Mutations in dynein link motor neuron degeneration to defects in retrograde transport

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.     

国产农用喷雾器喷头性能参数测定与分析

本文介绍了国产喷雾器喷头的几个主要性能参数与国外著名喷头公司产品的对比结果、并提出了改进我国喷头产品的若干建议。如:1.影响侧向进液式喷头喷量变异的因素。2.缝隙式扇雾喷头药液沉积横向分布中的牛角、对喷杆式喷雾器药液沉积分布均匀性有着极坏的影响。     

Superconductivity and the d-Shell

The various isotope effects on the superconducting transition temperature of Mo(2)B and W(2)B have been measured. Together with resistivity data they indicate that the d-shell forms a highly stable electron configuration for molybdenum and tungsten. This leads to an understanding of high superconducting transition temperatures.     

Physical mapping of a translocation breakpoint in neurofibromatosis

The gene for von Recklinghausen neurofibromatosis (NF1), one of the most common autosomal-dominant disorders of humans, was recently mapped to chromosome 17 by linkage analysis. The identification of two NF1 patients with balanced translocations that involved chromosome 17q11.2 suggests that the disease can arise by gross rearrangement of the NF1 locus, and that the NF1 gene might be identified by cloning the region around these translocation breakpoints. To further define the region of these translocations, a series of chromosome 17 Not I-linking clones has been mapped to proximal 17q and studied by pulsed-field gel electrophoresis. One clone, 17L1 (D17S133), clearly identifies the breakpoint in an NF1 patient with a t(1;17) translocation. A 2.3-megabase pulsed-field map of this region was constructed and indicates that the NF1 breakpoint is only 10 to 240 kilobases away from 17L1. This finding prepares the way for the cloning of NF1.     

Synchronized oscillation in coupled nanomechanical oscillators

We report measurements of synchronization in two nanomechanical beam oscillators coupled by a mechanical element. We charted multiple regions of frequency entrainment or synchronization by their corresponding Arnold's tongue diagrams as the oscillator was driven at subharmonic and rational commensurate frequencies. Demonstration of multiple synchronized regions could be fundamentally important to neurocomputing with mechanical oscillator networks and nanomechanical signal processing for microwave communication.