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101.
Mutant dynactin in motor neuron disease   总被引:24,自引:0,他引:24  
Impaired axonal transport in motor neurons has been proposed as a mechanism for neuronal degeneration in motor neuron disease. Here we show linkage of a lower motor neuron disease to a region of 4 Mb at chromosome 2p13. Mutation analysis of a gene in this interval that encodes the largest subunit of the axonal transport protein dynactin showed a single base-pair change resulting in an amino-acid substitution that is predicted to distort the folding of dynactin's microtubule-binding domain. Binding assays show decreased binding of the mutant protein to microtubules. Our results show that dysfunction of dynactin-mediated transport can lead to human motor neuron disease.  相似文献   
102.
Kim J  Chung YD  Park DY  Choi S  Shin DW  Soh H  Lee HW  Son W  Yim J  Park CS  Kernan MJ  Kim C 《Nature》2003,424(6944):81-84
The many types of insect ear share a common sensory element, the chordotonal organ, in which sound-induced antennal or tympanal vibrations are transmitted to ciliated sensory neurons and transduced to receptor potentials. However, the molecular identity of the transducing ion channels in chordotonal neurons, or in any auditory system, is still unknown. Drosophila that are mutant for NOMPC, a transient receptor potential (TRP) superfamily ion channel, lack receptor potentials and currents in tactile bristles but retain most of the antennal sound-evoked response, suggesting that a different channel is the primary transducer in chordotonal organs. Here we describe the Drosophila Nanchung (Nan) protein, an ion channel subunit similar to vanilloid-receptor-related (TRPV) channels of the TRP superfamily. Nan mediates hypo-osmotically activated calcium influx and cation currents in cultured cells. It is expressed in vivo exclusively in chordotonal neurons and is localized to their sensory cilia. Antennal sound-evoked potentials are completely absent in mutants lacking Nan, showing that it is an essential component of the chordotonal mechanotransducer.  相似文献   
103.
以相转移催化法合成了单体二烯丙基双酚S醚(DABSE),采用FTIR,1H-NMR对单体结构进行了验证。DSC结果表明:DABSE的熔点为133℃,热聚合温度高达280℃,并具有较宽的聚合峰。  相似文献   
104.
Cultured mammotropes incubated with dopamine for one hour exhibited changes in ultrastructure indicative of actively depressed biosynthetic and secretory activity. Peripheral relocation of rough endoplasmic reticulum appeared to create a barrier to secretory granule release by exocytosis. A decrease in the numbers of secretory granules indicated a decrease in prolactin production and enhanced lysosomal activity.  相似文献   
105.
The primary metric for gauging progress in the various semiconductor integrated circuit technologies is the spacing, or pitch, between the most closely spaced wires within a dynamic random access memory (DRAM) circuit. Modern DRAM circuits have 140 nm pitch wires and a memory cell size of 0.0408 mum(2). Improving integrated circuit technology will require that these dimensions decrease over time. However, at present a large fraction of the patterning and materials requirements that we expect to need for the construction of new integrated circuit technologies in 2013 have 'no known solution'. Promising ingredients for advances in integrated circuit technology are nanowires, molecular electronics and defect-tolerant architectures, as demonstrated by reports of single devices and small circuits. Methods of extending these approaches to large-scale, high-density circuitry are largely undeveloped. Here we describe a 160,000-bit molecular electronic memory circuit, fabricated at a density of 10(11) bits cm(-2) (pitch 33 nm; memory cell size 0.0011 microm2), that is, roughly analogous to the dimensions of a DRAM circuit projected to be available by 2020. A monolayer of bistable, [2]rotaxane molecules served as the data storage elements. Although the circuit has large numbers of defects, those defects could be readily identified through electronic testing and isolated using software coding. The working bits were then configured to form a fully functional random access memory circuit for storing and retrieving information.  相似文献   
106.
黄河灌区水热通量的观测与分析   总被引:4,自引:0,他引:4  
为解决农田节水问题,进行了田间水热通量观测和水热耦合循环过程机制分析。观测系统主要包括:常规气象、辐射、涡度相关法观测系统以及土壤水分观测剖面。该系统能够连续和稳定地观测土壤-植物-大气连续体中的水热循环过程,涡度相关法观测数据具有与国际同类观测一致的精度。对2005年山东省位山引黄灌区冬小麦和夏玉米生长期内水热平衡的分析表明:当叶面积指数较大时潜热是农田能量消耗的主要方式;在冬小麦返青至收割期内作物耗水略小于供水,而在玉米生长期内供水远大于作物耗水,灌区供水充分。  相似文献   
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