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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   
3.
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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The spectral dynamics of a mid-infrared multimode Cr(2+):ZnSe laser located in a vacuum sealed chamber containing acetylene at low pressure is analyzed by a stepping-mode high-resolution time-resolved Fourier transform interferometer. Doppler-limited absorption spectra of C(2)H(2) in natural isotopic abundance are recorded around 4000 cm(-1) with kilometric absorption path lengths and sensitivities better than 3 10(-8) cm(-1). Two cold bands are newly identified and assigned to the ν(1)+ν(4) (1) and ν(3)+ν(5) (1) transitions of (12)C(13)CH(2). The ν(1)+ν(5) (1) band of (12)C(2)HD and fourteen (12)C(2)H(2) bands are observed, among which for the first time ν(2)+2ν(4) (2)+ν(5) (-1).  相似文献   
6.
We mourn the death of Professor Dr. Gilbert Grynberg, April 17, 1948 - January 27, 2003. We have lost an excellent and highly esteemed scientist, an outstanding character and a good friend. He has been a very active colleague in the merger of Zeitschrift für Physik and Journal de Physique in the early days. As one of the Co-Editors-in-Chief of The European Physical Journal D, from its start, he has formed the scienti.c standards of EPJ. Untiringly he has served our Journal with greatest dedication. We are grateful that he has been with us. The editorial board and o.ce of EPJ B Siegfried Grossmann, Denis Jérome, Antonio Paoletti  相似文献   
7.
High linear alkenes (1-octene and 1-decene) have been hydroformylated using water-soluble rhodium complexes associated with sulfonated diphosphines in the presence of ionic surfactants or methanol. In all cases, the hydroformylation activities were higher than in experiments without additives. The selectivity in aldehydes was higher when we used cetyltrimethylammonium hydrogensulfate (CTAHSO4) as the surfactant or methanol as the co-solvent.  相似文献   
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We consider an M/G/1 queue with symmetric service discipline. The class of symmetric service disciplines contains, in particular, the preemptive last-come-first-served discipline and the processor-sharing discipline. It has been conjectured in Kella et al. [1] that the marginal distribution of the queue length at any time is identical for all symmetric disciplines if the queue starts empty. In this paper we show that this conjecture is true if service requirements have an Erlang distribution. We also show by a counterexample, involving the hyperexponential distribution, that the conjecture is generally not true. AMS Subject Classifications Primary—60K25; Secondary—90B22  相似文献   
10.
The aim of this paper is to show that free Burnside groups of sufficiently large odd exponent are non-amenable in a certain strong sense. More precisely their left regular representations are isolated from the trivial representation uniformly on finite generating sets. It follows that free Burnside groups are of uniform exponential growth. This answers a question of de la Harpe. This work has been partially supported by the NSF grant DMS-0605093 and by the RFBR Grant # 05-01-00892. Received: 25 April 2006  相似文献   
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