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Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant disease characterized by neoplasia of the parathyroid glands, the endocrine pancreas, and the anterior pituitary gland. In addition, families with isolated endocrine neoplasia, notably familial isolated hyperparathyroidism (FIHP) and familial acromegaly, have also been reported. However, whether these families constitute MEN 1 variants or separate entities remains speculative as the genetic bases for these diseases are unclear. The gene for MEN 1 has recently been cloned and characterized. Using single strand conformation analysis (SSCA) and sequencing, we performed mutation analysis in: a) a total of 55 MEN 1 families from 7 countries, b) 13 isolated MEN 1 cases without family history of the disease, c) 8 acromegaly families, and d) 4 FIHP families. Mutations were identified in 27 MEN 1 families and 9 isolated cases. The 22 different mutations spread across most of the 9 translated exons and included frameshift (11), nonsense (6), splice (2), missense mutations (2), and in-frame deletions (1). Among the 19 Finnish MEN 1 probands, a 1466del12 mutation was identified in 6 families with identical 11q13 haplotypes and in 2 isolated cases indicating a common founder. One frameshift mutation caused by 359del4 (GTCT) was found in 1 isolated case and 4 kindreds of different origin and haplotypes; this mutation therefore represents a common "warm" spot in the MEN1 gene. By analyzing the DNA of the parents of an isolated case one mutation was confirmed to be de novo. No mutation was found in any of the acromegaly and small FIHP families, suggesting that genetic defects other than the MEN1 gene might be involved and that additional such families need to be analyzed.  相似文献   
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We report results on audio copy detection for TRECVID 2009 copy detection task. This task involves searching for transformed audio queries in over 385?h of test audio. The queries were transformed in seven different ways, three of them involved mixing unrelated speech to the original query, making it a much more difficult task. We give results with two different audio fingerprints and show that mapping each test frame to the nearest query frame (nearest-neighbor fingerprint) results in robust audio copy detection. The most difficult task in TRECVID 2009 was to detect audio copies using predetermined thresholds computed from 2008 data. We show that the nearest-neighbor fingerprints were robust to even this task and gave actual minimal normalized detection cost rate (NDCR) of around 0.06 for all the transformations. These results are close to those obtained by using the optimal threshold for each transform. This result shows the robustness of the nearest-neighbor fingerprints. These nearest-neighbor fingerprints can be efficiently computed on a graphics processing unit, leading to a very fast search.  相似文献   
5.
Kim D  Warde C  Vaccaro K  Woods C 《Applied optics》2003,42(19):3756-3764
A discrete-component approach was taken to establish the operational feasibility of a novel, imaging, midinfrared, multispectral, polarimetric sensor for remote-sensing application. The sensor is designed to exploit the spectral and polarimetric characteristics of the scene as discriminants. Pixelated multispectral filters and polarization filters were designed and fabricated on sapphire and Si substrates, respectively, and both were characterized. A single-pixel spectropolarimetric composite filter was characterized by use of a Fourier transform infrared spectrometer and a Pt-Si thermal-imaging camera. The experimental results show excellent agreement with theoretical predictions.  相似文献   
6.
When streaming packetized media data over a lossy packet network, it is desirable to use transmission strategies that minimize the expected distortion subject to a constraint on the expected transmission rate. Because the computation of such optimal strategies is usually an intractable problem, fast heuristic techniques are often used. We first show that when the graph that gives the decoding dependencies between the data packets is reducible to a tree, optimal transmission strategies can be efficiently computed with dynamic programming algorithms. The proposed algorithms are much faster than other exact algorithms developed for arbitrary dependency graphs. They are slower than previous heuristic techniques but can provide much better solutions. We also show how to apply our algorithms to find high-quality approximate solutions when the dependency graph is not tree reducible. To validate our approach, we run simulations for MPEG1 and H.264 video data. We first consider a simulated packet erasure channel. Then we implement a real video streaming system and provide experimental results for an Internet connection.  相似文献   
7.
One cm wide monolithic laser diode arrays emitting around 810 nm with a 2 mm total aperture width have been characterised under CW conditions. CW operation up to 16 W has been achieved at a heatsink temperature of 70 degrees C. Several arrays have been lifetested at 10 W CW at a 20 degrees C heatsink temperature for a few thousand hours and have projected lifetimes of between 5000 and 17000 hours. The temperature dependence of the degradation rate was characterised, from which data an activation energy of 0.2 eV was obtained.<>  相似文献   
8.
The aim of this study was to develop a bio-preservation strategy for cold-smoked salmon (CSS) by the use of lactic acid bacteria previously selected for their capability to inhibit the growth of Listeria monocytogenes in the product. The spoiling potential of three Carnobacterium strains (Carnobacterium divergens V41, Carnobacterium piscicola V1 and SF668) was tested in sterile CSS blocks inoculated by 10(4-5) CFU g(-)(1) and stored under vacuum for 9 days at 4 degrees C followed by 19 days at 8 degrees C. C. divergens V41 grew a little faster than the other strains and none of the three carnobacteria showed any adverse effect on quality of the product, i.e. no off-odour detected by a trained panel, no total volatile basic nitrogen (TVBN) production, no acidification and no biogenic amine except a slight production of tyramine. An application on commercial CSS was tested by spraying C. divergens V41 (10(4-5) CFU g(-1)) on slices of four batches freshly processed in different smoke-houses. Microbial, chemical and sensory characteristics were weekly compared to a control during 4 weeks of vacuum storage. When the natural microflora was initially weak (two batches<20 CFU g(-1)), C. divergens V41 quickly reached 10(7-8) CFU g(-1) and a slight inhibition of endogenous Enterobacteriaceae, lactobacilli and yeasts was observed. The presence of C. divergens V41 was slightly detected (odour and flavour) but none of the sample was considered as spoiled by the sensory panel. When the natural microflora was initially high (2 batches>10(4-5) CFU g(-1)), no effect on the microflora, TVBN and biogenic amine production, nor on the sensory characteristics was observed in presence of C. divergens V41. In conclusion, bio-preservation of CSS using lactic acid bacteria such as C. divergens V41 is a promising way to inhibit the growth of pathogenic bacteria such as L. monocytogenes with low effect on the quality of the product.  相似文献   
9.
The relationship between smoking parameters and odour characteristics, evaluated by a trained sensory panel, were studied on smoked herring. In addition, a possible correlation between the content of 10 phenolic compounds and sensory perceptions was investigated. Five smoking techniques were applied, combining smoke production conditions, performed by pyrolysis of beech wood sawdust or by friction of beech wood log, with smoke deposition, either in a controlled kiln (traditional smoking) or by an electrostatic process. In the fifth smoking technique, a purified condensate of beech smokes was vaporised on fish fillets in the smokehouse. The time of smoking was 3 h for traditional smoking and the liquid smoke atomisation process and 12 min for the electrostatic method. The effects of three smoking temperatures (16, 24, 32 °C) were tested for both the traditional and the liquid smoke atomisation processes, as well as the effect of the position of the exhaust valve in the smokehouse in the case of the traditional method. Two different voltages were applied for the electrostatic process, 37 and 42 kV.  相似文献   
10.
The readability levels of 10 articles each from the Journal of Health Education, Journal of Physical Education, Recreation and Dance, Research Quarterly for Exercise and Sport, and Strategies: A Journal for Physical and Sport Educators were estimated. On average, the 40 articles were written at the college junior level (M = 14.9, SD = 1.6); however, significant between-journal differences were found, with between-journal effect sizes ranging from 0.36 to 3.64.  相似文献   
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