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51.
Jen JC Chan WM Bosley TM Wan J Carr JR Rüb U Shattuck D Salamon G Kudo LC Ou J Lin DD Salih MA Kansu T Al Dhalaan H Al Zayed Z MacDonald DB Stigsby B Plaitakis A Dretakis EK Gottlob I Pieh C Traboulsi EI Wang Q Wang L Andrews C Yamada K Demer JL Karim S Alger JR Geschwind DH Deller T Sicotte NL Nelson SF Baloh RW Engle EC 《Science (New York, N.Y.)》2004,304(5676):1509-1513
The mechanisms controlling axon guidance are of fundamental importance in understanding brain development. Growing corticospinal and somatosensory axons cross the midline in the medulla to reach their targets and thus form the basis of contralateral motor control and sensory input. The motor and sensory projections appeared uncrossed in patients with horizontal gaze palsy with progressive scoliosis (HGPPS). In patients affected with HGPPS, we identified mutations in the ROBO3 gene, which shares homology with roundabout genes important in axon guidance in developing Drosophila, zebrafish, and mouse. Like its murine homolog Rig1/Robo3, but unlike other Robo proteins, ROBO3 is required for hindbrain axon midline crossing. 相似文献
52.
Rensing SA Lang D Zimmer AD Terry A Salamov A Shapiro H Nishiyama T Perroud PF Lindquist EA Kamisugi Y Tanahashi T Sakakibara K Fujita T Oishi K Shin-I T Kuroki Y Toyoda A Suzuki Y Hashimoto S Yamaguchi K Sugano S Kohara Y Fujiyama A Anterola A Aoki S Ashton N Barbazuk WB Barker E Bennetzen JL Blankenship R Cho SH Dutcher SK Estelle M Fawcett JA Gundlach H Hanada K Heyl A Hicks KA Hughes J Lohr M Mayer K Melkozernov A Murata T Nelson DR Pils B Prigge M Reiss B Renner T Rombauts S Rushton PJ 《Science (New York, N.Y.)》2008,319(5859):64-69
We report the draft genome sequence of the model moss Physcomitrella patens and compare its features with those of flowering plants, from which it is separated by more than 400 million years, and unicellular aquatic algae. This comparison reveals genomic changes concomitant with the evolutionary movement to land, including a general increase in gene family complexity; loss of genes associated with aquatic environments (e.g., flagellar arms); acquisition of genes for tolerating terrestrial stresses (e.g., variation in temperature and water availability); and the development of the auxin and abscisic acid signaling pathways for coordinating multicellular growth and dehydration response. The Physcomitrella genome provides a resource for phylogenetic inferences about gene function and for experimental analysis of plant processes through this plant's unique facility for reverse genetics. 相似文献
53.
Rare earth element (REE) data from two ordinary chondrite chondrules show distinct negative chondrite-normalized concentration anomalies of samarium, europium, and ytterbium. The peculiar patterns may be the result of REE gas/solid fractionation at an oxygen fugacity lower than has been assumed for the canonical solar nebula. We suggest that the two ordinary chondrite chondrules acquired the fractionated REE patterns by incorporation of highly reduced, ultrarefractory condensates in their precursors. This interpretation implies that high-temperature condensation processes occurred in nebular environments with a strong deficit in oxygen, such as regions with an enhanced carbon/oxygen ratio. 相似文献
54.
55.
Viscosity of fluids in subduction zones 总被引:2,自引:0,他引:2
The viscosities of aqueous fluids with 10 to 80 weight percent dissolved silicates have been measured at 600 degrees to 950 degrees C and 1.0 to 2.0 gigapascals by in situ observation of falling spheres in the diamond anvil cell. The viscosities at 800 degrees C range from 10(-4) to 10(0.5) pascal seconds. The combination of low viscosities with a favorable wetting angle makes silicate-rich fluid an efficient agent for material transport at low-volume fractions. Our results therefore suggest that there may be a direct relationship between the position of the volcanic front and the onset of complete miscibility between water and silicate melt in the subducting slab. 相似文献
56.
