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1.
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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Translated from Zhurnal Prikladnoi Spektroskopii, Vol. 55, No. 4, pp. 535–540, October, 1991.  相似文献   
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Dependences of the current and the chemical composition of cathodic deposits on the electrolysis time are studied in an ammonia-citrate electrolyte for the deposition of a nickel-molybdenum alloy in potentiostatic conditions. At potentials less negative than ?0.93 V, no nickel-molybdenum alloy deposition occurs and in the course of electrolysis there is observed a decrease in the current, which is connected with the blocking of the electrode surface by the formed molybdenum oxides. In the region of potentials of deposition of a nickel-molybdenum alloy there is realized the following shape of a time dependence of the current. Within the first few seconds of the electrolysis process, the current somewhat decreases. Then there occurs its increase, which is connected with the alloy deposition on the cathode. In the course of electrolysis there occurs a decrease in the molybdenum content in the cathodic deposit.  相似文献   
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A series of foamed organoplastics based on a combination of foamed fully aromatic polyimide binders and Arimid T polyimide felt were prepared. New composite foamed organoplastics reinforced with montmorillonite nanoparticles were prepared using commercially available aromatic monomers.  相似文献   
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Comprehensive self-consistent simulations of the positive column plasma of a dc oxygen discharge are performed with the help of commercial CFDRC software (), which enables one to carry out computations in an arbitrary 3D geometry using fluid equations for heavy components and a kinetic equation for electrons. The main scaling laws for the spatial distributions of charged particles are determined. These scaling laws are found to be quite different in the parameter ranges that are dominated by different physical processes. At low pressures, both the electrons and negative ions in the inner discharge region obey a Boltzmann distribution; as a result, a flat profile of the electron density and a parabolic profile of the ion density are established there. In the ion balance, transport processes prevail, so that ion heating in an electric field dramatically affects the spatial distribution of the charged particles. At elevated pressures, the volume processes prevail in the balance of negative ions and the profiles of the charged particle densities in the inner region turn out to be similar to each other.  相似文献   
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The process of electroforming (the production of a carboniferous conducting medium when the current flows through an organic material under a high electric field) in open sandwichlike structures with an insulating gap several tens of nanometers in width is considered. It is shown experimentally that there are factors that both favor (external ballast resistor) and prevent (local spreading resistance and the presence of the initial conductivity in the insulating gap) the production of a single conducting element between the electrodes. A simple model of the process in terms of the equivalent electric circuit is proposed. The model helps to find the trade-off between these factors and to construct an I-V diagram, which exhibits a region within which a single conducting nanostructure can be electroformed. An expression that relates the minimum permissible resistance of the nanostructure to its geometric parameters is derived.  相似文献   
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Results from kinetic and fluid simulations of the positive column plasma of a dc oxygen discharge are compared using commercial CFDRC software (), which enables one to perform numerical simulations in an arbitrary 3D geometry with the use of both the fluid equations for all the components (fluid model) and the kinetic equation for the electron energy distribution function (kinetic model). It is shown that, for both the local and nonlocal regimes of the formation of the electron energy distribution function (EEDF), the non-Maxwellian EEDF can satisfactorily be approximated by two groups of electrons. This allows one to take into account kinetic effects within the conventional fluid model in the simplest way by using the proposed two-temperature approximation of the nonequilibrium and nonlocal EEDF (2T fluid model).  相似文献   
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