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The exceptional properties of graphene make it ideal as a reinforcement to enhance the properties of aluminum matrices and this critically depends on uniform dispersion. In this study, the dispersion issue was addressed by sonication and non-covalent surface functionalization of graphite nanoplatelets(GNPs) using two types of surfactant: anionic(sodium dodecyl benzene sulfate(SDBS)) and non-ionic polymeric(ethyl cellulose(EC)). After colloidal mixing with Al powder, consolidation was performed at two sintering temperatures(550 and 620°C). The structure, density, mechanical and wear properties of the nanocomposite samples were investigated and compared with a pure Al and a pure GNPs/Al nanocomposite sample. Noticeably, EC-based 0.5 wt% GNPs/Al samples showed the highest increment of 31% increase in hardness with reduced wear rate of 98.25% at 620°C, while a 22% increase in hardness with reduced wear rate of 96.98% at 550°C was observed, as compared to pure Al. Microstructural analysis and the overall results validate the use of EC-based GNPs/Al nanocomposites as they performed better than pure Al and pure GNPs/Al nanocomposite at both sintering temperatures. 相似文献
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A mutation in OTOF, encoding otoferlin, a FER-1-like protein, causes DFNB9, a nonsyndromic form of deafness 总被引:27,自引:0,他引:27
Yasunaga S Grati M Cohen-Salmon M El-Amraoui A Mustapha M Salem N El-Zir E Loiselet J Petit C 《Nature genetics》1999,21(4):363-369
Using a candidate gene approach, we identified a novel human gene, OTOF, underlying an autosomal recessive, nonsyndromic prelingual deafness, DFNB9. The same nonsense mutation was detected in four unrelated affected families of Lebanese origin. OTOF is the second member of a mammalian gene family related to Caenorhabditis elegans fer-1. It encodes a predicted cytosolic protein (of 1,230 aa) with three C2 domains and a single carboxy-terminal transmembrane domain. The sequence homologies and predicted structure of otoferlin, the protein encoded by OTOF, suggest its involvement in vesicle membrane fusion. In the inner ear, the expression of the orthologous mouse gene, mainly in the sensory hair cells, indicates that such a role could apply to synaptic vesicles. 相似文献
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Defects in whirlin,a PDZ domain molecule involved in stereocilia elongation,cause deafness in the whirler mouse and families with DFNB31 总被引:22,自引:0,他引:22
Mburu P Mustapha M Varela A Weil D El-Amraoui A Holme RH Rump A Hardisty RE Blanchard S Coimbra RS Perfettini I Parkinson N Mallon AM Glenister P Rogers MJ Paige AJ Moir L Clay J Rosenthal A Liu XZ Blanco G Steel KP Petit C Brown SD 《Nature genetics》2003,34(4):421-428
The whirler mouse mutant (wi) does not respond to sound stimuli, and detailed ultrastructural analysis of sensory hair cells in the organ of Corti of the inner ear indicates that the whirler gene encodes a protein involved in the elongation and maintenance of stereocilia in both inner hair cells (IHCs) and outer hair cells (OHCs). BAC-mediated transgene correction of the mouse phenotype and mutation analysis identified the causative gene as encoding a novel PDZ protein called whirlin. The gene encoding whirlin also underlies the human autosomal recessive deafness locus DFNB31. In the mouse cochlea, whirlin is expressed in the sensory IHC and OHC stereocilia. Our findings suggest that this novel PDZ domain-containing molecule acts as an organizer of submembranous molecular complexes that control the coordinated actin polymerization and membrane growth of stereocilia. 相似文献
4.
DMP1 mutations in autosomal recessive hypophosphatemia implicate a bone matrix protein in the regulation of phosphate homeostasis 总被引:14,自引:0,他引:14
Lorenz-Depiereux B Bastepe M Benet-Pagès A Amyere M Wagenstaller J Müller-Barth U Badenhoop K Kaiser SM Rittmaster RS Shlossberg AH Olivares JL Loris C Ramos FJ Glorieux F Vikkula M Jüppner H Strom TM 《Nature genetics》2006,38(11):1248-1250
Hypophosphatemia is a genetically heterogeneous disease. Here, we mapped an autosomal recessive form (designated ARHP) to chromosome 4q21 and identified homozygous mutations in DMP1 (dentin matrix protein 1), which encodes a non-collagenous bone matrix protein expressed in osteoblasts and osteocytes. Intact plasma levels of the phosphaturic protein FGF23 were clearly elevated in two of four affected individuals, providing a possible explanation for the phosphaturia and inappropriately normal 1,25(OH)2D levels and suggesting that DMP1 may regulate FGF23 expression. 相似文献
5.
Effect of the synthesis route on the structural and dielectric properties of SrBi<Subscript>1.8</Subscript>Y<Subscript>0.2</Subscript>Nb<Subscript>2</Subscript>O<Subscript>9</Subscript> ceramics
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Mohamed Afqir Amina Tachafine Didier Fasquelle Mohamed Elaatmani Jean-Claude Carru Abdelouahad Zegzouti Mustapha El Hammioui 《矿物冶金与材料学报》2018,25(11):1304-1312
This work is devoted to the synthesis and characterization of yttrium-doped SrBi2Nb2O9 ceramics prepared by three methods: solid state reaction, co-precipitation, and hydrothermal. Multiple characterizations, specifically scanning electron microscopy (SEM), X-ray powder diffraction (XRD), and Fourier transform infrared spectroscopy (FTIR), were used to validate the structural feature. The crystallite size was estimated by Scherrer’s formula and the Williamson–Hall plot. The effect of the process on the band intensities of the FTIR spectra was investigated. The crystallite size and microstructure of ceramics prepared from different synthesis processes were strongly influenced by the sinterability. SEM images revealed nanograin ceramics for materials prepared by co-precipitation and hydrothermal methods and micrograin ceramics prepared by the solid state method. The synthesized compounds underwent phase transitions at 480–465°C. The dielectric and electrical properties of these Y-doped SrBi2Nb2O9 ceramics appear to be dependent on the grain size. 相似文献
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Surfactant-decorated graphite nanoplatelets (GNPs) reinforced aluminum nanocomposites: sintering effects on hardness and wear
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Zeeshan Baig Othman Mamat Mazli Mustapha Asad Mumtaz Sadaqat Ali Mansoor Sarfraz 《矿物冶金与材料学报》2018,25(6):704-715
The exceptional properties of graphene make it ideal as a reinforcement to enhance the properties of aluminum matrices and this critically depends on uniform dispersion. In this study, the dispersion issue was addressed by sonication and non-covalent surface functionalization of graphite nanoplatelets (GNPs) using two types of surfactant: anionic (sodium dodecyl benzene sulfate (SDBS)) and non-ionic polymeric (ethyl cellulose (EC)). After colloidal mixing with Al powder, consolidation was performed at two sintering temperatures (550 and 620℃). The structure, density, mechanical and wear properties of the nanocomposite samples were investigated and compared with a pure Al and a pure GNPs/Al nanocomposite sample. Noticeably, EC-based 0.5wt% GNPs/Al samples showed the highest increment of 31% increase in hardness with reduced wear rate of 98.25% at 620℃, while a 22% increase in hardness with reduced wear rate of 96.98% at 550℃ was observed, as compared to pure Al. Microstructural analysis and the overall results validate the use of EC-based GNPs/Al nanocomposites as they performed better than pure Al and pure GNPs/Al nanocomposite at both sintering temperatures. 相似文献
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