69 resultados para ULTRASTRUCTURAL-CHANGES

em Consorci de Serveis Universitaris de Catalunya (CSUC), Spain


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Guanylate cyclase activating proteins are EF-hand containing proteins that confer calcium sensitivity to retinal guanylate cyclase at the outer segment discs of photoreceptor cells. By making the rate of cGMP synthesis dependent on the free intracellular calcium levels set by illumination, GCAPs play a fundamental role in the recovery of the light response and light adaptation. The main isoforms GCAP1 and GCAP2 also localize to the synaptic terminal, where their function is not known. Based on the reported interaction of GCAP2 with Ribeye, the major component of synaptic ribbons, it was proposed that GCAP2 could mediate the synaptic ribbon dynamic changes that happen in response to light. We here present a thorough ultrastructural analysis of rod synaptic terminals in loss-of-function (GCAP1/GCAP2 double knockout) and gain-of-function (transgenic overexpression) mouse models of GCAP2. Rod synaptic ribbons in GCAPs−/− mice did not differ from wildtype ribbons when mice were raised in constant darkness, indicating that GCAPs are not required for ribbon early assembly or maturation. Transgenic overexpression of GCAP2 in rods led to a shortening of synaptic ribbons, and to a higher than normal percentage of club-shaped and spherical ribbon morphologies. Restoration of GCAP2 expression in the GCAPs−/− background (GCAP2 expression in the absence of endogenous GCAP1) had the striking result of shortening ribbon length to a much higher degree than overexpression of GCAP2 in the wildtype background, as well as reducing the thickness of the outer plexiform layer without affecting the number of rod photoreceptor cells. These results indicate that preservation of the GCAP1 to GCAP2 relative levels is relevant for maintaining the integrity of the synaptic terminal. Our demonstration of GCAP2 immunolocalization at synaptic ribbons at the ultrastructural level would support a role of GCAPs at mediating the effect of light on morphological remodeling changes of synaptic ribbons.

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Background: Trichothiodistrophy (TTD) is a rare autosomal recessive condition that is characterized by a specific congenital hair shaft dysplasia caused by deficiency of sulfur associated with a wide spectrum of multisystem abnormalities. In this article, we study clinical, microscopic, and ultrastructural findings of 20 patients with TTD with the aim to add further insights regarding to this rare condition. Additionally, analyses of our results are compared with those extracted from the literature in order to enhance its comprehensibility. Materials and Methods: Twenty cases of TTD were included: 7 from Mexico and 14 from Spain. Clinical, microscopic, scanning electron microscopy (SEM) studies and X-ray microanalysis (XrMa) were carried out in all of them. Genetic studies were performed in all seven Mexican cases. Patients with xeroderma pigmentosum and xeroderma pigmentosum/TTD-complex were excluded. Results: Cuticular changes and longitudinal crests of the hair shaft were demonstrated. These crests were irregular, disorganized, following the hair longest axis. Hair shaft sulfur deficiency was disposed discontinuously and intermittently rather than uniformly. This severe decrease of sulfur contents was located close to the trichoschisis areas. Only five patients did not show related disturbances. Micro-dolichocephaly was observed in five cases and represented the most frequent facial dysmorphism found. It is also remarkable that all patients with urologic malformations also combined diverse neurologic disorders. Moreover, three Mexican sisters demonstrated the coexistence of scarce pubic vellus hair, developmental delay, onychodystrophy, and maxillar/mandibullar hypoplasia. Conclusions: TTD phenotype has greatly varied from very subtle forms to severe alterations such as neurologic abnormalities, blindness, lamellar ichthyosis and gonadal malformations. Herein, a multisystem study should be performed mandatorily in patients diagnosed with TTD.

