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Slow-channel congenital myasthenic syndromes (SCCMSs) are rare genetic diseases caused by mutations in muscle nicotinic acetylcholine receptor (nAChR) subunits. Most of the known SCCMS-associated mutations localize at the transmembrane region near the ion pore. Only two SCCMS point mutations are at the extracellular domains near the acetylcholine binding site, α1(G153S) being one of them. In this work, a combination of molecular dynamics, targeted mutagenesis, fluorescent Ca2+ imaging and patch-clamp electrophysiology has been applied to G153S mutant muscle nAChR to investigate the role of hydrogen bonds formed by Ser 153 with C-loop residues near the acetylcholine-binding site. Introduction of L199T mutation to the C-loop in the vicinity of Ser 153 changed hydrogen bonds distribution, decreased acetylcholine potency (EC50 2607 vs. 146 nM) of the double mutant and decay kinetics of acetylcholine-evoked cytoplasmic Ca2+ rise (τ 14.2 ± 0.3 vs. 34.0 ± 0.4 s). These results shed light on molecular mechanisms of nAChR activation-desensitization and on the involvement of such mechanisms in channelopathy genesis.  相似文献   
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We describe the synthesis and the physical properties of polyaromatic hydrocarbons (PAHs) containing a phosphorus atom at the edge. In particular, the impact of the successive addition of aromatic rings on the electronic properties was investigated by experimental (UV/Vis absorption, fluorescence, cyclic voltammetry) and theoretical studies (DFT). The physical properties recorded in solution and in the solid state showed that the P‐containing PAHs exhibit properties expected for an emitter in white organic light‐emitting diodes (WOLEDs).  相似文献   
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We relate the Schramm–Loewner Evolution processes (SLE) to highest-weight representations of the Virasoro Algebra. The restriction properties of SLE that have been recently derived in [19] play a crucial role. In this setup, various considerations from conformal field theory can be interpreted and reformulated via SLE. This enables one to make a concrete link between the two-dimensional discrete critical systems from statistical physics and conformal field theory. To cite this article: R. Friedrich, W. Werner, C. R. Acad. Sci. Paris, Ser. I 335 (2002) 947–952.  相似文献   
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In response to a change of the Belgian National Directives whereby hospital laboratories became responsible for all point-of-care testing (POCT) performed within hospital walls a standardized and automated POC glucose-testing system was implemented in our hospital. The system consists of 50 AccuCheck Inform instruments (Roche Diagnostics, Vilvoorde, Belgium), 50 docking stations, a DataCare Server, and connections to the medical laboratory information system (MOLIS, Sysmex, Barchon, Belgium) and to the hospital information system. Implementation involved many parties and extensive preparation and communication. Key issues were bar-coded patient and user identification, training, and responsibilities. One year after the hospital wide implementation of this system the quality of POC glucose testing has significantly increased, thereby improving patient safety. This study describes a stepwise change over involving the medical laboratory and with a focus on hands-on quality.Presented at the ninth conference on Quality in the Spotlight, 18–19 March 2004, Antwerp, Belgium.  相似文献   
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We examine certain special features exhibited by various classes of linear operators acting in a hereditarily indecomposable Banach space. For instance, we show that the family of all Riesz operators in a H.I. space forms a closed, 2-sided ideal. We also give further characterizations of the class of scalar-type spectral operators (to those already given in [16]). The final section discusses some properties of the spectral maximal spaces of (necessarily decomposable) linear operators in such spaces. Conferenza tenuta il 16 settembre 1997 The support of the German Academic Exchange Scheme (DAAD) is gratefully acknowledged  相似文献   
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