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91.
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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An artificial crystal structure has been fabricated exhibiting a full three-dimensional photonic bandgap effect at optical communication wavelengths. The photonic crystal was constructed by stacking 0.7-micrometer period semiconductor stripes with the accuracy of 30 nanometers by advanced wafer-fusion technique. A bandgap effect of more than 40 decibels (which corresponds to 99.99% reflection) was successfully achieved. The result encourages us to create an ultra-small optical integrated circuit including a three-dimensional photonic crystal waveguide with a sharp bend.  相似文献   
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Cytokinin oxidase regulates rice grain production   总被引:25,自引:0,他引:25  
Most agriculturally important traits are regulated by genes known as quantitative trait loci (QTLs) derived from natural allelic variations. We here show that a QTL that increases grain productivity in rice, Gn1a, is a gene for cytokinin oxidase/dehydrogenase (OsCKX2), an enzyme that degrades the phytohormone cytokinin. Reduced expression of OsCKX2 causes cytokinin accumulation in inflorescence meristems and increases the number of reproductive organs, resulting in enhanced grain yield. QTL pyramiding to combine loci for grain number and plant height in the same genetic background generated lines exhibiting both beneficial traits. These results provide a strategy for tailormade crop improvement.  相似文献   
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Compact solid-state sources of terahertz (THz) radiation are being sought for sensing, imaging, and spectroscopy applications across the physical and biological sciences. We demonstrate that coherent continuous-wave THz radiation of sizable power can be extracted from intrinsic Josephson junctions in the layered high-temperature superconductor Bi2Sr2CaCu2O8. In analogy to a laser cavity, the excitation of an electromagnetic cavity resonance inside the sample generates a macroscopic coherent state in which a large number of junctions are synchronized to oscillate in phase. The emission power is found to increase as the square of the number of junctions reaching values of 0.5 microwatt at frequencies up to 0.85 THz, and persists up to approximately 50 kelvin. These results should stimulate the development of superconducting compact sources of THz radiation.  相似文献   
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A phase transition from a classical thermal mixed state to a quantum-mechanical pure state of exciton polaritons is observed in a GaAs multiple quantum-well microcavity from the decrease of the second-order coherence function. Supporting evidence is obtained from the observation of a nonlinear threshold behavior in the pump-intensity dependence of the emission, a polariton-like dispersion relation above threshold, and a decrease of the relaxation time into the lower polariton state. The condensation of microcavity exciton polaritons is confirmed.  相似文献   
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