Mutation of the matrix metalloproteinase 2 gene (MMP2) causes a multicentric osteolysis and arthritis syndrome.

The inherited osteolyses or 'vanishing bone' syndromes are a group of rare disorders of unknown etiology characterized by destruction and resorption of affected bones. The multicentric osteolyses are notable for interphalangeal joint erosions that mimic severe juvenile rheumatoid arthritis (OMIMs 166300, 259600, 259610 and 277950). We recently described an autosomal recessive form of multicentric osteolysis with carpal and tarsal resorption, crippling arthritic changes, marked osteoporosis, palmar and plantar subcutaneous nodules and distinctive facies in a number of consanguineous Saudi Arabian families. We localized the disease gene to 16q12-21 by using members of these families for a genome-wide search for homozygous-by-descent microsatellite markers. Haplotype analysis narrowed the critical region to a 1.2-cM region that spans the gene encoding MMP-2 (gelatinase A, collagenase type IV; (ref. 3). We detected no MMP2 enzymatic activity in the serum or fibroblasts of affected family members. We identified two family-specific homoallelic MMP2 mutations: R101H and Y244X. The nonsense mutation effects a deletion of the substrate-binding and catalytic sites and the fibronectin type II-like and hemopexin/TIMP2 binding domains. Based on molecular modeling, the missense mutation disrupts hydrogen bond formation within the highly conserved prodomain adjacent to the catalytic zinc ion.     

Type III (Arthus) allergic reactions and endotoxin toxicity in guinea-pigs.

Lipoplysaccharide (endotoxin) from E. coli cells produced lethal effects in guinea-pigs. Endotoxin caused no visible dermal change in normal animals, but produced skin reactions characterized by specific Arthus-type (Type III immune hypersensitivity) vascular inflammation in immunized animals. It is concluded that Arthus allergic reactions were evoked by endotoxin, however, endotoxin lethal toxicity appears independent of this process.     

声学多孔材料的孔结构优化

介绍了一种基于微结构的声学模型,可用来对多孔材料的微组织结构进行优化,从而使多孔材料具备最佳的声学性能.该模型是一种适用于低雷诺数的非稳态线性声学模型,主要微结构考虑平行排列的柱形杆件或圆球列阵,包括3个子模型:①声波传播方向平行于圆柱形杆件的子模型;②声波传播方向垂直于圆柱形杆件的子模型;③声波在圆球列阵中传播和吸收的子模型.前2个子模型通过考虑多边形周期边界条件的影响,计及了相邻圆柱形杆件间的交互作用.由于模型是线性的,因此可以结合起来描述任意角度入射声波的传播特性.第3个子模型可用于描述胞状多孔材料中节点处的情形.文中利用这3个子模型来扩大吸声材料的设计空间,根据不同用途所要求的声学特性计算最优的胞元结构,并对这种模型的应用领域进行了分析讨论.     

Ganglion cell dendrites are presynaptic in catfish retina

The retinal ganglion cells are third-order, spike-generating neurones whose axons transmit the output of the retina to the rest of the brain. It has long been believed that the dendrites of the retinal ganglion cells, like the dendrites of most other Golgi type I neurones, are only postsynaptic. Here we have studied the synapses made onto the ganglion cells in the catfish (Ictalurus punctatus), and we report that the distal dendrites of large-field ganglion cells make conventional chemical synapses onto other inner plexiform layer processes. We have also found that, more than 100 microns away from the cell perikaryon, the synapses made onto and by these ganglion cell dendrites are often tightly clustered. These synaptic clusters appear to be quite regularly spaced along the dendrites. Our results have important implications for the identification of ganglion cell dendrites within the inner plexiform layer as well as for the understanding of the ganglion cell response and receptive field generation.     

Type III (Arthus) allergic reactions and endotoxin toxicity in guinea-pigs

Summary Lipopolysaccharide (endotoxin) fromE. coli cells produced lethal effects in guinea-pigs. Endotoxin caused no visible dermal change in normal animals, but produced skin reactions characterized by specific Arthus-type (Type III immune hypersensitivity) vascular inflammation in immunized animals. It is concluded that Arthus allergic reactions were evoked by endotoxin, however, endotoxin lethal toxicity appears independent of this process.Acknowledgment. Supported by a grant from the National Health and Medical Research Council of Australia.     

多孔泡沫材料的声吸收特性

为了结合泡沫金属兼有的高吸声和高热传导两种特性以进一步提高其吸声性能,回顾了泡沫金属材料的应用和声学建模;通过对泡沫金属和用于制造泡沫金属的高分子基体材料的实验,比较了作者提出的声波通过泡沫金属传播的3种黏滞模型的预测结果,表明所有模型在泡沫金属典型胞元尺寸所对应的低雷诺数范围内是有效的(假定声波为线性,幅值低于160 dB).第一种模型考虑了声波沿平行于刚性圆柱束轴线方向传播时所受到的空气曳力,第二种模型考虑了声波沿垂直于刚性圆柱束轴线方向的传播,第三种模型考虑了声波通过球形节点的传播.结合这3种模型,提出了一种泡沫金属声学性能的综合模型,可以用来预测泡沫金属的声吸收特性.此外,还介绍了一种用于泡沫金属材料基本声传播特性实验的后处理技术.     

Centrifugal fibres synapse on dopaminergic interplexiform cells in the teleost retina

In teleost fish, centrifugal fibres originating in the olfactory bulb and containing FMRFamide-like and luteinizing hormone releasing hormone (LHRH)-like peptides project to the retina and terminate along the border of the inner nuclear and inner plexiform layers. Using a novel simultaneous two-colour immunolabelling technique, we have found that these centrifugal fibres are often closely apposed to the dopaminergic interplexiform cells. Contacts between centrifugal fibres and dopaminergic interplexiform cells were observed by electron microscopy to be conventional type synaptic junctions. Since the dopaminergic interplexiform cells make synapses on horizontal and bipolar cells, providing an intraretinal centrifugal pathway for information flow from the inner to the outer plexiform layers, we conclude that every neuron in the teleost retina is potentially susceptible to central influences via these centrifugal fibres and dopaminergic interplexiform cells.