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A submicroscopic genomic duplication in Xq22.2 that contains the entire proteolipid protein 1 gene (PLP1) is responsible for the majority of Pelizaeus–Merzbacher disease (PMD) patients. We previously developed an interphase FISH assay to screen for PLP1 duplications in PMD patients using peripheral blood and lymphoblastoid cell lines. This assay has been utilized as a clinical diagnostic test in our cytogenetics laboratory. To expand usage of the interphase FISH assay to prenatal diagnosis of PLP1 duplications, we examined three PMD families with PLP1 duplications utilizing aminiotic fluid samples. In two families the FISH assay revealed fetuses with PLP1 duplications, whereas the other fetus showed a normal copy number of PLP1. Haplotype analyses, as well as an additional FISH analysis using postnatal blood samples, confirmed the results of the prenatal analyses. Our study demonstrates utility of the interphase FISH assay in the prenatal diagnosis of PLP1 duplications in PMD. Copyright © 2001 John Wiley & Sons, Ltd.  相似文献   
43.
Carbon Molecular Sieving Membranes Derived from Lignin-Based Materials   总被引:1,自引:0,他引:1  
Carbon molecular sieving membranes were prepared by pyrolysis of lignocresol derived from lignin by the phase-separation method. Lignocresol membranes formed by a dip process on a porous -alumina tubing were carbonized at 400–800°C under nitrogen atmosphere. The thickness of the membrane formed on the outer surface of the substrate was about 400 nm judging from SEM observation. Gas-evolving behavior of lignocresol was measured using thermogravimetry-mass spectrometry (TG-MS). The gaseous products evolved from lignocresol included a number of fragments with higher molecular weights; whereas those from phenolic resin are mainly due to phenol and methylphenol. These evolved pyrolysis fragments effectively contribute to micropore formation of carbonized lignocresol membranes. Gas permeation rates through the membrane decreased in the order of increasing kinetic molecular diameter of the penetrant gas, and the membrane behaved like a molecular sieve. The permeation properties were dependent on heating conditions, and a pyrolysis temperature of 600°C gave the best membrane performance. Gas selectivities of the membrane prepared at 600°C were 50, 8, 290, and 87 for CO2/N2, O2/N2, H2/CH4, and CO2/CH4 at 35°C, respectively.  相似文献   
44.
The evolution of the gall-inducing ability in insects and the adaptive significance of the galling habit have been addressed by many studies. Cicadulina bipunctata, the maize orange leafhopper, is an ideal study organism for evaluating these topics because it can be mass-reared and it feeds on model plants such as rice (Oryza sativa) and maize (Zea mays). To reveal differences between gall inductions by C. bipunctata and other gall inducers, we conducted four experiments concerning (a) the relationship between the feeding site and gall-induction sites of C. bipunctata on maize, (b) the effects of leafhopper sex and density, (c) the effects of length of infestation on gall induction, and (d) the effects of continuous infestation. C. bipunctata did not induce galls on the leaves where it fed but induced galls on other leaves situated at more distal positions. The degree of gall induction was significantly correlated with infestation density and length. These results indicate that C. bipunctata induces galls in a dose-dependent manner on leaves distant from feeding sites, probably by injecting chemical(s) to the plant during feeding. We suggest that insect galls are induced by a chemical stimulus injected by gall inducers during feeding into the hosts.  相似文献   
45.
We tested the hypothesis that a large body size and rapid growth rate affect the survival of larval Pacific bluefin tuna, Thunnus orientalis (PBT), and analyzed larval growth in relation to environmental conditions. Seven high density larval patches of PBT were tracked with reference buoys in the northwestern Pacific Ocean for 28–171 h in May–June from 2004 to 2008. The otolith radii and daily growth rates of the survivor larvae (collected on later tracking days of each tracking session) tended to be larger and more rapid, respectively, than those of original larvae (collected on earlier tracking days). A large body size was found to positively affect the survival of larval PBT, as did a rapid growth rate, even at an early larval stage (7 days after hatching). Generalized linear modeling showed that the otolith radius was influenced positively by the sea temperature, stratification parameter and food density, while the growth rate was influenced positively by the sea temperature and food density.  相似文献   
46.
