607 research outputs found

    Pelizaeus-Merzbacher-Like disease presentation of MCT8 mutated male subjects.

    No full text
    Pelizaeus-Merzbacher Disease is an X-linked hypomyelinatiing leukodystrophy. We report mutations in the thyroid hormone transporter gene MCT8 in 11% of 53 families affected by hypomyelinating leukodystrophies of unknown aetiology. The 12 MCT8 mutated patients express initially a Pelizaeus-Merzbacher-Like disease phenotype with a latter unusual improvement of magnetic resonance imaging white matter signal despite absence of clinical progression. This observation underlines the interest of determining both free T3 and free T4 serum concentrations to screen for MCT8 mutations in young patients (<3 y) with a severe Pelizaeus-Merzbacher-Like disease presentation or older severe mentally retarded male patients with "hypomyelinated" regions

    Valores econômicos de características da raça Nelore em ciclo completo de produção no Pantanal.

    Get PDF
    A produção de gado de corte no Pantanal é conhecida por suas particularidades, tanto em questão de dificuldades de logística, como em singularidades de seu clima, com época de águas e seca bem definidas que resultam em diferenças nos índices zootécnicos dos rebanhos. Atualmente, os programas de melhoramento têm utilizado índices empíricos de seleção, atribuindo pesos às diferentes características de acordo com sua importância dentro sistema produtivo. A seleção dos animais por índices bioeconômicos, que consideram despesas e receitas, é interessante pois o progresso genético de várias características, objetivos de seleção, ocorreria de forma simultânea, incrementando a produtividade e lucratividade dos sistemas. O objetivo deste estudo foi calcular valores econômicos para objetivos de seleção da raça Nelore em um sistema de produção de ciclo completo à pasto no Pantanal.Título do artigo em inglês: Economic values for traits of the Nelore cattle in the Pantanal biome

    Selection indexes for nellore production system in the Brazilian Pantanal.

    Get PDF
    The objective was to develop selection indexes for Nellore cattle raised in full-cycle production system in the Brazilian Pantanal. The resulting offspring are retained as replacements or sold at two years of age. Preliminary analyses explored effects of scale on economic values (EV). However, given the available data, these effects were very small. Presented herein are results from a simulated system consisting of 5,000 cows with all animals maintained on pasture as is typical in Pantanal. The EV were determined by approximating the partial derivatives of the profit function, changing one trait at a time, by one unit, while keeping the other traits constant. Traits in the breeding objective were mature cow weight, direct and maternal weaning weight, postweaning average daily gain, subcutaneous fat depth, longissimus muscle area, and stayability. Economic values were calculated on the basis of number of animals (per head), number of animal units, and arroba of carcasss weight. Regardless of the basis, maternal weaning weight and subcutaneous fat depth made negligible contributions to the breeding objective. Proportions of variation in the breeding objectives (per head, per animal unit, per arroba) explained by cow weight, direct weaning weight, postweaning average daily gain, stayability, and longissimus muscle area were: 13, 13, 17; 6, 1, 5; 3, 3, 4; 67, 67, 61; and 11, 17, 13, respectively. These indexes may aid Nellore breeders in their selection decisions, thus facilitating the genetic progress and increased productivity and profitability of Pantanal herds.Animal production systems and agribusiness

    Taxing the Informal Economy: The Current State of Knowledge and Agendas for Future Research

    Get PDF
    This paper reviews the literature on taxation of the informal economy, taking stock of key debates and drawing attention to recent innovations. Conventionally, the debate on whether to tax has frequently focused on the limited revenue potential, high cost of collection, and potentially adverse impact on small firms. Recent arguments have increasingly emphasised the more indirect benefits of informal taxation in relation to economic growth, broader tax compliance, and governance. More research is needed, we argue, into the relevant costs and benefits for all, including quasi-voluntary compliance, political and administrative incentives for reform, and citizen-state bargaining over taxation

    Spatial variability of vegetation index and soil properties in an integrated crop-livestock system

