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111.
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
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Brains perform with remarkable efficiency, are capable of prodigious computation, and are marvels of communication. We are beginning to understand some of the geometric, biophysical, and energy constraints that have governed the evolution of cortical networks. To operate efficiently within these constraints, nature has optimized the structure and function of cortical networks with design principles similar to those used in electronic networks. The brain also exploits the adaptability of biological systems to reconfigure in response to changing needs. 相似文献
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Stimulated emission of radiation has not been directly observed in astrophysical situations up to this time. Here we demonstrate that photons from pulsar B1641-45 stimulate pulses of excess 1720-megahertz line emission in an interstellar hydroxyl (OH) cloud. As this stimulated emission is driven by the pulsar, it varies on a few-millisecond time scale, which is orders of magnitude shorter than the quickest OH maser variations previously detected. Our 1612-megahertz spectra are inverted copies of the 1720-megahertz spectra. This "conjugate line" phenomenon enables us to constrain the properties of the interstellar OH line-producing gas. We also show that pulsar signals undergo significantly deeper OH absorption than do other background sources, which confirms earlier tentative findings that OH clouds are clumpier on small scales than are neutral hydrogen clouds. 相似文献
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Many biological membranes adapt in response to environmental conditions. We investigated how the composition and architecture of photosynthetic membranes of a bacterium change in response to light, using atomic force microscopy. Despite large modifications in the membrane composition, the local environment of core complexes remained unaltered, whereas specialized paracrystalline light-harvesting antenna domains grew under low-light conditions. Thus, the protein mixture in the membrane shows eutectic behavior and can be mimicked by a simple model. Such structural adaptation ensures efficient photon capture under low-light conditions and prevents photodamage under high-light conditions. 相似文献
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Empathizing is the capacity to predict and to respond to the behavior of agents (usually people) by inferring their mental states and responding to these with an appropriate emotion. Systemizing is the capacity to predict and to respond to the behavior of nonagentive deterministic systems by analyzing input-operation-output relations and inferring the rules that govern such systems. At a population level, females are stronger empathizers and males are stronger systemizers. The "extreme male brain" theory posits that autism represents an extreme of the male pattern (impaired empathizing and enhanced systemizing). Here we suggest that specific aspects of autistic neuroanatomy may also be extremes of typical male neuroanatomy. 相似文献
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