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Background: Trichothiodistrophy (TTD) is a rare autosomal recessive condition that is characterized by a specific congenital hair shaft dysplasia caused by deficiency of sulfur associated with a wide spectrum of multisystem abnormalities. In this article, we study clinical, microscopic, and ultrastructural findings of 20 patients with TTD with the aim to add further insights regarding to this rare condition. Additionally, analyses of our results are compared with those extracted from the literature in order to enhance its comprehensibility. Materials and Methods: Twenty cases of TTD were included: 7 from Mexico and 14 from Spain. Clinical, microscopic, scanning electron microscopy (SEM) studies and X-ray microanalysis (XrMa) were carried out in all of them. Genetic studies were performed in all seven Mexican cases. Patients with xeroderma pigmentosum and xeroderma pigmentosum/TTD-complex were excluded. Results: Cuticular changes and longitudinal crests of the hair shaft were demonstrated. These crests were irregular, disorganized, following the hair longest axis. Hair shaft sulfur deficiency was disposed discontinuously and intermittently rather than uniformly. This severe decrease of sulfur contents was located close to the trichoschisis areas. Only five patients did not show related disturbances. Micro-dolichocephaly was observed in five cases and represented the most frequent facial dysmorphism found. It is also remarkable that all patients with urologic malformations also combined diverse neurologic disorders. Moreover, three Mexican sisters demonstrated the coexistence of scarce pubic vellus hair, developmental delay, onychodystrophy, and maxillar/mandibullar hypoplasia. Conclusions: TTD phenotype has greatly varied from very subtle forms to severe alterations such as neurologic abnormalities, blindness, lamellar ichthyosis and gonadal malformations. Herein, a multisystem study should be performed mandatorily in patients diagnosed with TTD.

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The focus of this thesis is the evolution of programmatic polarization in the post-authoritarian Chilean party system at the elite level. It shows the distance/proximity between parties located along the left-right ideological continuum on three sets of issues. The paper demonstrates that important changes have taken place in the meaning of the right and, especially, left poles. This implies convergence on socio-economic issues between parties, but persistence of differences on religious-value issues, and on issues related to the authoritarian/democratic cleavage. Distance between the poles has been reduced, and as a result the center has lost its own political space. In addition, the paper shows that the pattern followed by programmatic polarization at the elite level is explained by the authoritarian experience, the institutional framework, and socio-economic transformations. Together with this factors, the degree of negotiability of the issues and the cross-cutting nature of the cleavages have also shaped polarization.

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The plant cell wall is a strong fibrillar network that gives each cell its stable shape. It is constituted by a network of cellulose microfibrils embedded in a matrix of polysaccharides, such as xyloglucans. To enlarge, cells selectively loosen this network. Moreover, there is a pectin-rich intercellular material, the middle lamella, cementing together the walls of adjacent plant cells. Xyloglucan endotransglucosylase/hydrolases (XTHs) are a group of enzymes involved in the reorganisation of the cellulose-xyloglucan framework by catalysing cleavage and re-ligation of the xyloglucan chains in the plant cell wall, and are considered cell wall loosening agents. In the laboratory, it has been isolated and characterised a XTH gene, ZmXTH1, from an elongation root cDNA library of maize. To address the cellular function of ZmXTH1, transgenic Arabidopsis thaliana plants over-expressing ZmXTH1 (under the control of the CaMV35S promoter) were generated. The aim of the work performed was therefore the characterisation of these transgenic plants at the ultrastructural level, by transmission electron microscopy (TEM).The detailed cellular phenotype of transgenic plants was investigated by comparing ultra-thin transverse sections of basal stem of 5-weeks old plants of wild type (Col 0) and 35S-ZmXTH1 Arabidopsis plants. Transgenic plants show modifications in the cell walls, particularly a thicker middle lamella layer with respect the wild type plants, supporting the idea that the overexpression of ZmXTH1 could imply a pronounced wall-loosening. In sum, the work carried out reinforces the idea that ZmXTH1 is involved in the cell wall loosening process in maize.  

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En este trabajo analizaremos en primer lugar la presencia i la importancia de los alimentos que caracterizan la dieta mediterránea en los sistemas alimentarios españoles del siglo XIX. Veremos como evoluciona esta presencia y el su peso a la dieta dentro del proceso de transición nutricional moderna española, contemplando dos períodos: desde finales del XIX hasta la década de 1930, y la segunda mitad del XX, períodos caracterizados por un aumento de la ingesta de alimentos, cereales y patatas primer, y de productos de origen animal después, y por la progresiva convergencia con las dietes de les sociedades desarrolladas, que diluirían o minimizarían progresivamente las características diferenciales mediterráneos. Situaremos los cambios que se producen a España en el contexto europeo, i finalmente, analizaremos en que medida, en la etapa inicial, la dieta seguida se ajustaba a les necesidades de la población y a la dieta actualmente recomendada, y si se produjeron cambios significativos en el curso de la transición alimentaria.

