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1.
A family with two siblings, 10 and 8 years old, both with clinical and ultrastructural evidence of juvenile neuronal ceroid lipofuscinosis is described. The family was found to be informative for the restriction fragment length polymorphisms (RFLPs) detected by the probes pCJ52–95Ml (locus D16S148) and pCJ52-94Tl (locus D16S159) flanking the juvenile neuronal ceroid lipofuscinosis locus, CLN3. The parents were both heterozygous using these probes, while their two children with juvenile neuronal ceroid lipofuscinosis were both homozygous. Chorionic villi analysis showed that the fetus was heterozygous and had inherited the one allele of the mother which was not found in the two siblings. This suggested that the fetus had derived one healthy allele from the mother, the risk for a double crossing-over being less than 1 per cent. Electron microscopy showed no fingerprint inclusions in chorionic villi. The child was investigated at 6 months of age and found to be healthy, as new fingerprint inclusions were found at electron microscopy and no vacuolated lymphocytes were found in the blood smear. Due to the risk of heterogeneity, both DNA-based analysis and electron microscopy on chorionic villi are recommended for prenatal examination for juvenile neuronal ceroid lipofuscinosis.  相似文献   
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Summary The influence of pulsed magnetic fields (PMF) on the properties of nervous tissue was investigated. Hippocampal slices or synaptosomes obtained from hippocampal tissue were used as model systems. The amplitude of potentials recorded in vitro from one of the hippocampal pathways (Schaffer collaterals that use glutamate as a neurotransmitter) was employed as a measure of the influence of magnetic fields on synaptic efficiency. The synaptic glutamate turnover and radioactive calcium accumulation were used as markers of the PMF influence on biochemistry of synaptic mechanisms. The exposure of hippocampal slices for 30 min to PMF amplified evoked potentials. While the frequency of 0.16 Hz exerted the strongest effect, lower (0.01, 0.07, 0.03 Hz) and higher (0.5 Hz) frequencies were much less effective. The enhancement of the neuronal excitability was correlated with significant increase in the neuronal spontaneous activity mediated by electrical synapses. The PMF-induced changes in the excitability of the tissue were accompanied by an increase in the synaptic turnover of glutamate. The release of radioactive D-Aspartate (a glutamate analog used as a marker for glutamate turnover) from the slices, and its uptake by synaptosomes were enhanced, and reduced respectively, following the stimulation with pulsed magnetic fields. The frequency which was the most efficient in amplification of evoked potentials (0.16 Hz) was also the most effective in the modulation of the release and uptake processes. The PMF-induced changes in neurotransmitter turnover coincided with an increase in 45Ca2+ accumulation observed in hippocampal slices exposed to PMF.  相似文献   
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Effects of C60 nanoparticles (nominal concentrations 0, 15.4 and 154 mg/kg soil) on mortality, growth and reproduction of Lumbricus rubellus earthworms were assessed. C60 exposure had a significant effect on cocoon production, juvenile growth rate and mortality. These endpoints were used to model effects on the population level. This demonstrated reduced population growth rate with increasing C60 concentrations. Furthermore, a shift in stage structure was shown for C60 exposed populations, i.e. a larger proportion of juveniles. This result implies that the lower juvenile growth rate due to exposure to C60 resulted in a larger proportion of juveniles, despite increased mortality among juveniles. Overall, this study indicates that C60 exposure may seriously affect earthworm populations. Furthermore, it was demonstrated that juveniles were more sensitive to C60 exposure than adults.  相似文献   
