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1.
Dystrophin is a plasma membrane-associated cytoskeletal protein of the spectrin superfamily. The dystrophin cytoskeleton has been first characterized in muscle. Muscular 427 kDa dystrophin binds to subplasmalemmal actin filaments via its amino-terminal domain. The carboxy-terminus of dystrophin binds to a plasma membrane anchor, beta-dystroglycan, which is associated on the external side with the extracellular matrix receptor, alpha-dystroglycan, that binds to the basal lamina proteins laminin-1, laminin-2, and agrin. In the muscle, the dystroglycan complex is associated with the sarcoglycan complex that consists of several glycosylated, integral membrane proteins. The absence or functional deficiency of the dystrophin cytoskeleton is the cause of several types of muscular dystrophies including the lethal Duchenne muscular dystrophy (DMD), one of the most severe and most common genetic disorders of man. The dystrophin complex is believed to stabilize the plasma membrane during cycles of contraction and relaxation. Muscular dystrophin and several types of dystrophin variants are also present in extramuscular tissues, e.g. in distinct regions of the central nervous systems including the retina. Absence of dystrophin from these sites is believed to be responsible for some extramuscular symptoms of DMD, e.g. mental retardation and disturbances in retinal electrophysiology (reduced b-wave in electroretinograms). The reduced b-wave in electroretinograms indicated a disturbance of neurotransmission between photoreceptors and ON-bipolar cells. At least two different dystrophin variants are present in photoreceptor synaptic complexes. One of these dystrophins (Dp260) is virtually exclusively expressed in the retina. In the neuroretina, dystrophin is found in significant amounts in the invaginated photoreceptor synaptic complexes. At this location dystrophin colocalizes with dystroglycan. Agrin, an extracellular ligand of alpha-dystroglycan, is also present at this location whereas the proteins of the sarcoglycan complex appear to be absent in photoreceptor synaptic complexes. Dystrophin and dystroglycan are located distal from the ribbon-containing active synaptic zones where both proteins are restricted to the photoreceptor plasma membrane bordering on the lateral sides of the synaptic invagination. In addition, some neuronal profiles of the postsynaptic complex also contain dystrophin and beta-dystroglycan. These profiles appear to belong at least in part to projections of the photoreceptor terminals into the postsynaptic dendritic complex. In view of the abnormal neurotransmission between photoreceptors and ON-bipolar cells in DMD patients the dystrophin/beta-dystroglycan-containing projections of photoreceptor presynaptic terminals into the postsynaptic dendritic plexus might somehow modify the ON-bipolar pathway. Another retinal site associated with dystrophin/beta-dystropglycan is the plasma membrane of Müller cells where dystrophin/beta-dystroglycan appear to be present at particular high concentrations. At this location the dystrophin/dystroglycan complex may play a role in the attachment of the retina to the vitreous, and, under pathological conditions, in traction-induced retinal detachment. 相似文献
2.
S.K. Marik D.V.H. Rao A. Bhatnagar R.C. Pant A.C. Tikku S. Sankar 《Nuclear Engineering and Design》2006,236(7-8):730-746
Cirus, a 40 MW t, vertical tank type research reactor, having wide range of research facilities, was commissioned in the year 1960. This research reactor, situated at Mumbai, India has been operated and utilized extensively for isotope production, material testing and neutron beam research for nearly four decades. With a view to assess the residual life of the reactor, detailed ageing studies were carried out during the early 1990s. Based on these studies, refurbishment of Cirus for its life extension was taken up. During refurbishment, additional safety features were incorporated in various systems to qualify them for the current safety standards. This paper gives the details of the operating experiences, utilization of the reactor along with methodologies followed for carrying out detailed ageing studies, refurbishment and safety upgradation for its life extension. 相似文献
3.
The thermal degradation of cellulose and its phosphorylated products (phosphates, diethylphosphate, and diphenylphosphate) were studied in air and nitrogen by differential thermal analysis and dynamic thermogravimetry from ambient temperature to 750°C. From the resulting data various thermodynamic parameters were obtained following the methods of Broido and Freeman and Carroll. The values of Ea for decomposition for phosphorylated cellulose were found to be in the range 55–138 kJ mol?1 in air and 85–152 kJ mol?1 in nitrogen and depended upon the percent of phosphorus contents in the samples. The mass spectrum of cellobiose phosphate indicated the absence of the molecular ion, indicating that the compound was thermally unstable. The IR spectra of the pyrolysis residues of cellulose phosphate gave indication of formation of a compound having C?O and P?O groups. A fire retardancy mechanism for the thermal degradation of cellulose phosphate has been proposed. 相似文献
4.
Multifocus image fusion is the process of obtaining a single image from multiple partially focused images such that the newly formed image consists of the well-defined information extracted from each source image. This paper proposes the use of saliency of the source images based on Mutual Spectral Residual. Spectral Residual brings out the unique/salient features of an image in frequency domain. The idea of proposed mutual spectral residual is to emphasize the relative unique features of a source image with respect to the other source images. The relative unique features are utilized to form saliency maps for each source image. These saliency maps can clearly indicate the focused and defocused parts of an image. Based on the saliency maps obtained, the image fusion takes place in spatial domain. Visual inspection and quantitative evaluation of the fused images obtained by the proposed method, using different evaluation metrics, demonstrate its effectiveness over several existing image fusion methods. 相似文献
5.
