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11.
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   
12.
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   
13.
藏北高原土壤温度异常变化及其与雪灾关系初析   总被引:10,自引:1,他引:10  
通过对GAME-Tibet野外工作期间所得藏北高原一个年周期的土壤温度资料的初步分析,发现藏北高原1997 ̄1998年冬半年土壤日温差的分布存在明显的异常现象;指出了这种异常现象的发生可能与藏北高原1997年冬天的特大雪灾有关。从浅层土壤日温差的异常变化,定性地说明了藏北高原1997 ̄1998年冬半年不同地点雪灾的严重程度,但由于受资料的限制,目前尚无法进行定量的评估。  相似文献   
14.
The apparent aqueous solubility of the endocrine disruptor in hydroxypropyl‐cyclodextrin (HP‐CD) solutions was investigated for evaluating the remediation of soil contamination and ground water pollution. The apparent solubility of bisphenol A (BPA) in water was significantly increased in HP‐CD solutions. The solubilization effect of HP‐CD's is caused by the formation of inclusion complexes of BPA and cyclodextrin. The relative aqueous‐phase concentration of BPA linearly increased with increasing HP‐CD concentration. The solubility of BPA increased in the order of the addition of HP‐a‐CD, HP‐7‐CD, and HP‐ß‐CD. However, it did not depend upon the degree of hydroxypropylation substitution. It is concluded that HP‐CD may be utilized for the remediation of soil contamination.  相似文献   
15.
We investigated the effects of population fluctuation on the offspring’s sex allocation by a weakly polygynous mouse, Apodemus argenteus, for 3 years. In acorn-poor seasons, heavier mothers invested more in sons, and lighter mothers invested more in daughters. In acorn-rich seasons, heavier mothers invested more in daughters, and lighter mothers invested more in sons. Maternal body condition and litter size affected the sex allocation. Furthermore, there was a maternal investment trade-off between a son’s birth mass and the number of daughters. Based upon the effect of population fluctuation on the lifetime reproductive success of each sex, we proposed the new “safe bet hypothesis”. This hypothesis predicts that frequent and unpredictable change in female distribution, which is often caused by abrupt fall in food condition, favors female-biased maternal investment to offspring by polygynous mammals and is applicable to many small mammals inhabiting in unstable environments.  相似文献   
16.
Orally administered 3H-benzo(a)pyrene (BP) was concentrated in liver, kidney and lung of rat. Liver contained BP-diols, BP-quinones and 3-hydroxy-BP, and extremely high amounts of BP-quinones and unmetabolized BP were found in lung until after 7 hr of administration. In kidney, BP-quinones and BP-diols were major metabolites, but not BP or 3-hydroxy-BP. Low level of metabolites as conjugates with glucuronate and more polar compounds were also detected in kidney. These results suggested that an orally administered BP was metabolized to BP-diols and 3-hydroxy-BP mainly in liver, to BP-quinones mainly in lung, and to BP-diols and glucuronides or more polar compounds mainly in kidney.  相似文献   
17.
This study examines slag, fly ash, and deposited particles during melting of dewatered sewage sludge in a pilot plant. In addition, the chemical composition of particles in flue gas was simulated using a thermodynamics program, namely FACTSage 5.2. The results showed that the main components of slag were Al, Fe, Ca, P and Si; the minor components were Na, K, Mg, Cu, and Zn. The main chemical compound of slag was Ca4(Mg,Fe)5(PO4)6. For fly ash particles, heavy metals with the highest concentrations were in the order of Zn and As, Pb, Cu, and Cd, respectively. For non-heavy metals, Al, Fe and P were also found in significant amounts. The majority of deposited particles were composed of elements of Zn, P, S, Na, Fe, Al, Si, and Ca and such chemical compounds as Zn3(PO4)2, AlPO4, FePO4 and Fe(OH)3 while the minority consisted of elements of As, Cu, and Pb. Moreover, the compositions of deposited particles in each chamber differed due to different flue gas temperatures inside. In the secondary chamber at 760 degrees C, the amounts of Fe and Al were higher than Zn, whereas, in the other chambers (600-400 degrees C), the amount of Zn was higher. In other words, at the lower temperature the deposition of Zn was higher than the deposition of Fe and Al. In the water cooling section, volatile elements (i.e. Zn, As, Cu, Pb) were found in the highest concentrations due to a big difference in temperature between the wall surface and flue gas. From the simulation results, most of the elements in the gas phase were found to be chloride compounds, whereas those in the solid phase were in the form of oxide, sulfate, and phosphate compounds.  相似文献   
18.
