophical Magazine, 4th series 8 (1854) 535-540; also in The Collected Mathematical Papers of James Joseph Sylvester, vol. 2, Cambridge University Press, 1908, pp. 44-49; reprinted by Chelsea Publishing Company, New York, 1973.
Incremental Clomiphene Therapy: A New Method for Treating Persistent Anovulation

Thirty anovulatory patients, previously unresponsive to clomiphene citrate in standard dosage, were treated with a new incremental method of clomiphene therapy. Clomiphene was given continuously with dosage increments every 5 days, up to a maximum dose of 3750 mg, and ovarian response was monitored with urinary estrogen estimation and with ultrasound. Twenty-one patients (70%) ovulated on this regimen and 8 (27%) conceived; 6 patients who ovulated but did not conceive had other infertility factors. Clomiphene response was associated with a progressive rise in follicle-stimulating hormone, luteinizing hormone, and estrogen excretion; these parameters were unchanged in nonresponders. Side effects of treatment were minimal and only 1 case of hyperstimulation occurred. Preliminary testing with estrogen-amplified gonadotropin-releasing hormone did not differentiate responders from nonresponders. Incremental clomiphene treatment appears to be a less complicated alternative for patients who would otherwise require exogenous gonadotropin therapy
WHAT IS A BUSINESS FAMILY

Most businesses are argued to be family businesses, but this group of businesses is very heterogeneous. This is noticed in terms of differences in level of family involvement and influence in family businesses, and the F-PEC scale is a powerful tool when analysing such variations. However, all the families that are involved in these family businesses are also different. These families may be referred to as business families, but how do such families differ from each other? In order to be able to answer that question, one needs to start by defining a business family. During the search for such a definition, one will find that a business family may be positioned on a continuous scale ranging from business influenced families to family business influencing families.
State estimation of nonlinear systems using the Unscented Kalman Filter

This paper addresses the problem of estimating the state of a nonlinear system from measurements that are perturbed by a random source of noise. The Extended Kalman Filter is a type of all-purpose filter that tries to solve this problem by dealing with a linearized version of the system. A new methodology proposed in [1], named Unscented Kalman Filter, is presented. It uses the so-called unscented transformation to better describe the stochastic evolution of the state of the system. The aim of this paper is to compare and discuss the performance of each filter when applied to state estimation of a simplified model of the DELMAC autonomous surface craft.