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The 1998 Spanish reform of the Personal Income Tax eliminated the 15% deduction for private medical expenditures including payments on private health insurance (PHI) policies. To avoid an undesirable increase in the demand for publicly funded health care, tax incentives to buy PHI were not completely removed but basically shifted from individual to group employer-paid policies. In a unique fiscal experiment, at the same time that the tax relief for individually purchased policies was abolished, the government provided for tax allowances on policies taken out through employment. Using a bivariate probit model on data from National Health Surveys, we estimate the impact of said reform on the demand for PHI and the changes occurred within it. Our findings suggest that the total probability of buying PHI was not significantly affected. Indeed, the fall in the demand for individual policies (by 10% between 1997 and 2001) was offset by an increase in the demand for group employer-paid ones, so that the overall size of the market remained virtually unchanged. We also briefly discuss the welfare effects on the state budget, the industry and society at large.

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The contributions of this paper are twofold: On the one hand, the paper analyses the factors determining the growth in car ownership in Spain over the last two decades, and, on the other, the paper provides empirical evidence for a controversial methodological issue. From a methodological point of view, the paper compares the two alternative decision mechanisms used for modelling car ownership: ordered-response versus unordered-response mechanisms. A discrete choice model is estimated at three points in time: 1980, 1990 and 2000. The study concludes that on the basis of forecasting performance, the multinomial logit model and the ordered probit model are almost undistinguishable. As for the empirical results, it can be emphasised that income elasticity is not constant and declines as car ownership increases. Besides, households living in rural areas are less sensitive than those living in urban areas. Car ownership is also sensitive to the quality of public transport for those living in the largest cities. The results also confirmed the existence of a generation effect, which will vanish around the year 2020, a weak life-cycle effect, and a positive effect of employment on the number of cars per household. Finally, the change in the estimated coefficients over time reflects an increase in mobility needs and, consequently, an increase in car ownership.

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This document presents an integrated analysis of the performance of Catalonia based on an analysis of how the energy consumption (measured at the societal level for the Catalan Society) is used within both the productive sectors of the economy and the household, to generate added value, jobs, and to guarantee a given level of material standard of living to the population. The trends found in Catalonia are compared to the trends of other European Countries to contextualize the performance of Catalonia with respect to other societies that have followed different paths of economic development. The first part of the document consists of the Multi-Scale Integrated Analysis of Societal and Ecosystem Metabolism (MuSIASEM) approach that has been used to provide this integrated analysis of Catalan Society across different scales (starting from an analysis of the specific sectors of the Catalan economy as an Autonomous Community and scaling up to an intra-regional (European Union 14) comparison) and across different dimensions of analyses of energy consumption coupled with added value generation. Within the scope of this study, we observe the various trajectories of changes in the metabolic pattern for Catalonia and the EU14 countries in the Paid Work Sectors composed of namely, the Agricultural Sector, the Productive Sector and the Services and Government Sector also in comparison with the changes in the household sector. The flow intensities of the exosomatic energy and the added value generated for each specific sector are defined per hour of human activity, thus characterized as exosomatic energy (MJ/hour) (or Exosomatic Metabolic Rate) and added value (€/hour) (Economic Labour Productivity) across multiple levels. Within the second part of the document, the possible usage of the MuSIASEM approach to land use analyses (using a multi-level matrix of categories of land use) has been conducted.

