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Summary Male and female siblings demonstrated similar facial features and had seizures from birth. Neurologic development, which was delayed, began to deteriorate at 1 year. Sudden death occurred at 2 8/12 and 2 3/12 years of age associated with respiratory infections. Tanning of the skin was noted 2 months before death in the first child. In the second child, blood cortisol levels failed to increase after intravenous ACTH administration, and computerized axial tomography (CAT) scans were normal.At autopsy both patients demonstrated adrenal atrophy and degenerative changes of the white matter throughout the neuraxis. We propose that these siblings have a new form of adrenoleukodystrophy that can be distinguished from the X-linked form by onset at birth, clinical appearance, and pattern of inheritance.A comparison of these cases with a second disorder, Zellweger's syndrome, suggests that a distinctive phenotype is associated with intrauterine degeneration of white matter.  相似文献   
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Marking activities in many ungulate species involve some aggression directed towards the vegetation which usually affects the understorey layer. Roe deer bucks (Capreolus capreolus) usually defend mating territories within the forest, where they mark the vegetation and the ground by using chemical signals which are typically associated with the visual ones used. Here we describe the presence of roe-made clearings of about 2700 m2 average size which contained a clump of marks. Roe bucks affected the vegetation mainly by means of fraying and scratching with antlers around the stem of bushes, and defoliation by leaf-biting. The analysis of these activities suggests that their evolution may have been moulded by the effect of shrub clearing. Defoliation affecting more than 50% of leaves was only found within clearings. Also, fraying affected the whole perimeter of the stem and hence desiccated the bushes at higher rates within the clearings than outside. We found only one cleared area per male range, selectively located in a concave land formation. Producing a clump of scent marks within the mating territory may reduce the costs of scent marking, improve the effectiveness of scent guarding and fits well with the hypothesis of reliable scent matching by recipients. The concave shape of the clump, coupled with the cleared vegetation, may improve the perception of visual signals allowing for the location of scents. It is proposed that selection may favour the modification of the habitat as a component of roe deer marking behaviour, via its benefits in improving the broadcasting of visual signals associated with chemical ones.  相似文献   
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Reviews in Environmental Science and Bio/Technology - The growing and pervasive presence of plastic pollution has attracted considerable interest in recent years, especially small...  相似文献   
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Fumana is a diverse genus of the Cistaceae family, consisting of 21 currently accepted species. In this study, nuclear (ITS) and plastid (matK, trnT‐L) molecular markers were used to reconstruct the phylogeny and to estimate divergence times, including 19 species of Fumana. Phylogenetic analyses (Bayesian Inference, Maximum Parsimony and Maximum Likelihood) confirmed the monophyly of Fumana and did not support the infrageneric divisions previously established. The results support four main clades that group species that differ in vegetative and reproductive characters. Given the impossibility to define morphological characters common to all species within the clades, our proposal is to reject infrageneric divisions. Molecular dating and ancestral area analyses provide evidence for a Miocene diversification of the genus in the north‐western Mediterranean. Ancestral state reconstructions revealed ancestral character states for some traits related to xeric and arid habitats, suggesting a preadaptation to the Mediterranean climate.  相似文献   
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Chickpea contains high levels of protein, vitamins and minerals. Acceptable chickpea yield is the result of meeting nitrogen and phosphorus requirements. The effect of appropriately meeting such requirements reflects on growth and can easily be evaluated using growth analysis. This research determined: (a) The effect of nitrogen and phosphorus fertilization on phenology, net assimilation rate, number of green leaves, leaf area, leaf area index and leaf area duration; (b) Green chickpea yield and number of pods due to fertilization; and (c) The combination of nitrogen and phosphorus fertilization that yields the most net revenue. Nitrogen and phosphorus fertilization was evaluated; each at the doses of 0, 75 and 150 kg ha–1 (N0, N75, N150; P0, P75 and P150, respectively). The combination of the levels of both nutrients generated nine combinations of treatments which were distributed in the field in a randomized complete block design in an arrangement of divided plots with four repetitions. Timing of phenological phases were similar among treatments. Nitrogen and phosphorus fertilization increased number of leaves, leaf area index, and leaf area duration that translated into increased green chickpea yield (GCY). Combinations N150-P75 and N150-P150 produced the highest GCY. The highest net revenue and revenue per peso invested was obtained with N150-P75.  相似文献   
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Journal of Physiology and Biochemistry - The high-risk human papillomavirus (HR-HPV) E7 oncoprotein appears to be a major determinant for cell immortalization and transformation altering critical...  相似文献   
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