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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.
Vipul Ranatunga Jay S. Gunasekera 《Journal of Materials Engineering and Performance》2006,15(1):47-52
The paper summarizes the development of numerical procedures for modeling bulk deformation process and preform designing techniques
based on the upper bound elemental technique (UBET). UBET has a unique place where an approximate, but faster solution is
needed for decision making. In designing and optimizing multistage forging and profile ring-rolling processes, an approximate
solution can be used to identify the most influential process parameters. Once an optimum combination of process conditions
are determined, computationally intensive, but more accurate finite element analysis can be used to verify and refine results.
In this paper, UBET procedures for closed-die forging and profile ring rolling are high-lighted. Experimental investigations
are used to validate the model predictions. Also, the UBET-based preform design tool is presented as a process and die design
tool for multistage forging processes. Application of these techniques is presented with evidence of effective material usage
and extended overall die-life. 相似文献
5.
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
6.
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
7.
Vipul Mathur Author Vitae Varsha Apte Author Vitae 《Journal of Systems and Software》2009,82(1):39-55
A Web server, when overloaded, shows a severe degradation of goodput initially, with the eventual settling of goodput as load increases further. Traditional performance models have failed to capture this behavior. In this paper, we propose an analytical model, which is a two-stage and layered queuing model of the Web server, which is able to reproduce this behavior. We do this by explicitly modelling the overhead processing, the user abandonment and retry behavior, and the contention for resources, for the FIFO and LIFO queuing disciplines. We show that LIFO provides better goodput in most overload situations. We compare our model predictions with experimental results from a test bed and find that our results match well with measurements. 相似文献
8.
This paper presents a new semi-blind reference watermarking scheme based on discrete wavelet transform(DWT) and singular value decomposition(SVD) for copyright protection and authenticity. We are using a gray scale logo image as watermark instead of randomly generated Gaussian noise type watermark. For watermark embedding, the original image is transformed into wavelet domain and a reference sub-image is formed using directive contrast and wavelet coefficients. We embed watermark into reference image by modifying the singular values of reference image using the singular values of the watermark. A reliable watermark extraction scheme is developed for the extraction of watermark from distorted image. Experimental evaluation demonstrates that the proposed scheme is able to withstand a variety of attacks. We show that the proposed scheme also stands with the ambiguity attack also. 相似文献
9.
Fractional advection–dispersion equation (FADE) is a generalization of the classical ADE in which the first order time derivative and first and second order space derivatives are replaced by Caputo derivatives of orders 0<α?1, 0<β?1 and 1<γ?2, respectively. We use Caputo definition to avoid (i) mass balance error, (ii) hyper-singular improper integral, (iii) non-zero derivative of constant, and (iv) fractional derivative involved in the initial condition which is often ill-defined. We present an analytic algorithm to solve FADE based on homotopy analysis method (HAM) which has the advantage of controlling the region and rate of convergence of the solution series via the auxiliary parameter ? over the variational iteration method (VIM) and homotopy perturbation method (HPM). We find that the proposed method converges to the numerical/exact solution of the ADE as the fractional orders α, β, γ tend to their integral values. Numerical examples are given to illustrate the proposed algorithm. Example 5 describes the intermediate process between advection and dispersion via Caputo fractional derivative. 相似文献
10.
OBSERVER: An Approach for Query Processing in Global Information Systems Based on Interoperation Across Pre-Existing Ontologies 总被引:3,自引:0,他引:3
Eduardo Mena Arantza Illarramendi Vipul Kashyap Amit P. Sheth 《Distributed and Parallel Databases》2000,8(2):223-271
There has been an explosion in the types, availability and volume of data accessible in an information system, thanks to the World Wide Web (the Web) and related inter-networking technologies. In this environment, there is a critical need to replace or complement earlier database integration approaches and current browsing and keyword-based techniques with concept-based approaches. Ontologies are increasingly becoming accepted as an important part of any concept or semantics based solution, and there is increasing realization that any viable solution will need to support multiple ontologies that may be independently developed and managed. In particular, we consider the use of concepts from pre-existing real world domain ontologies for describing the content of the underlying data repositories. The most challenging issue in this approach is that of vocabulary sharing, which involves dealing with the use of different terms or concepts to describe similar information. In this paper, we describe the architecture, design and implementation of the OBSERVER system. Brokering across the domain ontologies is enabled by representing and utilizing interontology relationships such as (but not limited to) synonyms, hyponyms and hypernyms across terms in different ontologies. User queries are rewritten by using these relationships to obtain translations across ontologies. Well established metrics like precision and recall based on the extensions underlying the concepts are used to estimate the loss of information, if any. 相似文献