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41.
The syndrome of hypoparathyroidism associated with growth retardation, developmental delay, and dysmorphism (HRD) is a newly described, autosomal recessive, congenital disorder with severe, often fatal consequences. Since the syndrome is very rare, with all parents of affected individuals being consanguineous, it is presumed to be caused by homozygous inheritance of a single recessive mutation from a common ancestor. To localize the HRD gene, we performed a genomewide screen using DNA pooling and homozygosity mapping for apparently unlinked kindreds. Analysis of a panel of 359 highly polymorphic markers revealed linkage to D1S235. The maximum LOD score obtained was 4.11 at a recombination fraction of 0. Analysis of three additional markers-GGAA6F06, D1S2678, and D1S179-in a 2-cM interval around D1S235 resulted in LOD scores >3. Analysis of additional chromosome 1 markers revealed evidence of genetic linkage disequilibrium and place the HRD locus within an approximately 1-cM interval defined by D1S1540 and D1S2678 on chromosome 1q42-43.  相似文献   
42.
Poor performance of livestock that graze tall fescue (Festuca arundinacea Schreb.) has been associated with the endophyte fungus Neotyphodium coenophialum [Morgan-Jones and Gams] Glenn, Bacon, and Hanlin). Recent evidence suggests lowered Cu status and a depression of Cu-related immune function in steers that graze endophyte-infected (E+) tall fescue. Greenhouse and field studies investigated relationships between the endophyte and Cu concentrations in tall fescue. Seventeen infected 'Kenhy' clones were divided, and one plant of each pair was treated three times with Benomyl to remove the endophyte (E-). Plants were watered with nutrient solution in a greenhouse for 6 mo before sampling. Copper concentrations were greater (P < .001) in E- than in E+ clones (3.4 vs 2.8 microg/g; SE, .06). In the second greenhouse experiment, genetically similar E+ and E- 'Kentucky'-31 (KY-31) and 'Georgia Jessup' were grown from seed and fertilized with nutrient solution to produce mature plants. Copper concentrations were higher (P < .05) in E- than in E+ tall fescue (8.6 vs 7.6 microg/g; SE, .3). In a field plot experiment in Texas, E+ and E- KY-31 were grown with 0, 50, and 100% replacement of potential evapotranspiration. By September, Cu concentrations were higher (P < .05) in E- than in E+ tall fescue (7.3 vs 6.6 microg/g; SE, .2). In pasture experiments, KY-31 E+ (> 70% infection level) and E- (< 5% infection level) tall fescue were grown in Virginia at two locations with three rates of N fertilizer. Copper concentrations were higher (P < .05) in E- than in E+ tall fescue (4.8 vs 4.5 microg/g; SE, .1) and increased (P < .01) linearly in response to N. Our data demonstrate that the presence of the endophyte is associated with lower Cu concentrations in tall fescue, which may contribute to lowered Cu status in animals and thus contribute to the etiology of fescue toxicity.  相似文献   
43.
A smart home usually has a variety of devices or home appliance, instead of designing software for a specific home, this paper proposes a service-oriented framework with a set of ontology systems to support service and device publishing, discovery of devices and their services, composition of control software using existing control services that wrap devices, deployment, and execution of the composed service in an computing environment, monitoring the execution, and recovery from device failure. The ontology systems specify semantic information about devices, services, and workflows used in various smart home, and users can compose and recompose services for their specific needs. New devices, workflows, and services can be added into ontology. Most of the steps in this process can be automated including code generation. For example, service composition will be carried out in three steps: abstract workflow design, function construction, and device discovery, and different codes can be generated for different computing platforms such as Java and Open Services Gateway initiative environments. In this way, a variety of smart home can be constructed rapidly using the framework by discovery and composition using existing services and workflows. This paper illustrates this framework using a media control example to illustrate the ontology, discovery, composition, deployment, execution, monitoring, and recovery.  相似文献   
44.
Due to the explosive increases of data from both the cyber and physical worlds, the demand for database support in embedded systems is increasing. Databases for embedded systems, or embedded databases, are expected to provide timely in situ data services under various resource constraints, such as limited energy. However, traditional buffer cache management schemes, in which the primary goal is to minimize the number of I/O operations, is problematic since they do not consider the constraints of modern embedded devices such as limited energy and distinctive underlying storage. In particular, due to asymmetric read/write characteristics of flash memory-based storage of modern embedded devices, minimum buffer cache misses neither coincide with minimum power consumption nor minimum I/O deadline misses. In this paper we propose a novel power- and time-aware buffer cache management scheme for embedded databases. A novel multi-dimensional feedback control architecture is proposed and the characteristics of underlying storage of modern embedded devices is exploited for the simultaneous support of the desired I/O power consumption and the I/O deadline miss ratio. We have shown through an extensive simulation that our approach satisfies both power and timing requirements in I/O operations under a variety of workloads while consuming significantly smaller buffer space than baseline approaches.  相似文献   
45.