Newspapers impregnated with NaCl mixed with various chloride metals (CuCl2, MgCl2, MnCl2, FeCl3, NiCl2, and CoCl2) and electric wire coated with polyvinyl chloride (PVC) were combusted in a well-controlled incinerator. Exhaust gas samples collected at the outlet of the incinerator were analyzed for dioxins (PCDDs, PCDFs, and coplanar PCBs) by gas chromatography/mass spectrometry. The amount of total dioxins formed from newspaper samples ranged from 34.2 ng/g (with NaCl+CoCl2) to 67.0 ng/g (with NaCl+CuCl2). PCDFs composed 88-94% of the total dioxins formed in the exhaust gases. The highest levels of PCDF isomers obtained were Cl3-CDF from the sample with NaCl+CuCl2 (14.8 ng/g), Cl2-CDF from the sample with NaCl+MgCl2 (12.3 ng/g), and Cl(1)-CDF from samples with NaCl+MnCl2 (12.6 ng/g), with NaCl+FeCl3 (11.8 ng/g), and with NaCl+NiCl2 (13.3 ng/g), and with NaCl+CoCl2 (8.62 ng/g). The total of Cl4-8-CDDs comprised 76-88% of the total Cl1-8-CDDs. In particular, Cl7-CDDs had the highest levels except for the sample with NaCl+NiCl2. Total dioxins formed from samples of electric wire coated with PVC and PVC alone were 38.3 ng/g and 112 ng/g, respectively, suggesting that the presence of copper reduced dioxin formation.  相似文献   
19.
Identification of pollution sources and estimation of their contribution to dioxin pollution are important for taking better countermeasures against such sources. The information based on seventeen 2,3,7,8-chlorine-substituted congeners and 10 homologues is usually not sufficient for source identification. The usefulness of detailed congener-specific information of tetra- to octachlorinated polychlorinated dibenzo-p-dioxins and polychlorinated dibenzofurans was examined by principal component analysis and compared with conventional dioxin information. It was revealed that congener-specific information was far more effective for source identification than conventional information. For source apportioning, it was shown that multiple regression analysis with detailed source congener profiles could be carried out successfully when calculations were performed for individual homologues. This was because of the large variation in homologue composition in source dioxin profiles in addition to the difference in environmental behavior among the homologues.The newly proposed methods for statistical analysis were applied to the estimation of dioxin mass balance in Tokyo Bay basin. It was found that Tokyo Bay sediment core contained dioxins from three major sources: combustion, pentachlorophenol (PCP), and chloronitrophen (CNP). PCP and CNP contained large amounts of dioxin as impurity and were used extensively as paddy field herbicides in Japan in the past. The total dioxin load from the two herbicides to the basin during the past 45 years (1951-1995) was estimated to be five times larger than that from combustion source in terms of toxic equivalents (WHO-TEQ). However, in the surface sediment of the bay, the contribution from the herbicides was nearly equal to that from combustion. The herbicide contribution peaked during the 1960s and 1970s and gradually decreased thereafter.  相似文献   
20.
Spatial and temporal variations of benthic fauna over a wide region (>1,000 km) that includes complex ocean currents have seldom been quantitatively studied. The present study revealed malacofaunal variations on Japanese rocky shores affected by the Soya, Tsushima, Oyashio, and Kuroshio currents, and in the Inland Sea. On 29 shores in seven regions in these sea areas, previous researchers have recorded the number of molluscs in quadrats in spring and summer of 1978 and 1984–1986. Reanalyses of these records revealed the following patterns. (1) Shore-to-shore variation in malacofauna was correlated with shore-to-shore geographical distance less clearly for more southern regions. This might show that in southern regions many taxa with low relative abundance are haphazardly recorded on some shores and blur the effect of shore-to-shore distance on malacofaunal dissimilarity. (2) Region-to-region variation in malacofauna was significantly correlated with region-to-region geographical distance. This suggests the influences of environments and paleontological events specific to each current surrounding each region. (3) The pattern of dissimilarity between the Inland Sea and the other sea areas greatly changed from the 1970s to 1980s. This was due in part to the following density fluctuations on some shores in the Inland Sea: increases in the non-indigenous mussel Mytilus galloprovincialis, cultured oyster Crassostrea gigas, and littorinid Littorina brevicula reportedly abundant in sheltered areas; and decreases in the littorinid Nodilittorina radiata reportedly abundant in exposed areas and muricid gastropods Thais spp. showing imposex. This suggests an increase of anthropogenic perturbations during the study period in the Inland Sea which is surrounded by large cities and ports. Electronic supplementary material The online version of this article (doi:) contains supplementary material, which is available to authorized users.  相似文献   
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