A Gln747-->Pro substitution in the IIb subunit is responsible for a moderate IIbbeta3 deficiency in Glanzmann thrombasthenia

To clarify a molecular defect responsible for moderate alphaIIb beta3 deficiency, we examined two unrelated patients, MT and MS, suffering from type II and type I Glanzmann thrombasthenia (GT), respectively. Sequence analysis of polymerase chain reaction (PCR) fragments derived from platelet mRNA showed a single A-->C substitution at nucleotide (nt) 2334 leading to a Gln747--> Pro in alphaIIb in both patients. Allele-specific restriction enzyme analysis (ASRA) of genomic DNA demonstrated that patient MT was homozygous for the Gln747-->Pro substitution and patient MS was compound heterozygous for this substitution and for an RNA splice mutation at the consensus sequence of the splice acceptor site of exon 18 (AG-->AA). Furthermore, ASRA showed that, among 17 unrelated Japanese GT patients, this Gln747-->Pro substitution was detected in 4 patients, including MT and MS (homozygous, 2 patients; heterozygous, 2 patients). Cotransfection of Pro747alphaIIb and beta3 constructs into 293 cells resulted in moderate reduction in the amount of alphaIIb beta3 within the transfected cells as well as on the cell surface. However, Pro747alphaIIb beta3 bound the ligand mimetic monoclonal antibody (MoAb) PAC-1 after activation of alphaIIb beta3 by the MoAb PT25-2, suggesting that the mutant alphaIIb beta3 possesses the ligand-binding function. The association between the mutant proalphaIIb and beta3 was not disturbed. Surface labeling and pulse chase study showed that the Gln747-->Pro substitution moderately impaired both intracellular transport of the alphaIIb beta3 heterodimers to the Golgi apparatus and endoproteolytic cleavage of proalphaIIb into heavy and light chains. By contrast, replacement of Gln747 with Ala by mutagenesis did not impair alphaIIbbeta3 expression on the cell surface. These results suggest that the presence of Pro, rather than the absence of Gln, at amino acid residue 747 on alphaIIb is responsible for moderate alphaIIbbeta3 deficiency.     

Effect of solid/solution ratio on apatite formation from CaSiO<Subscript>3</Subscript> ceramics in simulated body fluid

The effect of the solid/solution (S/S) ratio on apatite formation from CaSiO₃ ceramics in simulated body fluid (SBF) was investigated. CaSiO₃ ceramics with a Ca/Si ratio of 0.91 were prepared by sintering CaSiO₃ powder coprecipitated from ethanol solutions of Ca(NO₃)₂⋅4H₂O and Si(OC₂H₅)₄ using NH₄OH as the precipitant. These ceramics were reacted with SBF at S/S ratios of 1.0, 2.5 and 8.3 mg/ml at 36.5 ^∘C for various times. Formation of apatite was observed at all the S/S ratios after soaking for 1 day. The amount and microstructure of the apatite obtained at a S/S ratio of 8.3 mg/ml, however, differed largely from the product formed at the other two S/S ratios. The apatite formed at S/S = 8.3 mg/ml was of smaller particle size, formed in smaller amount and with less preferred orientation of the (00l) of apatite crystals compared with those formed at S/S = 1.0 and 2.5 mg/ml. An increase of Ca and decrease of the P components occurred in the soaked SBF at S/S = 8.3 mg/ml, the changes being much more marked than with the other two S/S ratios. These differences in the concentration changes in SBF at different S/S ratios are attributed to the difference in the apatite formation from the CaSiO₃ ceramics.     

Positioning in Releasing Manipulation by Iterative Learning Control

In order to improve the positioning precision of the stop posture (position and orientation) of an object and decrease the trial numbers in our proposed releasing manipulation, two iterative learning control (ILC) schemes, learning control based on convergent condition (LCBCC), and learning control based on optimal principle (LCBOP) are designed in experimental-oriented way. These two methods are all based on a linearized system model. The experimental results show that these methods are effective. Having discussed the characteristics of these control methods, we conclude that in the case there is no enough system knowledge, LCBCC is the only choice to be used to learn the system knowledge; after the enough experience has been acquired, LCBOP is better than LCBCC, in the view of both of the convergent rate and the precision.     

Electrical Properties of (110)-Oriented Nondoped Mg2Si Films with p-Type Conduction Prepared by RF Magnetron Sputtering Method

Magnesium silicide (Mg₂Si) thick films with (110) orientation were fabricated on (001) sapphire substrate using radiofrequency magnetron sputtering. Stoichiometric Mg₂Si films with composition Si/(Mg + Si) = 0.33 were achieved over a range of vacuum from 10 mTorr to 140 mTorr and 300°C. On postannealing the film at 500°C, the out-of-plane lattice parameter shifted to lower values and the electrical conductivity increased by two orders of magnitude. A room-temperature Seebeck coefficient of 517 μV K^?1 was observed and found to decrease with increasing temperature; the Seebeck coefficient remained at a constant positive value of 212 μV K^?1 at 500°C. This can be related to the possibility of p-type conduction in this temperature range.     

Multi-disciplinary system decomposition of complex mechatronics systems

Modern mechatronics systems are increasingly becoming complex in terms of the size and multi-disciplinarity. System architecting of such multi-disciplinary systems defines subsystems and their interfaces through hierarchical system decomposition. As opposed to traditional system decomposition methods using building blocks associated with functions, this paper proposes a method to decompose a system by defining physical phenomena that constrain clustering patterns of system parameters. A case study of a printer demonstrates the method implemented on a computational tool, which supports systematic generation of decomposition candidates and configuration of sensor–actuator combinations based on the method.     

