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991.
992.
The authors described symptoms of Rubinstein-Taybi syndrome and presented a treatment of this disease. It concerned a 1-year-old baby with obstruction of left lacrimal ducts, psychomotor retardation and facial abnormalities. 相似文献
993.
CP Hayward GE Rivard WH Kane J Drouin S Zheng JC Moore JG Kelton 《Canadian Metallurgical Quarterly》1996,87(12):4967-4978
Multimerin is a massive soluble, multimeric protein found in platelets and endothelial cells. Recent studies identified multimerin as a specific coagulation factor V binding protein, complexed with platelet, but not plasma, factor V. These findings led us to investigate individuals with inherited factor V deficiencies for possible multimerin abnormalities. Platelet proteins were evaluated using immunoassays, sodium dodecyl sulfate-polyacrylamide gel electrophoresis, immunoblotting, immunoprecipitation, and direct binding studies. Patients with factor V Quebec, a disorder with abnormal platelet factor V, had a quantitative deficiency in multimerin (n = 11 tested; mean, 12.5%; range, 5% to 27% of the normal pool; normal range, 45% to 214%) with a normal multimer pattern. Quantitative and qualitative abnormalities were detected in their platelet factor V. An unrelated patient who was deficient in platelet and plasma factor V had normal platelet multimerin. The levels of platelet beta-thromboglobulin, von Willebrand factor, thrombospondin, and fibrinogen antigen were normal in the factor V Quebec patients. However, proteins with abnormal mobility were detected in their platelet lysate and releasate, and their platelet thrombospondin, von Willebrand factor, and fibrinogen showed evidence of proteolytic degradation. Platelet counts of the factor V Quebec patients ranged from mildly thrombocytopenic to low normal (mean, 159 x 10(9)/L; range, 104 to 198 x 10(9)/L). In addition, their platelets failed to aggregate in response to 6 to 10 micromol/L epinephrine despite normal numbers of platelet alpha 2-adrenergic receptors. These data indicate that patients with factor V Quebec have an inherited bleeding disorder distinct from other platelet disorders and associated with multiple abnormalities, including multimerin deficiency, abnormal platelet factor V, thrombospondin, von Willebrand factor, and fibrinogen, and an epinephrine aggregation defect. 相似文献
994.
The objectives of this study were to determine whether (a) students have self-insight into how they make overall evaluations of teaching effectiveness, and (b) there is a reasonably high level of consensus among the student raters in making overall evaluations. A policy-capturing approach was used with 3 different college student groups (94 introductory geology, 82 accounting, and 53 graduate education students) to determine the implicit and explicit weights of the teaching factors students used in arriving at their overall evaluations. The results indicated that the students had self-insight and a reasonably high level of consensus in making their overall evaluations, providing further evidence of the validity of students' overall evaluations of teaching effectiveness. (PsycINFO Database Record (c) 2010 APA, all rights reserved) 相似文献
995.
Thomas A. Moore Devens Gust Susan Hatlevig Ana L. Moore Lewis R. Makings Peter J. Pesski F.C. De Schryver M. van Der Auweraer Doris Lexa René V. Bensasson Michel Rougée 《Israel journal of chemistry》1988,28(2-3):87-95
Carotenoporphyrin-quinone triad molecules have previously been found to successfully mimic many aspects of photosynthetic electron and energy transfer. These molecules generate long-lived, energetic charge-separated states via a biomimetic multistep electron transfer scheme. In many of these molecules, the overall quantum yield for charge separation has been limited by an unfavorable partitioning of an intermediate charge-separated state between charge recombination and further reaction to yield the desired species. One strategy for overcoming this limitation is based on the fact that, in general, electron transfer rate constants do not depend linearly on reaction free energy change. In the triads, raising the energy of the intermediate charge-separated state would be expected to speed up the desired electron transfer (“normal” Marcus behavior), but slow down charge recombination (which formally lies in the “inverted” region). Laser flash photolysis and transient fluorescence measurements on two new triad molecules reveal enhanced quantum yields of long-lived charge-separated states which are consistent with this expectation. 相似文献
996.
Titanium aluminide compounds were synthesized by the thermal explosion mode of self-propagating high-temperature synthesis (SHS). The effects of heating rate on the combustion characteristics and the microstructures of the products were studied. It was found that the low density of the reacted sample was due to the outgassing of water vapour and other gases, which were released by dissociation of hydrated aluminium oxides. Higher heating rates resulted in a product of higher density and single-phase microstructure. At lower heating rates, the reaction product was a mixture of phases for TiAl and Ti3Al reactions. A liquid (Al)-solid (Ti) reaction mechanism is predicted for slow heating while a solid-solid mechanism is expected for high heating rates. The origin of porosity in the product is also discussed. 相似文献
997.