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The caterpillars of Eucheira socialis westwoodi cooperatively spin and maintain a hollow silken nest and an elaborate network of silken foraging trails on their host plant, madrone (Arbutus spp.: Ericaceae). Nests typically contain several hundred larvae. Two populations are known to harbor a sex ratio distorter. The primary sex ratio in these two populations for four generations has been exceedingly male biased (64–79% male). Lepidoptera larvae are easily sexed using external morphology, allowing us to uniquely mark male and female larvae and to assemble larval groups of particular sex ratios. We report here the results of experiments on sex-specific larval behavior and physiology and the effect of colony sex ratio on individual behavior. We found that male larvae spent more time spinning silk on the nest and less time feeding than female larvae. Males were the first to emerge from the nest and the first to venture out along trails to feed. Male-biased nests had a significantly greater amount of silk deposited on their surfaces than female-biased nests. In the field, male-biased nests produced heavier male and female pupae than female-biased nests. Male and female larvae in 75% male nests became active earlier than males and females in other sex ratio treatments. Received: 11 September 1998 / Received in revised form: 24 February 1999 / Accepted: 27 March 1999  相似文献   
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长江口中华鲟保护区及临近水域大型底栖动物研究   总被引:3,自引:0,他引:3  
根据2004年5月、8月、11月和2005年2月(Ⅰ年度)及2005年8月、11月和2006年2月、5月(Ⅱ年度)共2个水文年对T长江口中华鲟保护区水域(30°15′~31°45′N、121°30′~122°15′E)15个取样站8个航次的采泥样品和阿氏网样品,对底内动物和底上动物的种类组成、生物量、栖息密度、重要种的优势度、群落多样性和结构及时间空间动态变化进行分析.结果表明,底内动物共出现15种,种类组成贫乏,优势种变化明显,底内动物生物量和栖息密度平均值分别为1.44 g/m2和11.33个/m2,生物量和栖息密度总体呈现下降趋势,物种多样性指数低并有下降趋势,群落结构极不稳定;底上动物底栖动物59种,组成有明显季节和年度变化,底内动物生物量和栖息密度平均值分别为15.58 ×10-2g/m2和6.81×10-2个/m2;底上动物生物量和栖息密度略呈现下降趋势,优势种的种类及优势度年际有较大变化,种类数和生物量北支高于南支.底内动物2个年度生物量平均值季节变化为夏季>春季>秋季>冬季,底上动物2个年度生物量平均值季节变化为春季>冬季>秋季>夏季,底内动物与底上动物的季节变化具有一定的不平衡性.  相似文献   
9.
The age at which worker honey bees begin foraging varies under different colony conditions. Previous studies have shown that juvenile hormone (JH) mediates this behavioral plasticity, and that worker-worker interactions influence both JH titers and age at first foraging. These results also indicated that the age at first foraging is delayed in the presence of foragers, suggesting that colony age demography directly influences temporal division of labor. We tested this hypothesis by determining whether behavioral or physiological development can be accelerated, delayed, or reversed by altering colony age structure. In three out of three trials, earlier onset of foraging was induced in colonies depleted of foragers compared to colonies depleted of an equal number of bees across all age classes. In two out of three trials, delayed onset of foraging was induced in colonies in which foragers were confined compared to colonies with free-flying foragers. Finally, in three out of three trials, both endocrine and exocrine changes associated with reversion from foraging to brood care were induced in colonies composed of all old bees and devoid of brood; JH titers decreased and hypopharyngeal glands regenerated. These results demonstrate that plasticity in age-related division of labor in honey bee colonies is at least partially controlled by social factors. The implications of these results are discussed for the recently developed ‘‘activator-inhibitor” model for honey bee behavioral development. Received: 8 November 1995/Accepted after revision: 10 May 1996  相似文献   
10.
Prenatal diagnosis in a pregnancy at risk for late infantile neuronal ceroid lipofuscinosis (Batten's disease) was undertaken at 17 weeks' gestation by ultrastructural examination of amniotic fluid cells. The presence of curvilinear profiles indicated an affected fetus and the diagnosis was confirmed, after the pregnancy was terminated, by the finding of many typical curvilinear profiles in multiple tissues which included skin, amnion, umbilical vessels, blood, liver, and brain. Comparison between the involved cells in the amniotic fluid and fetal tissues suggests that these cells are probably derived from the periderm, and possibly also from the amnion. The prominent presence of cytosomes in the periderm and intermediate cells of the fetal epidermis and occasionally also in the endothelial cells of the dermis suggests that fetal skin may be a useful alternative site for assessing fetal involvement. Control specimens of the amniotic fluid, fetal skin, amnion, and liver showed no similar cytosomes. However, some control amniotic fluid samples did contain cells with large collections of irregular trilaminar membranes, and these could be open to misinterpretation. It is important that only typical curvilinear profiles are considered as an indication of an affected pregnancy.  相似文献   
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