    Get PDF
    The knowledge of soil property spatial variability is useful for determining the rational use of inputs, such as the site-specific application of lime and fertilizer. The objective of this study was to evaluate the vegetation index and spatial variability of physical and chemical soil properties in an integrated crop-livestock system (ICLS). Soil samples were taken from a 6.9 ha area in a regular hexagon grid at 0-0.20 m depths. Soil P, K, Ca, Mg, and cation exchange capacity - CEC; base saturation; clay and sand were analyzed. Soil electrical conductivity (ECa) was measured with a contact sensor. The site was evaluated at the end of the corn season (April) and during forage production (October) using Landsat 5 images, remote sensing techniques and a geographic information system (GIS). Results showed that the normalized difference vegetation index (NDVI) was associated with ECa and soil parameters, indicating crop and pasture variations in the ICLS. Geostatistics and GIS were effective tools for collecting data regarding the spatial variability of soil and crop indicators, identifying variation trends in the data, and assisting data interpretation to determine adequate management strategies.201

    Creatine and guanidinoacetate reference values in a French population

    Get PDF
    Creatine and guanidinoacetate are biomarkers of creatine metabolism. Their assays in body fluids may be used for detecting patients with primary creatine deficiency disorders (PCDD), a class of inherited diseases. Their laboratory values in blood and urine may vary with age, requiring that reference normal values are given within the age range. Despite the long known role of creatine for muscle physiology, muscle signs are not necessarily the major complaint expressed by PCDD patients. These disorders drastically affect brain function inducing, in patients, intellectual disability, autistic behavior and other neurological signs (delays in speech and language, epilepsy, ataxia, dystonia and choreoathetosis), being a common feature the drop in brain creatine content. For this reason, screening of PCDD patients has been repeatedly carried out in populations with neurological signs. This report is aimed at providing reference laboratory values and related age ranges found for a large scale population of patients with neurological signs (more than 6 thousand patients) previously serving as a background population for screening French patients with PCDD. These reference laboratory values and age ranges compare rather favorably with literature values for healthy populations. Some differences are also observed, and female participants are discriminated from male participants as regards to urine but not blood values including creatine on creatinine ratio and guanidinoacetate on creatinine ratio values. Such gender differences were previously observed in healthy populations; they might be explained by literature differential effects of testosterone and estrogen in adolescents and adults, and by estrogen effects in prepubertal age on SLC6A8 function. Finally, though they were acquired on a population with neurological signs, the present data might reasonably serve as reference laboratory values in any future medical study exploring abnormalities of creatine metabolism and transport

    The Impact of Worker Effort on Public Sentiment Towards Temporary Migrants

    Full text link
    Temporary and circular migration programs have been devised by many destination countries and supported by the European Commission as a policy to reduce welfare and social costs of immigration in destination countries. In this paper we present an additional reason for proposing temporary migration policies based on the characteristics of the foreign labor-effort supply. The level of effort exerted by migrants, which decreases over their duration in the host country, positively affects production, real wages and capital owners' profits. We show that the acceptance of job offers by migrants result in the displacement in employment of national workers. However it increases the workers' exertion, decreases prices and thus can counter anti-immigrant voter sentiment. Therefore, the favorable sentiment of the capital owners and the local population towards migrants may rise when temporary migration policies are adopted

    Social Closure and the Evolution of Cooperation via Indirect Reciprocity

    Get PDF
    Direct and indirect reciprocity are good candidates to explain the fundamental problem of evolution of cooperation. We explore the conditions under which different types of reciprocity gain dominance and their performances in sustaining cooperation in the PD played on simple networks. We confirm that direct reciprocity gains dominance over indirect reciprocity strategies also in larger populations, as long as it has no memory constraints. In the absence of direct reciprocity, or when its memory is flawed, different forms of indirect reciprocity strategies are able to dominate and to support cooperation. We show that indirect reciprocity relying on social capital inherent in closed triads is the best competitor among them, outperforming indirect reciprocity that uses information from any source. Results hold in a wide range of conditions with different evolutionary update rules, extent of evolutionary pressure, initial conditions, population size, and density
    corecore