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1.
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   
2.
A new method of efficiency maximization that utilizes sensing of the third-harmonic component of air-gap flux is proposed. This signal is used to determine the resulting instantaneous position of the fundamental component of the air gap flux and, consequently, the torque- and flux-producing components of the stator current. In addition, the third harmonic signal is also used to determine the rotor speed. Hence, the output power of the machine can be calculated with only a single sensor wire attached to the neutral point of the machine. The flux-producing component can be readily adjusted to produce the minimum input power for a fixed amount of output power (fixed speed)  相似文献   
3.
A new type of converter for variable-reluctance-machine (VRM) drives is described. In this converter topology, the energy extracted from an ongoing phase is stored in a dump capacitor. The energy stored is consequently used either to quickly turn on the next ongoing phase or to energize the conducting phase frequently during the conduction interval instead of being returned to the supply as for a conventional C-dump circuit. Since, the additional switch used to pass the energy to the C-dump capacitor is switched under a relatively-low-voltage condition and its switching frequency is relatively low, the rating of the additional switch is modest. The major advantage os this converter is that it uses a low-switching device/phase ratio while achieving better performance characteristics than the other topologies  相似文献   
4.
EGLE is a wide frequency band search- coil magnetometer designed and built at the Roma Tre University. It has been installed onboard the ISS by the Italian astronaut Roberto Vittori on April 25, 2005 within the LAZIO- EGLE experiment carried out during the ENEIDE Soyuz mission. The scope of the experiment is to test EGLE in space and to investigate geomagnetic field variations. The main applications of EGLE are the study of electromagnetic environment inside the ISS, the correlation of magnetic field data with particle fluxes detected by LAZIO particle detector, and the monitoring of ionospheric perturbations possibly caused by Earth seismic activity. Since continuous electromagnetic field measurements on board the ISS are important for diverse space applications, a magnetometer with a suitable design is requested. Appropriate solutions for these applications, which have been adopted by EGLE, are in particular the use of 1- Wire technology and the possibility to detect by means of a search- coil magnetometer a large portion of the ULF frequency band, usually measured by flux- gate probes. To investigate the topside ionosphere electromagnetic environment and stability of Van Allen radiation belts in relation with seismic and anthropogenic electromagnetic emissions, a specific satellite mission (the ESPERIA project) has been designed for the Italian Space Agency (ASI), and up to now a few instruments of its payload have been built and tested in space. One of them is exactly the EGLE search- coil magnetometer. The first magnetic observations performed by this instrument reveal to be promising and demand for a further and deeper analysis based on a longer time series of data.  相似文献   
5.
Formation of amylin-containing islet amyloid deposits may contribute to the progressive deterioration of beta cell function in non-insulin-dependent diabetes mellitus. As diabetes mellitus occurs in male, but rarely in female transgenic mice expressing human amylin in their pancreatic beta cells, it is of interest to study the influence of estradiol (E2) and testosterone (T) on amylin-induced cytotoxicity in human cells. The insulinoma cell line CM, thyroid epithelial cells (TEC) in primary culture, and nontransformed fibroblast lines were used. The occurrence of apoptotic cell death was assessed by nuclear labeling with propidium iodide. Amylin was cytotoxic on all cell types tested, but had the most pronounced effect on TEC and the weakest on the CM cell line. Although both E2 and T decreased the proportion of apoptotic cells in cultures kept in the absence of amylin, neither of the two hormones was able to counteract amylin-induced cytotoxicity. beta cell death and hyperglycemia can thus presumably not be prevented by the neutralization of amylin effects by sex steroids.  相似文献   
6.
PURPOSE: A large number of medical centers have recently instituted the use of High Dose-Rate Afterloading Brachytherapy (HDRAB). There is wide variation in treatment regimens, techniques, and dosimetry being used and there are no national standard protocols or guidelines for optimal therapy. METHODS AND MATERIALS: The Clinical Research Committee (CRC) of the American Endocurietherapy Society (AES) met to formulate consensus guidelines for HDRAB in cervical, endometrial, and endobronchial tumors. CONCLUSION: Each center is encouraged to follow a consistent treatment policy in a controlled fashion with complete documentation of treatment parameters and outcome including efficacy and morbidity. Until further clinical data becomes available, the linear quadratic model can be used as a guideline to formulate a new HDR regimen exercising caution when changing from a Low Dose Rate (LDR) to a HDRAB regimen. The treatments should be fractionated as much as practical to minimize long term morbidity. As more clinical data becomes available, the guidelines will mature and be updated by the Clinical Research Committee of the AES.  相似文献   
7.
Currently, there is little doubt that the immune system plays a role in the neurodegenerative process in Alzheimer's disease (AD). Inflammatory proteins such as complement components, enzymes, eicosanoids, and cytokines are found in association with cerebral amyloid plaques and may exacerbate the fundamental pathology of AD, by stimulating Amyloid beta (A beta) production, supporting its aggregation and increasing its cytotoxicity. Activated microglia and astrocytes are the main source of these proteins, and A beta may trigger their release. Interestingly, there are also indications that the immune system may play a protective role against the development of AD. Microglial cells have been shown to degrade A beta, and recent evidence suggests that autoreactive A beta-specific T cells may be relevant to the elimination of the peptide. This mechanism seems, however, impaired in the majority of patients with AD. The immune system seems thus to represent a natural line of defense against the accumulation of dangerous amyloidogenic substances. Impairment of this specific immunological defense mechanism and the failure to eliminate a toxic metabolite can be the basis for a chronic nonspecific inflammatory process in the brain, as described above. AD is a good example how an immune response initially aiming at maintaining the integrity of the body may fail and consequently lead to tissue destruction and neuronal loss.  相似文献   
8.
A cross-modal priming paradigm was used to examine the comprehension of metaphors varying in familiarity and aptness. In Exps 1 and 2, high-familiar metaphors showed availability of the figurative meaning, but low-familiar (LF) metaphors did not. In Exp 3, only LF metaphors that had been rated highly apt showed evidence of figurative activation. Exp 4 showed evidence of figurative activation for most LF and moderate-apt metaphors. The locus of activation was investigated in Exp 5, in which the individual words of the metaphor (topic and vehicle) served as primes. Neither topic nor vehicle showed evidence of priming the metaphor target, suggesting that activation of the metaphorical target in Exps 1–4 was not caused by lexical activation of the words within the metaphors, but rather was due to activation of emergent properties of the metaphorical phrase. (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   
9.
A new mathematical model of the power circuit of a three-phase voltage source converter (VSC) was developed in the stationary and synchronous reference frames. The mathematical model was then used to analyze and synthesize the voltage and current control loops for the VSC. Analytical expressions were derived for calculating the gains and time constants of the current and voltage regulators. The mathematical model was used to control a 140-kW regenerative VSC. The synchronous reference-frame model was used to define feedforward signals in the current regulators to eliminate the cross coupling between the d and q phases. It allowed the reduction of the current control loops to first-order plants and improved their tracking capability. The bandwidths of the current and voltage-control loops were found to be approximately 20 and 60 times (respectively) smaller than the sampling frequency. All control algorithms were implemented in a digital signal processor. All results of the analysis were experimentally verified  相似文献   
10.
Theoretical and numerical methods for deriving pressure–impulse (PI) diagrams for structural elements subjected to transient loads are described in this paper. Three different search algorithms for deriving PI diagrams numerically were developed by the authors and are presented. The PI diagrams of a linear elastic system under rectangular and triangular load pulses are derived using both theoretical and numerical methods and the results are compared. The application of these approaches to the behavior assessment of tested structural elements is illustrated.  相似文献   
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