CCDC103 mutations cause primary ciliary dyskinesia by disrupting assembly of ciliary dynein arms

Cilia are essential for fertilization, respiratory clearance, cerebrospinal fluid circulation and establishing laterality. Cilia motility defects cause primary ciliary dyskinesia (PCD, MIM244400), a disorder affecting 1:15,000-30,000 births. Cilia motility requires the assembly of multisubunit dynein arms that drive ciliary bending. Despite progress in understanding the genetic basis of PCD, mutations remain to be identified for several PCD-linked loci. Here we show that the zebrafish cilia paralysis mutant schmalhans (smh(tn222)) encodes the coiled-coil domain containing 103 protein (Ccdc103), a foxj1a-regulated gene product. Screening 146 unrelated PCD families identified individuals in six families with reduced outer dynein arms who carried mutations in CCDC103. Dynein arm assembly in smh mutant zebrafish was rescued by wild-type but not mutant human CCDC103. Chlamydomonas Ccdc103/Pr46b functions as a tightly bound, axoneme-associated protein. These results identify Ccdc103 as a dynein arm attachment factor that causes primary ciliary dyskinesia when mutated.     

Navigating the signalling network in mouse cardiac myocytes

Cardiac myocytes have a complex network of signals that regulates their essential role in the rhythmic pumping of the heart. This network is an appealing model system in which to study the basic principles underlying cellular signalling mechanisms. Progress in this effort has come through the establishment of standardized myocyte isolation and culture procedures and characterization of important signalling responses.     

声学多孔材料的孔结构优化

介绍了一种基于微结构的声学模型,可用来对多孔材料的微组织结构进行优化,从而使多孔材料具备最佳的声学性能.该模型是一种适用于低雷诺数的非稳态线性声学模型,主要微结构考虑平行排列的柱形杆件或圆球列阵,包括3个子模型:①声波传播方向平行于圆柱形杆件的子模型;②声波传播方向垂直于圆柱形杆件的子模型;③声波在圆球列阵中传播和吸收的子模型.前2个子模型通过考虑多边形周期边界条件的影响,计及了相邻圆柱形杆件间的交互作用.由于模型是线性的,因此可以结合起来描述任意角度入射声波的传播特性.第3个子模型可用于描述胞状多孔材料中节点处的情形.文中利用这3个子模型来扩大吸声材料的设计空间,根据不同用途所要求的声学特性计算最优的胞元结构,并对这种模型的应用领域进行了分析讨论.     

Aquatic invertebrate fauna of a northern prairie stream: range extensions and water quality characteristics

The benthic macroinvertebrate fauna of southern Saskatchewan, Canada, has received little attention relative to other regions of western North America. Therefore, little is known of the related aquatic ecosystem health and biogeography of regional aquatic insects. Here we present the results of an aquatic macroinvertebrate survey for the Pipestone Creek watershed in southeastern Saskatchewan. We qualitatively sampled aquatic macroinvertebrates in 5 sites on 4 dates through spring, summer, and fall 2006. Sampling produced 294 taxa of macroinvertebrates including 25 provincial range extensions to the southeast corner of the province. Presence/absence data of taxa grouped the sites into lentic and lotic sites. However, the relative proportions of the taxa varied greatly among sites, with no 2 sites having a community similarity greater than 50%. Functional feeding group analyses separated the sites into collector-dominated and scraper/grazer-dominated sites. However, the taxonomic make-up of the feeding groups varied among sites and also among dates. A modified Hilsenhoff Biotic Index of the site communities indicated that all were influenced by organic pollution. Results of this study suggest that although the watershed is enormously diverse, its biological communities are likely influenced by organic pollution. Further, range expansions of species found here, such as the stonefly Perlesta placida , have implications for invasion pathways and post-glaciation species islands in a prairie landscape.     

RanGTP mediates nuclear pore complex assembly

In metazoa, the nuclear envelope breaks down and reforms during each cell cycle. Nuclear pore complexes (NPCs), which serve as channels for transport between the nucleus and cytoplasm, assemble into the reforming nuclear envelope in a sequential process involving association of a subset of NPC proteins, nucleoporins, with chromatin followed by the formation of a closed nuclear envelope fenestrated by NPCs. How chromatin recruitment of nucleoporins and NPC assembly are regulated is unknown. Here we demonstrate that RanGTP production is required to dissociate nucleoporins Nup107, Nup153 and Nup358 from Importin beta, to target them to chromatin and to induce association between separate NPC subcomplexes. Additionally, either an excess of RanGTP or removal of Importin beta induces formation of NPC-containing membrane structures--annulate lamellae--both in vitro in the absence of chromatin and in vivo. Annulate lamellae formation is strongly and specifically inhibited by an excess of Importin beta. The data demonstrate that RanGTP triggers distinct steps of NPC assembly, and suggest a mechanism for the spatial restriction of NPC assembly to the surface of chromatin.