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991.
992.
    
Head and neck paragangliomas (HNPGLs) are rare neuroendocrine neoplasms derived from the parasympathetic paraganglia of the head and neck. At least 30% of HNPGLs are linked to germline mutations, predominantly in SDHx genes. In this study, we analyzed an extended cohort of Russian patients with HNPGLs using whole-exome sequencing and found a highly frequent missense variant p.H102R in the SDHD gene. We determined this variant in 34% of the SDHD mutation carriers. This variant was associated with somatic loss of the gene wild-type allele. Data from the B allele frequency method and microsatellite and microdeletion analysis indicated evident LOH at the 11p15.5 region and potential loss of the whole of chromosome 11. We found hypermethylation of H19-DMR in all tumors, whereas differential methylation of KvDMR was mostly retained. These findings do not support the paternal transmission of SDHD:p.H102R but are in agreement with the Hensen model. Using targeted sequencing, we also studied the variant frequency in a control cohort; we found SDHD:p.H102R in 1.9% of cases, allowing us to classify this variant as pathogenic. The immunohistochemistry of SDHB showed that the SDHD:p.H102R mutation, even in combination with wild-type allele loss, does not always lead to SDH deficiency. The obtained results demonstrate the frequent variant associated with HNPGLs in a Russian population and support its pathogenicity. Our findings help with understanding the mechanism of tumorigenesis and are also important for the development of cost-effective genetic screening programs.  相似文献   
993.
A high-efficiency separation of oil and water can be achieved by using specially designed amphiphilic porous membrane. However, the preparation of such membranes often involves complex multistep chemical processes. Herein, we report an amphiphilic composite membrane (polystyrene [PS]/bacterial cellulose [BC] membrane) consisting of hydrophobic recycled PS and hydrophilic BC, fabricated by a facile in situ fermentation process. Not only these membranes exhibit a combination of contrasting wettability but also comprise of a hierarchical network of microfibers and nanofibers, which makes them ideal for oil–water separation. The structural and morphological properties of as-produced BC, recycled PS membrane, and PS/BC composite membrane were studied by Fourier transform infrared spectroscopy and scanning electron microscopy, respectively. The ability of the membranes to separate oil and water was tested by using an emulsion of hexane-in-water as the feed and the collected filtrates were characterized by optical microscopy and UV–Vis spectroscopy. PS membranes were unable to separate oil and water, while the PS/BC membrane efficiently separated water from the emulsion. PS/BC composite membranes showed a high water recovery of more than 90%, against only 57% recovery shown by BC. Mechanisms of oil–water separation for each membrane are discussed. The reusability of the PS/BC composite membrane was also demonstrated.  相似文献   
994.
    
The c subunit of the ATP synthase is an inner mitochondrial membrane (IMM) protein. Besides its role as the main component of the rotor of the ATP synthase, c subunit from mammalian mitochondria exhibits ion channel activity. In particular, c subunit may be involved in one of the pathways leading to the formation of the permeability transition pore (PTP) during mitochondrial permeability transition (PT), a phenomenon consisting of the permeabilization of the IMM due to high levels of calcium. Our previous study on the synthetic c subunit showed that high concentrations of calcium induce misfolding into cross-β oligomers that form low-conductance channels in model lipid bilayers of about 400 pS. Here, we studied the effect of cyclophilin D (CypD), a mitochondrial chaperone and major regulator of PTP, on the electrophysiological activity of the c subunit to evaluate its role in the functional properties of c subunit. Our study shows that in presence of CypD, c subunit exhibits a larger conductance, up to 4 nS, that could be related to its potential role in mitochondrial toxicity. Further, our results suggest that CypD is necessary for the formation of c subunit induced PTP but may not be an integral part of the pore.  相似文献   
995.
High-velocity sedimentation, translational isothermal diffusion, and viscometry in H2O and DMF are used to investigate the samples and fractions of poly(N-methyl-N-vinylacetamide) synthesized by free-radical polymerization and fractionated in a chloroform-diethyl ether system. Molecular masses M and the Mark-Kuhn-Houwink-Sakurada relations are obtained for the fractions in the molecular mass range M × 10−3 = 3.5−540.0. The negative temperature coefficient of intrinsic viscosity is revealed for both solvents. The length of the Kuhn statistical segment and the hydrodynamic diameter of poly(N-methyl-N-vinylacetamide) macromolecules are estimated; the hydrodynamic volumes occupied by water-soluble poly(N-methyl-N-vinylacetamide), poly(1-vinyl-2-pyrrolidone), poly(vinylformamide), and pullulan molecules are compared.  相似文献   
996.
薄膜太阳电池的发展值得重视   总被引:1,自引:0,他引:1  
根据当前光伏市场的迅速发展,要求人们寻找一种高效率低成本的适合规模化生产的太阳电池。作者认为,薄膜太阳电池具有优良的特性,是一种很有发展前途的低成本太阳电池。美、日、欧正在迅速成功地开展薄膜太阳电池的研究与开发,而中国在该项技术上还处于实验室研究阶段,应加快向企业化过渡。  相似文献   
997.
The restoration of the graphite masonry of cell 52-16 in the reactor in the third power-generating unit of the Leningrad nuclear power plant is described. The process reduces to moving graphite blocks and columns from adjacent cells into the damaged cell, from which defective blocks are removed beforehand. The columns are moved in a manner so that the empty cell ends up in the region of the steam-gas exhaust passage through the upper metal structure of the reactor unit. The missing blocks are placed in the reactor through the passage. Organizational-technical and radiation questions concerning the work performed are described. 6 figures. Leningrad Nuclear Power Plant. Translated from Atomnaya énergiya, Vol. 87, No. 5, pp. 356–359, November, 1999.  相似文献   
998.
999.
Semiconductors - III–V/Ge/Si(001), III–V/Ge/SOI(001), and III–V/GaAs(001) heterostructures are fabricated and investigated. The Ge buffer layer for the III–V/Ge/Si structure...  相似文献   
1000.
A high-performance low-power 153 Mb SRAM is developed in 45 nm high-k Metal Gate technology. Dynamic SRAM PMOS forward-body-bias (FBB) and Active-Controlled SRAM VCC in Sleep are integrated in the design to lower Active-VCCmin and Standby Leakage, respectively. FBB improves the Active-VCCmin by up to 75 mV, and Active-Controlled SRAM VCC distribution tightened by 100 mV, both of which result in further power reduction. A 0.346 mum2 6T-SRAM bit-cell is used which is optimized for VCCmin, performance, leakage and area. The design operates at high-speed over a wide voltage range, and has a maximum frequency of 3.8 GHz at 1.1 V. The 16 KB Subarray was also used as the building block in on-die 6 MB Cache for Intel Core 2 Duo CPU in 45 nm technology.  相似文献   
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