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.     

应用测力—落刀装置对积屑瘤、鳞刺的研究

本文研制了一种测力—落刀装置,对积屑瘤和鳞剌的某些问题进行了试验研究。结果表明:在积屑瘤存在的区域内,由于刀具实际前角的增加,以及切屑与积屑瘤的接触长度小于自然接触长度,从而导致了切削力的降低。积屑瘤不是整体稳定不变的,也不是高频地整体脱落后再重新生长。而是基体比较稳定,头部产生高频的分裂、成长,其分裂的部分留在工件表面成为鳞剌,同时,引起了切削力的较大波动。切削力动态分量越大,相应的鳞剌高度越高。     

最优受教育年限选择的模型分析

为进一步定量分析个人教育投资现象,构建了一般性地反映人们在就学和就业之间进行选择的最优化模型,并对影响人们教育投资决策行为的因素进行了分析.在简单的假定前提下运用数学语言再现了教育经济和劳动经济研究中的相关结论.     

Cu（Ⅱ） effect on the conformation of regenerated silk fibroin in dilute aqueous solution

Much attention has been paid to the natural mechanism of silkworm spinning due to the impressive mechanical properties of the natural fibers. In this work, we studied the effect of Cu（Ⅱ） ions on the secondary structure of Bombyx mori regenerated silk fibroin （SF） in dilute solution by circular dichroism （CD）. The results indicate that a given amount of Cu（Ⅱ） induces the SF conformational transition from random coil to β-sheet, however, further addition of Cu（Ⅱ） is unfavorable for this conversion. Meanwhile, the conformational changes induced by Cu（Ⅱ） follow a nucleation-dependent aggregation mechanism, which is similar to that found in Prion protein （PrP） denaturation and Aβ-peptide aggregations, leading to the neurodegenerative disease. This work would help one understand further the natural spinning process of silkworm. Additionally, it would be significant for the study of the nervous system diseases, because silk fibroin, extracted in large amounts from Bombyx mori silkworm gland, could be a proper model to study PrP denaturation and Aβ-peptide aggregations.     

Why the Afshar experiment does not refute complementarity

A modified version of Young's experiment by Shahriar Afshar demonstrates that, prior to what appears to be a “which-way” measurement, an interference pattern exists. Afshar has claimed that this result constitutes a violation of the Principle of Complementarity. This paper discusses the implications of this experiment and considers how Cramer's Transactional Interpretation easily accommodates the result. It is also shown that the Afshar experiment is analogous in key respects to a spin one-half particle prepared as “spin up along x”, subjected to a nondestructive confirmation of that preparation, and post-selected in a specific state of spin along z. The terminology “which-way” or “which-slit” is critiqued; it is argued that this usage by both Afshar and his critics is misleading and has contributed to confusion surrounding the interpretation of the experiment. Nevertheless, it is concluded that Bohr would have had no more problem accounting for the Afshar result than he would in accounting for the aforementioned pre- and post-selection spin experiment, in which the particle's preparation state is confirmed by a nondestructive measurement prior to post-selection. In addition, some new inferences about the interpretation of delayed choice experiments are drawn from the analysis.     

分布交互仿真环境研究

分布交互仿真是一种新兴的仿真技术，分布交互仿真环境的研究具有重人的理论意义和实用价值。本文分析了分布交互仿真环境的功能需求，指出了它的设计原则。     

茴香酸乙酯的合成

从茴香油生产茴香醛的废渣、废液中提取副产品茴香酸，合成茴香酸乙酯。对合成条件进行了研究，取得了满意的结果。     

树与色树

给出了色树的两种性质；（１）色树相关联的两个顶点对应的串是色分划的不同色组对应的串；（２）色树顶点的对应串所含链的上等。并应用构造方法证明了任意树都是以树，从而解决了遗留问题。     

Using genetically-defined rodent strains for the identification of hippocampal traits relevant for two-way avoidance behavior: a non-invasive approach

Summary Genetically-defined rodent strains permit the identification of hippocampal traits which are of functional relevance for the performance of two-way avoidance behavior. This is exemplified here by analyzing the relationship between infrapyramidal mossy fibers (a tiny projection terminating upon the basal dendrites of hippocampal pyramidal neurons) and two-way avoidance learning in about 800 animals. The necessary steps include 1) identification of structural traits sensitive to selective breeding for extremes in two-way avoidance, 2) testing the robustness of the associations found by studying individual and genetical correlations between hippocampal traits and behavior, 3) establishing causal relationships by Mendelian crossing of strains with extreme structural traits and studying the behavioral consequences of such structural randomization, 4) confirming causal relationships by manipulating the structural variable in inbred (isogenic) strains, thereby eliminating the possibility of genetic linkage, and 5) ruling out the possibility of spurious associations by studying the correlations between the hippocampal trait and other behaviors known to depend on hippocampal functioning.In comparison with the classical lesion approach for identifying relationships between brain and behavior, the present procedure appears to be superior in two aspects: it is non-invasive, and it focuses automatically on those brain traits which are used by natural selection to shape behaviorally-defined animal populations, i.e., it reveals the natural regulators of behavior.