Hafezparast M Klocke R Ruhrberg C Marquardt A Ahmad-Annuar A Bowen S Lalli G Witherden AS Hummerich H Nicholson S Morgan PJ Oozageer R Priestley JV Averill S King VR Ball S Peters J Toda T Yamamoto A Hiraoka Y Augustin M Korthaus D Wattler S Wabnitz P Dickneite C Lampel S Boehme F Peraus G Popp A Rudelius M Schlegel J Fuchs H Hrabe de Angelis M Schiavo G Shima DT Russ AP Stumm G Martin JE Fisher EM 《Science (New York, N.Y.)》2003,300(5620):808-812
Degenerative disorders of motor neurons include a range of progressive fatal diseases such as amyotrophic lateral sclerosis (ALS), spinal-bulbar muscular atrophy (SBMA), and spinal muscular atrophy (SMA). Although the causative genetic alterations are known for some cases, the molecular basis of many SMA and SBMA-like syndromes and most ALS cases is unknown. Here we show that missense point mutations in the cytoplasmic dynein heavy chain result in progressive motor neuron degeneration in heterozygous mice, and in homozygotes this is accompanied by the formation of Lewy-like inclusion bodies, thus resembling key features of human pathology. These mutations exclusively perturb neuron-specific functions of dynein. 相似文献
57.
Mueller CA Broz P Müller SA Ringler P Erne-Brand F Sorg I Kuhn M Engel A Cornelis GR 《Science (New York, N.Y.)》2005,310(5748):674-676
Many pathogenic bacteria use injectisomes to deliver effector proteins into host cells through type III secretion. Injectisomes consist of a basal body embedded in the bacterial membranes and a needle. In Yersinia, translocation of effectors requires the YopB and YopD proteins, which form a pore in the target cell membrane, and the LcrV protein, which assists the assembly of the pore. Here we report that LcrV forms a distinct structure at the tip of the needle, the tip complex. This unique localization of LcrV may explain its crucial role in the translocation process and its efficacy as the main protective antigen against plague. 相似文献
58.
The extent to which synaptic activity can signal a sensory stimulus limits the information available to a neuron. We determined the contribution of individual synapses to sensory representation by recording excitatory postsynaptic currents (EPSCs) in cerebellar granule cells during a time-varying, quantifiable vestibular stimulus. Vestibular-sensitive synapses faithfully reported direction and velocity, rather than position or acceleration of whole-body motion, via bidirectional modulation of EPSC frequency. The lack of short-term synaptic dynamics ensured a highly linear relationship between velocity and charge transfer, and as few as 100 synapses provided resolution approaching psychophysical limits. This indicates that highly accurate stimulus representation can be achieved by small networks and even within single neurons. 相似文献
59.
Junt T Schulze H Chen Z Massberg S Goerge T Krueger A Wagner DD Graf T Italiano JE Shivdasani RA von Andrian UH 《Science (New York, N.Y.)》2007,317(5845):1767-1770
Platelets are generated from megakaryocytes (MKs) in mammalian bone marrow (BM) by mechanisms that remain poorly understood. Here we describe the use of multiphoton intravital microscopy in intact BM to visualize platelet generation in mice. MKs were observed as sessile cells that extended dynamic proplatelet-like protrusions into microvessels. These intravascular extensions appeared to be sheared from their transendothelial stems by flowing blood, resulting in the appearance of proplatelets in peripheral blood. In vitro, proplatelet production from differentiating MKs was enhanced by fluid shear. These results confirm the concept of proplatelet formation in vivo and are consistent with the possibility that blood flow-induced hydrodynamic shear stress is a biophysical determinant of thrombopoiesis. 相似文献
60.
Recent discourse in the field of participatory agricultural research has focused on how to blend various forms and intensities
of stakeholder participation with quality agricultural science, moving beyond the simple “farmer-first” ideology of the 1980s
and early 1990s. Yet, most existing frameworks of participation in agricultural research still adhere to a linear typology
of participatory research with an inherent claim of “the more participation, the better.” In this article, we propose a new
framework that looks at participatory research elements along different dimensions and attributes and thus takes into account
the diversity and dynamics of agricultural research projects. The framework provides a basis for agricultural researchers
engaged in participatory processes with local stakeholders to decide for which issues and in which phases certain participatory
elements could be used in a specific research context. Rather than aiming at maximizing the adoption of participatory methods,
it can thus become a tool for optimizing the use of participatory approaches in agricultural research. We conclude that this
framework can be a starting point for a more thoughtful integration of participatory elements in agricultural research projects
that does justice to the multidimensional and dynamic nature of stakeholder participation in varying contexts. 相似文献