Lipid classes,fatty acid composition and triacylglycerol molecular species in the kernels of pumpkin (Cucurbita spp) seeds

The lipids extracted from the kernels of pumpkin (Cucurbita spp) seeds of three cultivars were classified by thin layer chromatography into six fractions: steryl esters (SEs, 0.5–1.2%), triacylglycerols (TAGs, 92.7–93.4%), free fatty acids (FFAs, 2.9–3.5%), sn‐1,3‐diacylglycerols (1,3‐DAGs, 0.4–0.9%), sn‐1,2‐diacylglycerols (1,2‐DAGs, 0.7–0.9%) and phospholipids (PLs, 1.5%). Fatty acids derivatised as methyl esters were analysed by gas chromatography with flame ionisation detection. Molecular species and fatty acid distributions of TAGs, isolated from the total lipids in the kernels, were analysed by a combination of argentation thin layer chromatography (TLC) and gas chromatography. A modified argentation TLC procedure, developed to optimise the separation of the complex mixture of total TAGs, provided 11 different groups of TAGs, based on both the degree of unsaturation and the total chain length of fatty acid groups. With a few exceptions, SM₂ (5.8–20.1%), S₂D (8.8–11.2%), M₃ (6.7–24.8%), SMD (6.8–16.7%), M₂D (16.7–23.6%), SD₂ (4.6–15.1%) and MD₂ (4.9–18.6%) were the main TAG components. These results suggest that there are significant differences (P < 0.05) not only in fatty acid distributions of acyl lipids but also in molecular species of TAGs among the three cultivars. The differences in pumpkin cultivars could be appreciable, based on the distribution of molecular species in TAGs. However, pumpkin seed kernels could be utilised successfully as a source of edible oils for human consumption. Copyright © 2004 Society of Chemical Industry     

Lipid classes,fatty acid composition and triacylglycerol molecular species of kidney beans (Phaseolus vulgaris L.)

Seed oils from five legume cultivars of Phaseolus vulgaris, grown in Japan, were extracted and classified by thin‐layer chromatography (TLC) into seven fractions: hydrocarbons (HC; 0.7–1.4 wt‐%), steryl esters (SE; 1.7–3.3 wt‐%), triacylglycerols (TAG; 33.8–45.9 wt‐%), free fatty acids (FFA; 0.6–1.5 wt‐%), sn‐1,3‐diacylglycerols (1,3‐DAG; 0.3–1.0 wt‐%), sn‐1,2‐diacylglycerols (1,2‐DAG; 0.4–1.2 wt‐%) and phospholipids (PL; 49.4–58.8 wt‐%). Fatty acids derivatized as methyl esters were analyzed by gas chromatography (GC) and a flame ionization detector. Molecular species and the fatty acid distribution of TAG isolated from the total lipids in the beans were analyzed by a combination of argentation‐TLC and GC. A modified argentation‐TLC procedure, developed to optimize the separation of the complex mixture of total TAG, provided 18 different groups of TAG, based on both the degree of unsaturation and the total length of the three acyl chains of fatty acid groups. SDT (3.2–4.2 wt‐%), M₂T (3.8–5.0 wt‐%), D₃ (4.8–5.9 wt‐%), MDT (8.0–13.9 wt‐%), D₂T (12.5–15.8 wt‐%), MT₂ (19.4–22.7 wt‐%), DT₂ (17.8–23.5 wt‐%) and T₃ (9.2–13.0 wt‐%) were the main TAG components. The dominant fatty acids of TAG were α‐linolenic (48.5–57.8 wt‐%) and linoleic (16.7–25.8 wt‐%) acids, with appreciable amounts of palmitic (8.3–13.2 wt‐%) and oleic (7.8–13.8 wt‐%) acids. The high content of α‐linolenic acid in the cultivars of P. vulgaris could very likely play a beneficial role in reducing the risk of coronary heart disease among the large populations consuming them in Japan.     

Difference of (Ca2+)i movements in platelets stimulated by thrombin and TRAP: the involvement of alpha(IIb)beta3-mediated TXA2 synthesis

This study investigated the difference of [Ca2+]i movement in platelets in response to thrombin and TRAP. The involvement of alpha(IIb)beta3 in this signaling was also studied. Stimulation of platelets with thrombin at 0.03 U/ml caused platelet aggregation and a two-peak increase in [Ca2+]i. The second peak of [Ca2+]i, but not the first peak was abolished by the inhibition of platelet aggregation with alpha(IIb)beta3 antagonists or by scavenging endogenous ADP with apyrase. A cyclooxygenase inhibitor, aspirin, and a TXA2 receptor antagonist, BM 13505, also abolished the second peak of [Ca2+]i but not the first peak, although these regents did not inhibit aggregation. Under the same assay conditions, measurement of TXB2 demonstrated that alpha(IIb)beta3 antagonists and aspirin almost completely inhibited the production of TXB2. In contrast to thrombin-stimulation, TRAP caused only a single peak of [Ca2+]i even in the presence of platelet aggregation, and a high level of [Ca2+]i increase was needed for the induction of platelet aggregation. The inhibition of aggregation with alpha(IIb)beta3 antagonists had no effect on [Ca2+]i change and TXB2 production induced by TRAP. Inhibition studies using anti-GPIb antibodies suggested that GPIb may be involved in the thrombin response, but not in the TRAP. Our findings suggest that low dose thrombin causes a different [Ca2+]i response and TXA2 producing signal from TRAP. Endogenous ADP release and fibrinogen binding to alpha(IIb)beta3 are responsible for the synthesis of TXA2 which results in the induction of the second peak of [Ca2+]i in low thrombin- but not TRAP-stimulated platelets.     

Residential load characteristics observed at KEPCO power system

Residential load characteristics are studied, and those load characteristics that are important for power system studies are identified. The results are used to determine the load model parameters and their statistical correlation to the load composition is presented separately