Hypertension induces renal fibrosis or tubular interstitial fibrosis, which eventually results in end-stage renal disease. Epithelial-to-mesenchymal transition (EMT) is one of the underlying mechanisms of renal fibrosis. Though previous studies showed that Ecklonia cava extracts (ECE) and dieckol (DK) had inhibitory action on angiotensin (Ang) I-converting enzyme, which converts Ang I to Ang II. It is known that Ang II is involved in renal fibrosis; however, it was not evaluated whether ECE or DK attenuated hypertensive nephropathy by decreasing EMT. In this study, the effect of ECE and DK on decreasing Ang II and its down signal pathway of angiotensin type 1 receptor (AT1R)/TGFβ/SMAD, which is related with the EMT and restoring renal function in spontaneously hypertensive rats (SHRs), was investigated. Either ECE or DK significantly decreased the serum level of Ang II in the SHRs. Moreover, the renal expression of AT1R/TGFβ/SMAD was decreased by the administration of either ECE or DK. The mesenchymal cell markers in the kidney of SHRs was significantly decreased by ECE or DK. The fibrotic tissue of the kidney of SHRs was also significantly decreased by ECE or DK. The ratio of urine albumin/creatinine of SHRs was significantly decreased by ECE or DK. Overall, the results of this study indicate that ECE and DK decreased the serum levels of Ang II and expression of AT1R/TGFβ/SMAD, and then decreased the EMT and renal fibrosis in SHRs. Furthermore, the decrease in EMT and renal fibrosis could lead to the restoration of renal function. It seems that ECE or DK could be beneficial for decreasing hypertensive nephropathy by decreasing EMT and renal fibrosis.  相似文献   
46.
Simulated microgravity (SMG) induced the changes in cell proliferation and cytoskeleton organization, which plays an important factor in various cellular processes. The inhibition in cell cycle progression has been considered to be one of the main causes of proliferation inhibition in cells under SMG, but their mechanisms are still not fully understood. This study aimed to evaluate the effects of SMG on the proliferative ability and cytoskeleton changes of Chang Liver Cells (CCL-13). CCL-13 cells were induced SMG by 3D clinostat for 72 h, while the control group were treated in normal gravity at the same time. The results showed that SMG reduced CCL-13 cell proliferation by an increase in the number of CCL-13 cells in G0/G1 phase. This cell cycle phase arrest of CCL-13 cells was due to a downregulation of cell cycle-related proteins, such as cyclin A1 and A2, cyclin D1, and cyclin-dependent kinase 6 (Cdk6). SMG-exposed CCL-13 cells also exhibited a downregulation of α-tubulin 3 and β-actin which induced the cytoskeleton reorganization. These results suggested that the inhibited proliferation of SMG-exposed CCL-13 cells could be associate with the attenuation of major cell cycle regulators and main cytoskeletal proteins.  相似文献   
47.
不锈钢与铝合金薄壁管的摩擦焊   总被引:1,自引:1,他引:1       下载免费PDF全文
  相似文献   
48.
Oligomerization of glycine (gly) and diglycine (gly2) on montmorillonite was performed as cyclic, drying-wetting process at temperatures below 100°C, under varying reaction conditions. The influence of substrate/clay ratio, temperature and pH was found to be different for amino acid (AA) dimerization, cyclic anhydride (CA) formation and peptide chain elongation. High temperatures and neutral pH favour CA formation over diglycine production. An AA/catalyst ratio of 0.2 mmol/g leads to optimal yields. This supports the assumption that amino acid dimerization and CA formation take place at the edges of clay particles. Peptide chain elongation, starting from gly2, produces higher yields at higher temperatures and neutral pH.  相似文献   
49.
Novel polypyrrole (PPy)/polydimethylsiloxane (PDMS) composite membrane was prepared by interfacial polymerization to make a very effective gas separation membrane. We found that Polymerized PPy films as thin as ~200 nm could be chemically synthesized as freestanding membranes by using the interfacial polymerization technique. Additionally, we show that difference morphology of PPys films was obtained by controlling polymerization rate and more dense films were formed at low polymerization rate. Wide X-ray diffraction study showed the d spacing value of the PPy film decreased from 4.89 å to 3.67 å by the rate of polymerization decreases. According to d spacing value decrease, selectivity of a PPy composite membrane was increased dramatically and permeability was reduced gradually. This high selectivity was derived from d spacing closed to the kinetic diameter of nitrogen. These results indicated that the permeability is controlled by the diffusion coefficient, reflecting the packed structure of the PPy film. The highest selectivity value of composite membrane that was prepared by interfacial polymerization was O2/N2=17.2 and permeability for O2 was 40.2 barrer.  相似文献   
50.
This paper describes an improvement of the technique to measure interfacial tension in immiscible polymer blends. Our method is based on the droplet retraction method, in which one relates the kinetics of relaxation of a deformed droplet to the interfacial tension between the matrix and droplet. Previously, the problem with this technique has been the difficulty in preparing axisymmetric ellipsoidal droplets. In our work, we demonstrate that perfect axisymmetric ellipsoidal droplets are produced at a later stage of relaxation of short imbedded fibers. With this technique, we utilize the strengths of both the deformed droplet method and the imbedded fiber retraction method while overcoming their shortcomings. The interfacial tension value thus obtained was compared to that by conventional methods. Additionally, the effect of confinement by external walls on the interfacial tension measurement was studied. Confinement affects interfacial tension measurement when the gap between the walls is less than two times the equilibrium drop size.  相似文献   
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