A large body of research has considered the positive aspects of information technology (IT) use. However, emerging research and practice commentary is beginning to focus on complex and often alarming ways in which use of IT affects organisational and social life. This Special Issue focuses on these ‘dark’ effects of IT use. 相似文献
6.
This paper presents an application of a hybrid fuzzy multi-objective evolutionary algorithm (HFMOEA) for solving a highly constraint, mixed integer type, complex multi-objective reactive power market clearing (RPMC) problem for the competitive electricity market environment. In HFMOEA based multi-objective optimization approach, based on the output of a fuzzy logic controller crossover and mutation probabilities are varied dynamically. It enhances stochastic search capabilities of HFMOEA. In multi-objective RPMC optimization framework, two objective functions namely the total payment function (TPF) for reactive power support from generators and synchronous condensers and the total real transmission loss (TRTL) are minimized simultaneously for clearing the reactive power market. The proposed HFMOEA based multi-objective RPMC scheme is tested on a standard IEEE 24 bus reliability test system and its performance is compared with five other multi-objective evolutionary techniques such as MOPBIL, NSGA-II, UPS-EMOA and SPEA-2 and a new extended form of NSGA (ENSGA-II). Applying all these six evolutionary techniques, a detailed statistical analysis using T-test and boxplots is carried out on three performance metrics (spacing, spread and hypervolume) data for RPMC problem. The obtained simulation results confirm the overall superiority of HFMOEA to generate better Pareto-optimal solutions with higher convergence rate as compared to above mentioned algorithms. Further, TPF and TRTL values corresponding to the best compromise solutions are obtained using said multi-objective evolutionary techniques. These values are compared with one another to take better market clearing decisions in competitive electricity environment. 相似文献
7.
Given a graph with edges colored Red and Blue, we study the problem of sampling and approximately counting the number of matchings with exactly k
Red edges. We solve the problem of estimating the number of perfect matchings with exactly k
Red edges for dense graphs. We study a Markov chain on the space of all matchings of a graph that favors matchings with k
Red edges. We show that it is rapidly mixing using non-traditional canonical paths that can backtrack. We show that this chain
can be used to sample matchings in the 2-dimensional toroidal lattice of any fixed size ℓ with k
Red edges, where the horizontal edges are Red and the vertical edges are Blue.
An extended abstract appeared in J.R. Correa, A. Hevia and M.A. Kiwi (eds.) Proceedings of the 7th Latin American Theoretical Informatics Symposium, LNCS 3887, pp. 190–201, Springer, 2006.
N. Bhatnagar’s and D. Randall’s research was supported in part by NSF grants CCR-0515105 and DMS-0505505.
V.V. Vazirani’s research was supported in part by NSF grants 0311541, 0220343 and CCR-0515186.
N. Bhatnagar’s and E. Vigoda’s research was supported in part by NSF grant CCR-0455666. 相似文献
8.
Tripathi Kuldeep Narayan Yadav Ashish Mohan Sharma S. C. 《Wireless Personal Communications》2022,124(3):2475-2504
Wireless Personal Communications - This work aims to implement a clustering scheme to separate vehicles into a cluster that is based on various parameters, such as the total number of relay nodes,... 相似文献
9.
Constitutive relations and field equations have been extended for a porous medium composed of two solids and containing two chemically non-reactive immiscible fluids. By generalizing the closure relation of porosity change and employing this into the mass balance equations, the stress–strain relations have been developed. The idea of generalized compressibility tests is invoked to find the value of dimensionless parameters appearing in the closure relation of porosity change. By generalizing momentum balance equations of Lo et al. (Water Resour Res 41:1–20, 2005), the propagation of dilatational and rotational waves is explored. It is found that four dilatational and two rotational waves exist in the porous medium. In contrast to Biot’s theory, the presence of the second fluid and second solid in the porous medium gives rise to additional P- and S-waves. Variation of phase speeds and corresponding attenuation coefficients of existing waves versus frequency, saturation of the fluid phases and solid fraction are computed numerically and depicted graphically. 相似文献
10.
Tapan K. Sengupta Aditi Sengupta Soumyo Sengupta Ashish Bhole K. S. Shruti 《International Journal of Thermophysics》2016,37(4):36
Here, the fundamental problem of Rayleigh–Taylor instability (RTI) is studied by direct numerical simulation (DNS), where the two air masses at different temperatures, kept apart initially by a non-conducting horizontal interface in a 2D box, are allowed to mix. Upon removal of the partition, mixing is controlled by RTI, apart from mutual mass, momentum, and energy transfer. To accentuate the instability, the top chamber is filled with the heavier (lower temperature) air, which rests atop the chamber containing lighter air. The partition is positioned initially at mid-height of the box. As the fluid dynamical system considered is completely isolated from outside, the DNS results obtained without using Boussinesq approximation will enable one to study non-equilibrium thermodynamics of a finite reservoir undergoing strong irreversible processes. The barrier is removed impulsively, triggering baroclinic instability by non-alignment of density, and pressure gradient by ambient disturbances via the sharp discontinuity at the interface. Adopted DNS method has dispersion relation preservation properties with neutral stability and does not require any external initial perturbations. The complete inhomogeneous problem with non-periodic, no-slip boundary conditions is studied by solving compressible Navier–Stokes equation, without the Boussinesq approximation. This is important as the temperature difference between the two air masses considered is high enough (\(\Delta T = 70\) K) to invalidate Boussinesq approximation. We discuss non-equilibrium thermodynamical aspects of RTI with the help of numerical results for density, vorticity, entropy, energy, and enstrophy. 相似文献