Hereditary complement factor I deficiency

Summary We describe four cases (from three families) of hereditary factor I deficiency, bringing the total number of cases now reported to 23. In one family there are two affected siblings: one has suffered recurrent pyogenic infections; the other is asymptomatic. In the second family, the patient had recurrent pyogenic infections and a self-limiting vasculitic illness; in the third family, the patient suffered recurrent pyogenic and neisserial infections. All four patients had markedly reduced concentrations of C3 in the serum (family 1 propositus: 28%; family 1 asymptomatic sibling: 15%; family 2: 31%; and family 3: 31 % normal human serum) which was in the form of C3b. Low lgG2 levels may occur in primary C3 deficiency, and reduction in lgG2 concentration to 1.14 g/l (normal: 1.30-5.90 g/l) was found in the patient from family 2. Using radioligand binding assays, we demonstrated increased binding of C3b to erythrocytes in a patient with factor I deficiency. This C3b could not be cleaved by autologous serum but could be cleaved by normal serum or purified factor I. We review and compare the published cases of C3, factor H and factor I deficienc

Top Management Team Diversity: A systematic Review

Empirical research investigating the impact of top management team (TMT) diversity on executives’ decision making has produced inconclusive results. To synthesize and aggregate the results on the diversity-performance link, a meta-regression analysis (MRA) is conducted. It integrates more than 200 estimates from 53 empirical studies investigating TMT diversity and its impact on the quality of executives’ decision making as reflected in corporate performance. The analysis contributes to the literature by theoretically discussing and empirically examining the effects of TMT diversity on corporate performance. Our results do not show a link between TMT diversity and performance but provide evidence for publication bias. Thus, the findings raise doubts on the impact of TMT diversity on performance

Economic liberalization and the antecedents of top management teams: evidence from Turkish 'big' business

There has been an increased interest in the last two decades in top management teams (TMTs) of business firms. Much of the research, however, has been US-based and concerned primarily with TMT effects on organizational outcomes. The present study aims to expand this literature by examining the antecedents of top team composition in the context of macro-level economic change in a late-industrializing country. The post-1980 trade and market reforms in Turkey provided the empirical setting. Drawing upon the literatures on TMT and chief executive characteristics together with punctuated equilibrium models of change and institutional theory, the article develops the argument that which firm-level factors affect which attributes of TMT formations varies across the early and late stages of economic liberalization. Results of the empirical investigation of 71 of the largest industrial firms in Turkey broadly supported the hypotheses derived from this premise. In the early stages of economic liberalization the average age and average organizational tenure of TMTs were related to the export orientation of firms, whereas in later stages, firm performance became a major predictor of these team attributes. Educational background characteristics of teams appeared to be under stronger institutional pressures, altering in different ways in the face of macro-level change

The Political De-Determination of Legal Rules and the Contested Meaning of the ‘No Bailout’ Clause

Traditional debates on legal theory have devoted a great deal of attention to the question of the determinacy of legal rules. With the aid of social sciences and linguistics, this article suggests a way out of the ‘determinate-indeterminate’ dichotomy that has dominated the academic debate on the topic so far. Instead, a dynamic approach is proposed, in which rules are deemed to undergo processes of political ‘de-determination’ and ‘re-determination’. To illustrate this, the article uses the example of Art. 125 of the Treaty on the Functioning of the European Union, the ‘no bailout’ provision, which played a major role in the management of the Euro-crisis. As will be shown, with the start of the crisis, this provision, whose meaning was once scarcely controversial, became the object of intense interpretative disagreement. As it became politically relevant, the rule also became the site of interpretative competitions, until the intervention of the European Court of Justice disambiguated and redefined its meaning

Real time optical pressure sensing for tactile detection using gold nanocomposite material

For the first time, we propose in this work a new concept of optical tactile pressure sensing. We develop a sensor integrating an optical tapered fiber force sensor based on electromagnetic (EM) coupling effect. The sensor consists of a tapered multimode Si fiber which couples the EM field coming from a broad band lamp source with the flexible gold/PDMS nanocomposite material (GNM). PDMS polymer film was used since it is suitable for the generation of gold nanoparticles starting from gold precursors and consecutively is suitable for light coupling: the formed gold nanoparticles increase the effective refractive index of the PDMS and support the EM coupling with the tapered region. By applying different weights that can be translated to pressure forces to the sensor, we experimentally observe in real time the intensity reduction of the transmittivity response at the output of the fiber sensor. This effect is most likely due to displacement of gold nanoparticles near the tapered region during the pressure application

Insecticidal Effect of Diatomaceous Earth Against Three Species of Stored-Product Psocids on Maize, Rice, and Wheat