The vertical diffusional mass (solute) transfer through a suspended sediment layer, e.g. at the bottom of a lake, reservoir or estuary, by the propagation of velocity fluctuations from above was investigated. The attenuation of the velocity fluctuations in the suspension layer and the associated effect on solute transfer through the suspension layer was simulated. To represent large eddies traveling downstream in water over a high-concentration suspended sediment layer, a streamwise velocity fluctuation moving in downstream direction was imposed along the upper boundary of the suspension layer. Velocity fluctuations and downstream velocity were normalized by the shearvelocity (U*) at the top of the suspension layer. Streamwise and vertical velocity components inside the suspension layer, were obtained from the 2-D continuity and the Navier–Stokes equations. The persistence of turbulence with depth—as it penetrates from the overlying water into the suspension layer—was found to depend on its amplitude, its period, and on the apparent viscosity of the suspension. The turbulence was found to propagate efficiently into the suspension layer when its frequency is low, and the apparent viscosity of the suspension is high. Effects on vertical mass transfer were parameterized by penetration depth and effective diffusion coefficient, and related to apparent viscosity of the suspension, Schmidt number and shear velocity on top of the suspension layer. The enhancement of turbulence penetration by viscosity is similar to the flow near an oscillating flat plate (Stokes’ second problem), but is opposite to turbulence penetration into a stationary porous and permeable sediment bed. The information is applicable to water quality modeling mear the sediment/water interface of lakes, river impoundments and estuaries.  相似文献   
47.
Forests managed through peoples' participation can help to achieve a number of sustainable development goals (SDGs). This paper, drawing data from four participatory forest management (PFM) projects in south-eastern Bangladesh, explored: (a) the factors that influenced the outcomes of PFM; and (b) contribution of PFM towards achieving selected SDGs. The research used observation, semi-structured household interviews, group discussions, vegetation survey and Ostromʼs design principles for data collection, Divergent forest conditions and factors in four PFM sites have had differential contribution to SDGs. Heterogeneity of user groups, insufficient coordination, absence of motivation, and land tenure conflicts had affected the attainment of SDGs. The PFM helped to achieve 9–11 SDGs comprising poverty reduction (Goal 1), improving food security (Goal 2), maintaining health and well-being (Goal 3), enhancing childrensʼ education (Goal 4), fair access to jobs and committees (Goals 5 and 10), sources of clean drinking water (Goal 6), affordable energy for cooking (Goal 7), increased household economy (Goal 8), climate change through carbon sequestration (Goal 13), forest conservation (Goal 15), and partnerships with relevant institutions (Goal 16). In order to achieve SDGs, PFM could be viewed as an integral part of government development strategies, which needs sectoral coordination and integration of relevant policies.  相似文献   
48.
Hori H  Ishida K  Inoue N  Koike K  Kutsuna S 《Chemosphere》2011,82(8):1129-1134
The decomposition of hydroperfluorocarboxylic acids [H-PFCAs; HCnF2nCOOH (= 4 and 6)] induced by heteropolyacid photocatalyst H4SiW12O40 in water was investigated, and the results are compared with the results for the corresponding perfluorocarboxylic acids (PFCAs; CnF2n+1COOH). This is the first report on the photochemical decomposition of H-PFCAs, which are being developed as alternative surfactants to environmentally persistent and bioaccumulative PFCAs. H-PFCAs were not decomposed by irradiation with UV-Visible light (>290 nm) in the absence of H4SiW12O40. In contrast, UV-Visible light irradiation of H-PFCAs in the presence of H4SiW12O40 efficiently decomposed H-PFCAs to F and CO2. The decomposition reactions showed pseudo-first-order kinetics, and the decomposition rate constants were 1.8-2.5 times higher than those for the corresponding PFCAs. The reaction mechanism can be explained by elimination of H+ from the ω-H atom of the H-PFCAs by the excited catalyst, followed by formation of perfluorodicarboxylic acids.  相似文献   
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50.
The organotrophic potentiality of heterotrophic microorganisms 50 cm above the ocean floor (5,207 m depth) was measured in situ, at a station in the subarctic Pacific Ocean (44° N; 154° E). In certain enriched deep-sea samples incubated in situ, the microorganisms exhibited nearly the same organotrophic activity as those in the surface water layers. Rod-shaped barophilic bacteria (0.4 to 0.6 μ x 1.0 to 2.0 μ), which multiplied at a generation time of about 10 h, were responsible for this activity.  相似文献   
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