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31.
Roscioli T Kamsteeg EJ Buysse K Maystadt I van Reeuwijk J van den Elzen C van Beusekom E Riemersma M Pfundt R Vissers LE Schraders M Altunoglu U Buckley MF Brunner HG Grisart B Zhou H Veltman JA Gilissen C Mancini GM Delrée P Willemsen MA Ramadža DP Chitayat D Bennett C Sheridan E Peeters EA Tan-Sindhunata GM de Die-Smulders CE Devriendt K Kayserili H El-Hashash OA Stemple DL Lefeber DJ Lin YY van Bokhoven H 《Nature genetics》2012,44(5):581-585
Walker-Warburg syndrome (WWS) is an autosomal recessive multisystem disorder characterized by complex eye and brain abnormalities with congenital muscular dystrophy (CMD) and aberrant a-dystroglycan glycosylation. Here we report mutations in the ISPD gene (encoding isoprenoid synthase domain containing) as the second most common cause of WWS. Bacterial IspD is a nucleotidyl transferase belonging to a large glycosyltransferase family, but the role of the orthologous protein in chordates is obscure to date, as this phylum does not have the corresponding non-mevalonate isoprenoid biosynthesis pathway. Knockdown of ispd in zebrafish recapitulates the human WWS phenotype with hydrocephalus, reduced eye size, muscle degeneration and hypoglycosylated a-dystroglycan. These results implicate ISPD in a-dystroglycan glycosylation in maintaining sarcolemma integrity in vertebrates. 相似文献
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Lorena Beatriz Quiroga María Daniela Moreno Ariel Aníbal Cataldo Juan Héctor Aragón-Traverso María Victoria Pantano Juan Pablo Segundo Olivares 《Journal of Natural History》2015,49(27-28):1703-1716
The American bullfrog Lithobates catesbeianus has been introduced around the world, with invasive populations reported from almost all South American countries. A population of this species was introduced in the Calingasta department of San Juan province, which is an arid environment in western Argentina. This work provides information on the dietary composition of an invasive population of L. catesbeianus, and compares the degree of dietary overlap between adults and juveniles. Stomach contents of 169 bullfrogs (82 adults and 87 juveniles) were analysed. Adults consumed 40 prey taxa and Hymenoptera (Insecta) was the most numerous prey item (41.8%), followed by Araneae (13.6%) and Aeglidae (13.4%). Juveniles consumed 29 prey taxa and Hymenoptera constituted the highest percentage in prey number (77.2%). The trophic overlap niche index at the same level shows a value of 0.64 overlap in dietary community between adults and juveniles of this bullfrog. Aeglidae was volumetrically the most important trophic item (25.4%), followed by Anura (25.02%). Our results showed that cannibalism in bullfrogs is more common than the consumption of native anurans, coinciding with that reported in other populations of introduced bullfrogs. The high similarity in the diets of both size classes and the association between the size of the predator and prey suggest that the impact caused by bullfrogs throughout their ontogeny is high and probably has an impact on their prey. Freshwater crabs are the main items in the diet of Lithobates catesbeianus in other introduced populations and are usually the most conspicuous at our study site. The crabs in freshwater ecosystems are part of the lowest trophic level in the food chain. The major threats to the southern region’s freshwater crabs include deforestation, farming and exotic species. Lithobates catesbeianus has a generalist diet and high overlap between adults and juveniles. 相似文献
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以中国(大陆)为例,把12个公认的城市群空间分布与10大河流流域空间分布叠加,结果表明两点:一是流域与城市群形成了较好的空间对应关系;二是城市群的中心城市一般在流域的最佳区位. 相似文献
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The shallow-water tanaidacean fauna of the Bass Strait has been the subject of recent intensive studies. The present paper extends this work into the deeper waters of the region, describing two new species and one new genus. The new species of the genus Paradoxapseudes has a combination of three maxillule palp setae, no plumose setae on the basis of pereopod 1 nor proximal serration on the antennal peduncle. The second species represents a new genus of the family Anarthruridae, having six marginal setae on the third maxilliped palp article and spines on the merus and carpus of the anterior pereopods. The high diversity of Tanaidacea in Australian waters is discussed. In particular, we conclude that Australian coasts suffer a diversity of immigration routes, have sufficient marine longevity, and afford such a diversity of available niches to have allowed multiple colonization and subsequent allopatric speciation of Tanaidacea. http://www.zoobank.org/urn:lsid:zoobank.org:pub:EE309A5A-E06D-416F-95BD-4C8D0D2BEB97 相似文献
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This paper describes a distributed estimation scheme (DES) for a bandwidth constrained ad hoc sensor network.The DES is universal in the sense that operations on all sensors are identical and independent of noise distribution.The scheme requires each sensor to transmit just a 1-bit message per observation.Simulation results show that the scheme achieves much better mean-squares error (MSE) performance than the simplified isotropic universal DES and even outperforms the isotropic universal DES which requires more than twice the bandwidth of this scheme. 相似文献
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S Peña-Llopis S Vega-Rubín-de-Celis A Liao N Leng A Pavía-Jiménez S Wang T Yamasaki L Zhrebker S Sivanand P Spence L Kinch T Hambuch S Jain Y Lotan V Margulis AI Sagalowsky PB Summerour W Kabbani SW Wong N Grishin M Laurent XJ Xie CD Haudenschild MT Ross DR Bentley P Kapur J Brugarolas 《Nature genetics》2012,44(9):1072
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