We evaluated the efficacy of three diatomaceous earth (DE) formulations, Dryacide, Protect-It, and Insecto, against three Psocoptera species, Liposcelis entomophila (Enderlein) (Liposcelididae), Lepinotus reticulatus Enderlein (Trogiidae), and Liposcelis decolor (Pearman), in the laboratory. Bioassays were conducted in three grain commodities, wheat, rice and maize, at 30°C and 75% RH, and the DEs were applied at the recommended dose rates of 1,000, 400, and 500 ppm for Dryacide, Protect-It, and Insecto, respectively. Differences in adult mortality were found among grains and DEs for L. entomophila and L. reticulatus, but these trends were not consistent for all combinations tested. Type of grain and DE did not affect L. decolor mortality significantly. Moreover, mortality increased with increasing exposure time for L. entomophila and L. reticulatus, but there was no effect of exposure time on L. decolor. After 7 d of exposure, mortalities of L. entomophila, L. reticulatus, and L. decolor were 56, 55, and 40%, respectively, and the respective mortality levels after 14 d were 63, 71, and 42%. Progeny production after 30 d was significantly suppressed for all species in the treated grains. However, progeny production was still high in the treated grains and reached 54, 42, and 76 individuals/10 g of grain for L. entomophila, L. reticulatus, and L. decolor, respectively. Progeny production did not vary with commodity. Our results suggest that DEs, when used alone, will not provide effective control of psocids

Green composites of poly(3-hydroxybutyrate) containing graphene nanoplatelets with desirable electrical conductivity and oxygen barrier properties

Poly(3-hydroxybutyrate), a green polymer originating from prokaryotic microbes, has been used to prepare composites with graphene nanoplatelets (GnP) at different concentrations. The films were fabricated by drop-casting and were hot-pressed at a temperature lower than their melting point to provide the molecular chains enough energy to reorientate while avoiding melting and degradation. It was found that hot-pressing increases crystallinity and improves mechanical properties. The Young’s modulus increased from 1.2 to 1.6 GPa for the poly(3-hydroxybutyrate) (P(3HB)) films and from 0.5 to 2.2 GPa for the 15 wt % P(3HB)/GnP composites. Electrical resistivity decreases enormously with GnP concentration and hot-pressing, reaching 6 Ω sq–1 for the hot-pressed 30 wt % P(3HB)/GnP composite. Finally, the hot-pressed P(3HB) samples exhibit remarkable oxygen barrier properties, with oxygen permeability reaching 2800 mL μm m–2 day–1, which becomes 895 mL μm m–2 day–1 when 15% GnP is added to the biopolymer matrix, one of the lowest values known for biopolymers and biocomposites. We propose that these biocomposites are used for elastic packaging and electronics

Reversible wettability of hybrid organic/inorganic surfaces of systems upon light irradiation/storage cycles

In this work we present hybrid organic/inorganic structures that can exhibit reversible surface wettability, altered in a controllable manner. In particular, we use the method of photo-patterning to produce polymeric SU-8 pillars of specific geometries, onto which we subsequently deposit colloidal TiO2 nanorods. In this way, we combine the microroughness of the polymeric pillars with the nanoroughness of the nanorod-coating to create highly hydrophobic surfaces. The hydrophobicity of these systems can be changed reversibly into hydrophilicity upon irradiation of the hybrid structures with pulsed UV laser light. This behaviour is due to the well-known property of TiO2, that becomes superhydrophilic upon UV light irradiation. This property is reversible and we monitor the recovery of our hybrid polymeric/inorganic-nanorods structures to their initial hydrophobic character upon dark storage and heating. The wetting behaviour has been modelled and analysed according to the surface geometry. The direct implementation of such structures into microfluidics devices is demonstrated. Copyright © 2010 Inderscience Enterprises Ltd

Patterned structures of in situ size controlled CdS nanocrystals in a polymer matrix under UV irradiation.

A method of in situ formation of patterns of size controlled CdS nanocrystals in a polymer matrix by pulsed UV irradiation is presented. The films consist of Cd thiolate precursors with different carbon chain lengths embedded in TOPAS polymer matrices. Under UV irradiation the precursors are photolyzed, driving to the formation of CdS nanocrystals in the quantum size regime, with size and concentration defined by the number of incident UV pulses, while the host polymer remains macroscopically/microscopically unaffected. The emission of the formed nanocomposite materials strongly depends on the dimensions of the CdS nanocrystals, thus, their growth at the different phases of the irradiation is monitored using spatially resolved photoluminescence by means of a confocal microscope. X-ray diffraction measurements verified the existence of the CdS nanocrystals, and defined their crystal structure for all the studied cases. The results are reinforced by transmission electron microscopy. It is proved that the selection of the precursor determines the efficiency of the procedure, and the quality of the formed nanocrystals. Moreover it is demonstrated that there is the possibility of laser induced formation of well-defined patterns of CdS nanocrystals, opening up new perspectives in the development of nanodevices

Director Characteristics and Firm Performance

The traditional methodology examining optimal boards relates a simple board variable (e.g. independence or board demography) to firm performance, however, ig- noring other board characteristics. This paper investigates how the education and business experience of directors affect firm performance. The sample consists of 1,574 directorships from 224 listed firms in Switzerland. Using OLS and including control variables, the results show that graduates of minor Swiss universities are negatively related to Tobin’s Q, and industrial knowledge and Tobin’s Q are nega- tively correlated if the firm has more divisions. In addition, director fixed effects (or unobserved characteristics) are significant, but improve the explanatory power of the models only by 5 percent