Mutations in lectin complement pathway genes COLEC11 and MASP1 cause 3MC syndrome

3MC syndrome has been proposed as a unifying term encompassing the overlapping Carnevale, Mingarelli, Malpuech and Michels syndromes. These rare autosomal recessive disorders exhibit a spectrum of developmental features, including characteristic facial dysmorphism, cleft lip and/or palate, craniosynostosis, learning disability and genital, limb and vesicorenal anomalies. Here we studied 11 families with 3MC syndrome and identified two mutated genes, COLEC11 and MASP1, both of which encode proteins in the lectin complement pathway (collectin kidney 1 (CL-K1) and MASP-1 and MASP-3, respectively). CL-K1 is highly expressed in embryonic murine craniofacial cartilage, heart, bronchi, kidney and vertebral bodies. Zebrafish morphants for either gene develop pigmentary defects and severe craniofacial abnormalities. Finally, we show that CL-K1 serves as a guidance cue for neural crest cell migration. Together, these findings demonstrate a role for complement pathway factors in fundamental developmental processes and in the etiology of 3MC syndrome.     

Designing fast oxide-ion conductors based on La2Mo2O9

The ability of solid oxides to conduct oxide ions has been known for more than a century, and fast oxide-ion conductors (or oxide electrolytes) are now being used for applications ranging from oxide fuel cells to oxygen pumping devices. To be technologically viable, these oxide electrolytes must exhibit high oxide-ion mobility at low operating temperatures. Because of the size and interaction of oxygen ions with the cationic network, high mobility can only be achieved with classes of materials with suitable structural features. So far, high mobility has been observed in only a small number of structural families, such as fluorite, perovskites, intergrowth perovskite/Bi2O2 layers and pyrochlores. Here we report a family of solid oxides based on the parent compound La2Mo2O9 (with a different crystal structure from all known oxide electrolytes) which exhibits fast oxide-ion conducting properties. Like other ionic conductors, this material undergoes a structural transition around 580 degrees C resulting in an increase of conduction by almost two orders of magnitude. Its conductivity is about 6 x 10(-2) S cm(-1) at 800 degrees C, which is comparable to that of stabilized zirconia, the most widely used oxide electrolyte. The structural similarity of La2Mo2O9 with beta-SnWO4 (ref. 14) suggests a structural model for the origin of the oxide-ion conduction. More generally, substitution of a cation that has a lone pair of electrons by a different cation that does not have a lone pair--and which has a higher oxidation state--could be used as an original way to design other oxide-ion conductors.     

A Heuristic Algorithm for QoS Multicast Routing

In recent years, QoS multicast routing has continued to be a very important research topic in the areas of networks. This paper presents a heuristic algorithm for the QoS multicast routing (HAQMR). This heuristic algorithm deals with delay and bandwidth constraints and has low cost. The HAQMR attempts to significantly reduce the overhead for constructing a multicast tree, the proof for correctness of the HAQMR is given, and the performance of the HAQMR is evaluated by simulations. The study shows that HAQMR provides an available approach to QoS multicast routing.     

Design for the simulation of space based information network

1 .INTRODUCTIONNewcommunication network si mulations are nee-dedin space based networkto demonstrate ensuingadvances in global operations and distributed re-source management . Theseinclude full-scale si mu-lation of networking, protocols ,information dis-tribution,and display to lead tointegrated demon-strations of full-scale land-,air-,sea-,and space-based network having explicit modelling of net-work dynamics ,inter-satellite links (ISLs) , up-downlinks ( UDLs) ,schedulers ,protocols …     

PRELIMINARY STUDY ON RETRO-REFLECTIVE COATED PAPER BASED ON MICROGLASS BEADS

INTRODUCTIONThe micro-glass bead[1-2] is a new kind of silicate material, with good properties of chemical stability, mechanical strength as well as electro-resistance. When its diameter is below 0.08mm and the reflective index is in the range of 1.9~2.1, retro-reflective characteristics can be achieved. When a beam of ray from any direction irradiates the surface of the micro-glass beads, it can be converged at the special reflective layer consisted of focuses of micro-glass beads, owing …