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Izaddoost S  Nam SC  Bhat MA  Bellen HJ  Choi KW 《Nature》2002,416(6877):178-183
Drosophila Crumbs (Crb) is required for apical-basal polarity and is an apical determinant in embryonic epithelia. Here, we describe properties of Crb that control the position and integrity of the photoreceptor adherens junction and photosensitive organ, or rhabdomere. In contrast to normal photoreceptor adherens junctions and rhabdomeres, which span the depth of the retina, adherens junctions and rhabdomeres of Crb-deficient photoreceptors initially accumulate at the top of the retina and fail to maintain their integrity as they stretch to the retinal floor. We show that Crb controls localization of the adherens junction through its intracellular domain containing a putative binding site for a protein 4.1 superfamily protein (FERM). Although loss of Crb or overexpression of the FERM binding domain causes mislocalization of adherens junctions, they do not result in a significant loss of photoreceptor polarity. Mutations in CRB1, a human homologue of crb, are associated with photoreceptor degeneration in retinitis pigmentosa 12 (RP12) and Leber congenital amaurosis (LCA). The intracellular domain of CRB1 behaves similarly to its Drosophila counterpart when overexpressed in the fly eye. Our studies may provide clues for mechanisms of photoreceptor degeneration in RP12 and LCA.  相似文献   
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本文较深入地研究了脱硫石膏粉煤灰化学成分、杂质、颗粒特征和激发剂等对脱硫石膏、粉煤灰胶结材性能的影响 研究表明 ,激发剂是影响脱硫石膏、粉煤灰胶结材性能的主要因素 这为脱硫石膏、粉煤灰资源化利用打下良好理论基础 经实际应用表明 ,研究结果与实际情况能很好相符  相似文献   
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介绍用计算机软件产生正弦稳态扫频信号的编程方法。通过对D/A变换与A/D采集的同步输出可以获得不同频率量程和设定电压的扫频输出  相似文献   
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一类高阶非线性系统的零解稳定性   总被引:1,自引:0,他引:1  
由Барбащин公式得到的四阶常系数线性系统的Ляпунов函数出发,通过类比法构造了一类四阶非线性系统的Ляпунов函数,并由此得到了这些系统零解的全局渐近稳定性的充分条件  相似文献   
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Zhai RG  Zhang F  Hiesinger PR  Cao Y  Haueter CM  Bellen HJ 《Nature》2008,452(7189):887-891
Neurodegeneration can be triggered by genetic or environmental factors. Although the precise cause is often unknown, many neurodegenerative diseases share common features such as protein aggregation and age dependence. Recent studies in Drosophila have uncovered protective effects of NAD synthase nicotinamide mononucleotide adenylyltransferase (NMNAT) against activity-induced neurodegeneration and injury-induced axonal degeneration. Here we show that NMNAT overexpression can also protect against spinocerebellar ataxia 1 (SCA1)-induced neurodegeneration, suggesting a general neuroprotective function of NMNAT. It protects against neurodegeneration partly through a proteasome-mediated pathway in a manner similar to heat-shock protein 70 (Hsp70). NMNAT displays chaperone function both in biochemical assays and cultured cells, and it shares significant structural similarity with known chaperones. Furthermore, it is upregulated in the brain upon overexpression of poly-glutamine expanded protein and recruited with the chaperone Hsp70 into protein aggregates. Our results implicate NMNAT as a stress-response protein that acts as a chaperone for neuronal maintenance and protection. Our studies provide an entry point for understanding how normal neurons maintain activity, and offer clues for the common mechanisms underlying different neurodegenerative conditions.  相似文献   
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