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31.
本书是施普林格出版社2005年出版的一本关于螺旋压缩机的新著。螺旋式压缩机是一种最常见的用于压缩气体的机器,由于其构造简单、工作效率高而受到人们的关注。本书系统阐述了螺旋压缩机的种类、几何参数转子的设计工作原理和螺旋压缩机的计算。 相似文献
32.
B.布沙 《国外科技新书评介》2006,(7):10-10
“纳米材料”是指在纳米尺度(0.1~100nm范围,1nm=10^-9m)上研究物质的特征和相互作用,以及利用纳米尺度的特征开发新产品的一门科学和技术。纳米科技包含纳米材料、纳米器件和对它们的检测与表征等应用性很强的研究和技术领域。通常说的纳米检测和表征是指在纳米尺度上分析纳米结构材料和器件的组成、构造,并且进一步探索新现象,作为发展新的器件和功能材料的手段。 相似文献
33.
在无线通讯系统中,网络通过从数据库中查找用户信息来跟踪用户,这样就需要把用户信息事先存储在大量的数据库中。本书讨论了有关在数据库中复制用户信息并更新的若干问题。 相似文献
34.
本书全面介绍了数字通信系统的理论和实际应用方法,并且包含了在数字信号传输中需要运用的关于设计、实现和测试的最新方法和技术。此书介绍的基本技术有:纠错码、多路复用、数字调制、数字用户线路、电缆调制、移动无线网络、城域网和光纤网络。新的工业标准有:声音/视频编码、SONET/SDH、DWDM、ATM、宽带无线网、网络同步和网络管理。每一章节后都有评述和参考文献和大量习题(无答案)。 相似文献
35.
Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency 总被引:13,自引:0,他引:13
Irwin WA Bergamin N Sabatelli P Reggiani C Megighian A Merlini L Braghetta P Columbaro M Volpin D Bressan GM Bernardi P Bonaldo P 《Nature genetics》2003,35(4):367-371
Collagen VI is an extracellular matrix protein that forms a microfilamentous network in skeletal muscles and other organs. Inherited mutations in genes encoding collagen VI in humans cause two muscle diseases, Bethlem myopathy and Ullrich congenital muscular dystrophy. We previously generated collagen VI-deficient (Col6a1-/-) mice and showed that they have a muscle phenotype that strongly resembles Bethlem myopathy. The pathophysiological defects and mechanisms leading to the myopathic disorder were not known. Here we show that Col6a1-/- muscles have a loss of contractile strength associated with ultrastructural alterations of sarcoplasmic reticulum (SR) and mitochondria and spontaneous apoptosis. We found a latent mitochondrial dysfunction in myofibers of Col6a1-/- mice on incubation with the selective F1F(O)-ATPase inhibitor oligomycin, which caused mitochondrial depolarization, Ca2+ deregulation and increased apoptosis. These defects were reversible, as they could be normalized by plating Col6a1-/- myofibers on collagen VI or by addition of cyclosporin A (CsA), the inhibitor of mitochondrial permeability transition pore (PTP). Treatment of Col6a1-/- mice with CsA rescued the muscle ultrastructural defects and markedly decreased the number of apoptotic nuclei in vivo. These findings indicate that collagen VI myopathies have an unexpected mitochondrial pathogenesis that could be exploited for therapeutic intervention. 相似文献
36.
Chiang C Jacobsen JC Ernst C Hanscom C Heilbut A Blumenthal I Mills RE Kirby A Lindgren AM Rudiger SR McLaughlan CJ Bawden CS Reid SJ Faull RL Snell RG Hall IM Shen Y Ohsumi TK Borowsky ML Daly MJ Lee C Morton CC MacDonald ME Gusella JF Talkowski ME 《Nature genetics》2012,44(4):390-7, S1
We defined the genetic landscape of balanced chromosomal rearrangements at nucleotide resolution by sequencing 141 breakpoints from cytogenetically interpreted translocations and inversions. We confirm that the recently described phenomenon of 'chromothripsis' (massive chromosomal shattering and reorganization) is not unique to cancer cells but also occurs in the germline, where it can resolve to a relatively balanced state with frequent inversions. We detected a high incidence of complex rearrangements (19.2%) and substantially less reliance on microhomology (31%) than previously observed in benign copy-number variants (CNVs). We compared these results to experimentally generated DNA breakage-repair by sequencing seven transgenic animals, revealing extensive rearrangement of the transgene and host genome with similar complexity to human germline alterations. Inversion was the most common rearrangement, suggesting that a combined mechanism involving template switching and non-homologous repair mediates the formation of balanced complex rearrangements that are viable, stably replicated and transmitted unaltered to subsequent generations. 相似文献
37.
38.
Mutations in the gene encoding pejvakin, a newly identified protein of the afferent auditory pathway, cause DFNB59 auditory neuropathy 总被引:5,自引:0,他引:5
Delmaghani S del Castillo FJ Michel V Leibovici M Aghaie A Ron U Van Laer L Ben-Tal N Van Camp G Weil D Langa F Lathrop M Avan P Petit C 《Nature genetics》2006,38(7):770-778
Auditory neuropathy is a particular type of hearing impairment in which neural transmission of the auditory signal is impaired, while cochlear outer hair cells remain functional. Here we report on DFNB59, a newly identified gene on chromosome 2q31.1-q31.3 mutated in four families segregating autosomal recessive auditory neuropathy. DFNB59 encodes pejvakin, a 352-residue protein. Pejvakin is a paralog of DFNA5, a protein of unknown function also involved in deafness. By immunohistofluorescence, pejvakin is detected in the cell bodies of neurons of the afferent auditory pathway. Furthermore, Dfnb59 knock-in mice, homozygous for the R183W variant identified in one DFNB59 family, show abnormal auditory brainstem responses indicative of neuronal dysfunction along the auditory pathway. Unlike previously described sensorineural deafness genes, all of which underlie cochlear cell pathologies, DFNB59 is the first human gene implicated in nonsyndromic deafness due to a neuronal defect. 相似文献
39.
PLETHORA proteins as dose-dependent master regulators of Arabidopsis root development 总被引:1,自引:0,他引:1
Galinha C Hofhuis H Luijten M Willemsen V Blilou I Heidstra R Scheres B 《Nature》2007,449(7165):1053-1057
40.
A color based system using multiple templates was developed and implemented for detecting human faces in color images. The algorithm consists of three image processing steps. The first step is human skin color statistics. Then it separates skin regions from non-skin regions. After that, it locates the frontal human face(s) within the skin regions. In the first step, 250 skin samples from persons of different ethnicities are used to determine the color distribution of human skin in chromatic color space in order to get a chroma chart showing likelihoods of skin colors. This chroma chart is used to generate, from the original color image, a gray scale image whose gray value at a pixel shows its likelihood of representing the skin. The algorithm uses an adaptive thresholding process to achieve the optimal threshold value for dividing the gray scale image into separate skin regions from non skin regions. Finally, multiple face templates matching is used to determine if a given skin region represents a frontal human face or not. Test of the system with more than 400 color images showed that the resulting detection rate was 83%, which is better than most color-based face detection systems. The average speed for face detection is 0.8 second/image (400 x 300 pixels) on a Pentium 3 (800MHz) PC. 相似文献