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El Delta del Ebro se halla situado en el Mediterráneo, en NE de la Península Ibérica (sur de Cataluña). Su formación ha sido resultado de la interacción del mar y del Ebro, un río que con sus 910 km de longitud drena una cuenca de unos 85.362 km2. Sus características dependen de los diferentes regímenes climáticos de su extensa cuenca, lo que explica la complejidad de su régimen. A lo largo del periodo estudiado el Delta fue siempre una realidad geológica aunque experimentó importantes cambios en su morfología. En consecuencia, sus recursos naturales siempre pudieron ser explotados, aunque estas actividades se hubieron de adaptar a su cambiante morfología y a sus peculiares condiciones naturales, diferentes de las zonas terrestres más inmediatas.

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In this study, we analyse the degree of polarisation-a concept fundamentally different from that of inequality-in the international distribution of CO2 emissions per capita in the European Union. It is analytically relevant to examine the degree of instability inherent to a distribution and, in the analysed case, the likelihood that the distribution and its evolution will increase or decrease the chances of reaching an agreement. Two approaches were used to measure polarisation: the endogenous approach, in which countries are grouped according to their similarity in terms of emissions, and the exogenous approach, in which countries are grouped geographically. Our findings indicate a clear decrease in polarisation since the mid-1990s, which can essentially be explained by the fact that the different groups of countries have converged (i.e. antagonism among the CO2 emitters has decreased) as the contribution of energy intensity to between-group differences has decreased. This lower degree of polarisation in CO2 distribution suggests a situation more conducive to the possibility of reaching EU-wide agreements on the mitigation of CO2 emissions.

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Ontogenetic changes in digestive capabilities were analyzed in larvae and first juveniles of the spider crab Maja brachydactyla. Activities of five proteinases (total proteases, trypsin, chymotrypsin, pepsin-like and aminopeptidase), three carbohydrases (amylase, maltase and chitinase), an esterase and an alkaline phosphatase were studied to evaluate digestive enzyme profiles of the species. Both quantitative (spectrophotometry and fluorometry) and qualitative (SDS-PAGE) approaches were used. All assayed enzymes were active from hatching (zoea I-ZI) throughout larval development and in first juveniles. Significant variations during ontogeny were found only in total activities likely as a consequence of digestive system development. Specific activity varied little over ontogeny, being significant only for chitinase. Total proteases, trypsin and pepsin-like activities showed a similar pattern of increase as larval ontogeny advanced, decreasing significantly in juveniles. Chymotrypsin continued to increase, showing maximum activity after metamorphosis. Proteinase zymograms confirmed strong proteolytic activity in first zoeas, with increasing bands over the course of ontogeny, decreasing after metamorphosis. A group of bands with high molecular mass was specific to larval stages. Amylase and maltase showed a parallel pattern of continuous increase of total activity as development advanced. Gel-SDS-PAGE showed unchanged patterns of amylase activity in first zoeas of different ages and the most complex set of bands during larval ontogeny in second zoea. Esterase total activity increased significantly as ZI's aged likely reflecting introduction of a lipid-enriched diet. The importance of lipid accumulation at the beginning of ontogeny was also confirmed by the protease/esterase and amylase/esterase activity ratios, which decreased from hatch to late ZI and might be explained as an adaptation, ensuring the next molt. The results suggest that larvae of M. brachydactyla are capable of digesting a variety of dietary substrates as soon as they hatch.

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This paper examines the impact of local human capital on individuals’ wages through external effects. Employing wage regressions, it is found that changes in individuals’ wages are positively associated with changes in the shares of high-paid occupation workers in the British travel-to-work-areas for the late 1990s. I examine this positive association for different occupational groups (defined by pay) in order to disentangle between production function and consumer demand driven theoretical explanations. The wage effect is found to be stronger and significant for the bottom-paid occupational quintile compared to the middle-paid ones, and using also sectoral controls the paper argues to provide evidence for the existence of consumer demand effects.

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This paper conducts an empirical analysis of the relationship between wage inequality, employment structure, and returns to education in urban areas of Mexico during the past two decades (1987-2008). Applying Melly’s (2005) quantile regression based decomposition, we find that changes in wage inequality have been driven mainly by variations in educational wage premia. Additionally, we find that changes in employment structure, including occupation and firm size, have played a vital role. This evidence seems to suggest that the changes in wage inequality in urban Mexico cannot be interpreted in terms of a skill-biased change, but rather they are the result of an increasing demand for skills during that period.