CM St Croix CA Harms SR McClaran GA Nickele DF Pegelow WB Nelson JA Dempsey 《Canadian Metallurgical Quarterly》1998,85(4):1556-1563
Insulin-like growth factor binding proteins (IGFBP) proteases have been proposed to be involved in changes of serum IGFBP pattern during pregnancy. IGFBP-4 and -5 are degraded specifically by proteases in pregnancy serum in vitro, whereas IGFBP-3 proteolytic activity was also detected in nonpregnancy serum. To identify and characterize IGFBP proteases, human pregnancy serum was fractionated by size exclusion chromatography revealing IGFBP-4 protease activities in fractions coeluting with proteins of approximately 600-kDa and 50- to 100-kDa molecular mass. In both fractions, a predominant 50-kDa gelatinase was found, suggesting that parts of the gelatinase activity might aggregate or are complexed with other proteins forming a higher molecular complex. Hydroxyapatite chromatography and chromatofocusing of the 50- to 100-kDa serum fraction showed that the IGFBP-4 protease and the 50-kDa gelatinase activity were copurified. When the 50-kDa gelatinase-containing band was excised from the polyacrylamide gel, it exhibited IGFBP-4 proteolytic activity, resulting in the formation of 17- and 10-kDa fragments. [125I] IGFBP substrate zymography combined with fragment blotting showed that the 1,10-phenanthroline-sensitive 50-kDa protease activity purified by chromatofocusing also cleaved IGFBP-3 and -5. Other proteases detected in pregnancy serum fractions with Mr estimates of 79-, 30-, and 22-kDa degraded IGFBP-3 and -5 but not IGFBP-4. [125I] IGFBP-5 substrate zymography revealed that the 30-kDa IGFBP protease was inhibited by serine protease inhibitors. Whereas 1,10-phenanthroline inhibited the IGFBP proteolytic activity in the solution assay, serine protease inhibitors failed to affect proteolysis, indicating the predominant contribution of the metalloproteinase to IGFBP proteolysis. Tissue inhibitors of matrix metalloproteinases-1 and -2 revealed weak or no inhibition of IGFBP-4 and -5 proteolytic activity, whereas a hydroxamic acid-based inhibitor, potentially inhibiting disintegrin metalloproteases, completely prevented the proteolysis of IGFBPs. Whereas no specific immunoreactivity of the 50-kDa protein with antimatrix metalloproteinase-1, -2, -3, -9, or -13 antibodies was observed, antidisintegrin domain-specific antibodies bound to the 50-kDa gelatinase. These studies provide the first direct biochemical evidence that human pregnancy serum contains a 50-kDa IGFBP protease with properties of a soluble disintegrin metalloproteinase that appears to be potentially involved in regulating IGF bioavailability for placental and fetal growth. 相似文献
998.
AG Zuccari Y Oshida M Miyazaki K Fukuishi H Onose BK Moore 《Canadian Metallurgical Quarterly》1997,7(5):345-355
In Part I, it was found that (i) 2 vol.% admixture of reinforcing elements in PMMA (Jet) resin matrix had a significant beneficial effects on the mechanical properties, and (ii) among these, zirconia exhibited the greatest improvements in modulus of elasticity, transverse strength, toughness, and hardness number. Using the best combination (i.e., PMMA resin matrix and 2 vol.% ZrO2), exothermic temperature raise and polymerization shrinkage were further investigated. Deterioration in mechanical properties due to prolonged water sorption were also studied for 5 weeks. The following can be concluded: (1) By increasing liquid/powder ratio for PMMA control samples, the peak temperature occurrence was retarded by 3 min and raised by 8 degrees C. (2) The effect of admixed oxide particles to PMMA resin matrix or the heat generated during polymerization was not significant. (3) The polymerization volumetric shrinkage was influenced by the a mixture of particles, with increases as large as 0.9% (or 0.3% in linear shrinkage). (4) PMMA resin admixed with 2 vol.% of zirconia particles showed a continuous weight gain due to water sorption, mechanical properties appears to be increasing up to 1-week sorption, followed by rapid drop of all properties. (5) Autopolymerizing acrylic resins are a resin-resin composite material of pre-polymerized beads embedded in a newly formed acrylic matrix. The main fracture modality appears to occur through the matrix and at the interface, although some trans-beads fractures were identified. (6) It was suggested that incorporating certain type of oxide particles into the pre-polymerized beads would provide stronger resin matrix. 相似文献
999.
由于成本提高和产品周期缩短,芯片开发者正致力于芯片设计的一次性成功。在芯片的设计过程中,制造商正在使用一些方法帮助设计者理解和实现面向制造(DFM)的设计技术。他们具备芯片效果、工艺细节、制造成本方面的知识,能够给设计者提供指导,帮助设计者提高产量并降低芯片成本。 相似文献
1000.
In order to investigate the relationship between the bioactive conformation of a peptide and its set of thermodynamically accessible structures in solution, the conformational profile of the tetrapeptide Ac-Pro-Ala-Pro-Tyr-OH was characterized by computational methods. Search of the conformational space was performed within the molecular mechanics frame-work using the AMBER4.0 force field with an effective dielectric constant of 80. Unique structures of the peptide were compared with its bioactive conformation for the protein Streptomyces griseus Protease A, as taken from the crystal structure of the enzyme-peptide complex. The results show that the bound conformation is close to one of the unique conformations characterized in the conformational search of the isolated peptide. Moreover, the lowest energy minimum characterized in the conformational search exhibits large deviations when compared to the bound conformation of the crystal structure. 相似文献