136 research outputs found

    Um modelo de caracterização e avaliação ecológica para o planeamento e gestão

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    Decorrem actualmente na Universidade de Évora diversos projectos de investigação visando o desenvolvimento de metodologias de caracterização e avaliação ecológica com vista ao planeamento e gestão do território. Tais projectos, desenvolvidos a escalas complementares (1:10 000; 1:25 000 e 1:100 000), pretendem via a constituir um quadro metodológico inovador integrando num único plano de caracterização e avaliação, o plano dos sistemas de uso e o plano dos sistemas ecológicos espaciais. A necessidade de um tal sistema integrado justifica-se pela carência de um referencial de caracterização e avaliação do território com vista à articulação od sprocesos de gestão e à promoção dos objectivos de Conservação da Natureza. Com efeito, a necessidade de assegurar a preservação da biodiversidade da globalidade do território impõe uma abordagem à gestaõ do uso deste que assegure que o nível de fragmentação, de conectividade residual, e de complementaridade funcional asseguram as condições para a sustentação viável de meta-populações das espécie-objectivo. Para atingir tal objectivo importa conhecer e conseguir articular nos processos de simulação e gestão, as necessidades, processos e funções dos diferentes usos antrópicos de uma dada região e as necessidades processos, funções e condicionantes espaciais dos diferentes sistemas ecológicos susceptiveis de ocorrerem nessa região ou locais. Interessa igualmente identificat a forma como essa articulação se processa e a sua hierarquização escalar e funcional

    Density Perturbations in the Brans-Dicke Theory

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    We analyse the fate of density perturbation in the Brans-Dicke Theory, giving a general classification of the solutions of the perturbed equations when the scale factor of the background evolves as a power law. We study with details the cases of vacuum, inflation, radiation and incoherent matter. We find, for the a negative Brans-Dicke parameter, a significant amplification of perturbations.Comment: 26 pages, latex fil

    The alpha-galactosidase A p.Arg118Cys variant does not cause a Fabry disease phenotype: data from individual patients and family studies

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    Acessível em: www.ncbi.nlm.nih.gov/pmc/articles/PMC4423738/Lysosomal α-galactosidase A (α-Gal) is the enzyme deficient in Fabry disease (FD), an X-linked glycosphingolipidosis caused by pathogenic mutations affecting the GLA gene. The early-onset, multi-systemic FD classical phenotype is associated with absent or severe enzyme deficiency, as measured by in vitro assays, but patients with higher levels of residual α-Gal activity may have later-onset, more organ-restricted clinical presentations. A change in the codon 118 of the wild-type α-Gal sequence, replacing basic arginine by a potentially sulfhydryl-binding cysteine residue - GLA p.(Arg118Cys) -, has been recurrently described in large FD screening studies of high-risk patients. Although the Cys118 allele is associated with high residual α-Gal activity in vitro, it has been classified as a pathogenic mutation, mainly on the basis of theoretical arguments about the chemistry of the cysteine residue. However its pathogenicity has never been convincingly demonstrated by pathology criteria. We reviewed the clinical, biochemical and histopathology data obtained from 22 individuals of Portuguese and Spanish ancestry carrying the Cys118 allele, including 3 homozygous females. Cases were identified either on the differential diagnosis of possible FD manifestations and on case-finding studies (n=11; 4 males), or on unbiased cascade screening of probands' close relatives (n=11; 3 males). Overall, those data strongly suggest that the GLA p.(Arg118Cys) variant does not segregate with FD clinical phenotypes in a Mendelian fashion, but might be a modulator of the multifactorial risk of cerebrovascular disease. The Cys118 allelic frequency in healthy Portuguese adults (n=696) has been estimated as 0.001, therefore not qualifying for "rare" condition

    Phenotypic, cytogenetic and spike fertility characterization of a population of male-sterile triticale

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    Triticale (X Triticosecale Wittmack) is a good cereal for production of flour and feed. A segregating population of triticale was developed from a male-sterile (MS) plant. To determine whether this new source of male sterility in triticale is appropriate for use in breeding programs the expression of the male sterility phenotype was characterized through spike fertility, meiotic behavior, and pollen. Controlled crosses between male-sterile plants and control varieties male-fertile (MF) of triticale were also conducted, and cytological analyses were performed in the F2 and backcross plants. Plants with male-sterile phenotypes displayed reduced spike fertility when compared to plants with male-fertile phenotypes. Compared to male-fertile plants, male-sterile plants exhibited a lower percentage of normal meiotic cells, a reduced meiotic index and reduced pollen viability. The F2 plants had improved pollen fertility when compared to the male-sterile population; however there were no corresponding improvements in the percentage of normal meiotic cells or in the meiotic index. A single generation of backcrosses resulted in an improved meiotic index and increased pollen viability. However, no changes in the percentage of normal meiotic cells were observed. Meiotic instability, which was shown to be inheritable, was the likely cause of male sterility. Therefore, the use of this population in triticale breeding was considered to be inappropriate because it could promote or contribute to the maintenance of meiotic instability, which is commonly observed in this species
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