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Nephronophthisis (NPHP), an autosomal recessive cystic kidney disease, leads to chronic renal failure in children. The genes mutated in NPHP1 and NPHP4 have been identified, and a gene locus associated with infantile nephronophthisis (NPHP2) was mapped. The kidney phenotype of NPHP2 combines clinical features of NPHP and polycystic kidney disease (PKD). Here, we identify inversin (INVS) as the gene mutated in NPHP2 with and without situs inversus. We show molecular interaction of inversin with nephrocystin, the product of the gene mutated in NPHP1 and interaction of nephrocystin with beta-tubulin, a main component of primary cilia. We show that nephrocystin, inversin and beta-tubulin colocalize to primary cilia of renal tubular cells. Furthermore, we produce a PKD-like renal cystic phenotype and randomization of heart looping by knockdown of invs expression in zebrafish. The interaction and colocalization in cilia of inversin, nephrocystin and beta-tubulin connect pathogenetic aspects of NPHP to PKD, to primary cilia function and to left-right axis determination.  相似文献   
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Delta blues     
Clarke T 《Nature》2003,422(6929):254-256
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The on-going Three Gorges Project (TGP) aims to build one of the largest hydropower stations in the world. Previous investigations have concluded that the reservoir is likely to induce medium-to-intense earthquakes. 23 GPS stations have been established to maintain a geodetic and seismological monitoring network around the reservoir to monitor seismicity and crustal deformation, and to facilitate mitigation activities in case an intense earthquake occurs. The velocity of GPS site was derived from 3 repeated GPS surveys. Meanwhile, another effort has been made to investigate the feasibility of application of satellite interferometric synthetic aperture radar (InSAR) techniques in detecting crustal deformation possibly caused by impoundment of the Three Gorges Reservoir. It has been made to derive digital elevation models (DEM) using European Remote Sensing (ERS) synthetic aperture radar (SAR) imagery. Pairs of ERS SAR images in raw format are processed to generate digital elevation models. This effort will help us evaluate the accuracy of the InSAR techniques for further derivation of background deformation patterns and future detection of reservoir-induced crustal deformation in the area. Foundation item: Supported by the Seismological Union Foundation of China (602015), NASA grant of US(NAG5-9065) and Key Project of China Seismological Bureau(95-12-02) Biography: Qiao Xue-jun(1968-), male, Associated professor, research direction: crustal deformation.  相似文献   
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Dendrobaena veneta is one of the most widely used compost worms now distributed all over Europe and has been described under several synonymous names. Besides the typical stripy red specimens, another form bearing pinkish pigmentation also exists, mostly found in natural habitats in the East Mediterranean. Here we provide the first integrative taxonomic analysis of D. veneta, including some closely related species. Both the morphological and molecular results highly support the resurrection of D. succinta, a former synonym of D. veneta. In addition, we describe a peculiar new species, D. karacadagi sp. n. from Anatolia, and prove the highly homoplasious character of the cross section of the longitudinal musculature.

urn:lsid:zoobank.org:pub:336D3F7A-AEA1-465B-9096-03070C761A6A  相似文献   

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We study Federal Open Market Committee members' individual forecasts of inflation and unemployment in the period 1992–2004. Our results imply that Governors and Bank presidents forecast differently, with Governors submitting lower inflation and higher unemployment rate forecasts than bank presidents. For Bank presidents we find a regional bias, with higher district unemployment rates being associated with lower inflation and higher unemployment rate forecasts. Bank presidents' regional bias is more pronounced during the year prior to their elections or for nonvoting bank presidents. Career backgrounds or political affiliations also affect individual forecast behavior.  相似文献   
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The cardiovascular system developed early in evolution and is pivotal for the transport of oxygen, nutrients, and waste products within the organism. It is composed of hollow tubular structures and has a high level of complexity in vertebrates. This complexity is, at least in part, due to the endothelial cell lining of vertebrate blood vessels. However, vascular lumen formation by endothelial cells is still controversially discussed. For example, it has been suggested that the lumen mainly forms via coalescence of large intracellular vacuoles generated by pinocytosis. Alternatively, it was proposed that the vascular lumen initiates extracellularly between adjacent apical endothelial cell surfaces. Here we discuss invertebrate and vertebrate cardiovascular lumen formation and highlight the possible modes of blood vessel formation. Finally, we point to the importance of a better understanding of vascular lumen formation for treating human pathologies, including cancer and coronary heart disease.  相似文献   
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