Mutations in SMAD3 cause a syndromic form of aortic aneurysms and dissections with early-onset osteoarthritis

Thoracic aortic aneurysms and dissections are a main feature of connective tissue disorders, such as Marfan syndrome and Loeys-Dietz syndrome. We delineated a new syndrome presenting with aneurysms, dissections and tortuosity throughout the arterial tree in association with mild craniofacial features and skeletal and cutaneous anomalies. In contrast with other aneurysm syndromes, most of these affected individuals presented with early-onset osteoarthritis. We mapped the genetic locus to chromosome 15q22.2-24.2 and show that the disease is caused by mutations in SMAD3. This gene encodes a member of the TGF-β pathway that is essential for TGF-β signal transmission. SMAD3 mutations lead to increased aortic expression of several key players in the TGF-β pathway, including SMAD3. Molecular diagnosis will allow early and reliable identification of cases and relatives at risk for major cardiovascular complications. Our findings endorse the TGF-β pathway as the primary pharmacological target for the development of new treatments for aortic aneurysms and osteoarthritis.     

<Emphasis Type="Italic">Dictyostelium</Emphasis> dynamin B modulates cytoskeletal structures and membranous organelles

Dictyostelium discoideum cells produce five dynamin family proteins. Here, we show that dynamin B is the only member of this group of proteins that is initially produced as a preprotein and requires processing by mitochondrial proteases for formation of the mature protein. Our results show that dynamin B-depletion affects many aspects of cell motility, cell-cell and cell-surface adhesion, resistance to osmotic shock, and fatty acid metabolism. The mature form of dynamin B mediates a wide range and unique combination of functions. Dynamin B affects events at the plasma membrane, peroxisomes, the contractile vacuole system, components of the actin-based cytoskeleton, and cell adhesion sites. The modulating effect of dynamin B on the activity of the contractile vacuole system is unique for the Dictyostelium system. Other functions displayed by dynamin B are commonly associated with either classical dynamins or dynamin-related proteins.     

New use of BCG for recombinant vaccines

BCG, a live attenuated tubercle bacillus, is the most widely used vaccine in the world and is also a useful vaccine vehicle for delivering protective antigens of multiple pathogens. Extrachromosomal and integrative expression vectors carrying the regulatory sequences for major BCG heat-shock proteins have been developed to allow expression of foreign antigens in BCG. These recombinant BCG strains can elicit long-lasting humoral and cellular immune responses to foreign antigens in mice.     

Phenotypic differences between alpha beta versus beta T-cell receptor transgenic mice undergoing negative selection

T-cell differentiation in the thymus is thought to involve a progression from the CD4-CD8- phenotype through CD4+CD8+ intermediates to mature CD4+ or CD8+ cells. There is evidence that during this process T cells bearing receptors potentially reactive to 'self' are deleted by a process termed 'negative selection' One example of this process occurs in mice carrying polymorphic Mls antigens, against which a detectable proportion of T cells are autoreactive. These mice show clonal deletion of thymic and peripheral T-cell subsets that express the autoreactive V beta 3 segment of the T-cell antigen receptor, but at most a two-fold depletion of thymic cells at the CD4+CD8+ stage. By contrast, transgenic mice bearing both alpha and beta chain genes encoding autoreactive receptors recognizing other ligands, show severe depletion of CD4+CD8+ thymocytes as well, suggesting that negative selection occurs much earlier. We report here the Mls 2a/3a mediated elimination of T cells expressing a transgene encoded V beta 3-segment, in T-cell receptor alpha/beta and beta-transgenic mice. Severe depletion of CD4+CD8+ thymocytes is seen only in the alpha/beta chain transgenic mice, whereas both strains delete mature V beta 3 bearing CD4+ and CD8+ T cells efficiently. We conclude that severe CD4+CD8+ thymocyte deletion in alpha/beta transgenic mice results from the premature expression of both receptor chains, and does not reflect a difference in the timing or mechanism of negative selection for Mls antigens as against the allo- and MHC class 1-restricted antigens used in the other studies.     

ZYS—2阻燃不饱和聚酯树脂的研究

本文论述了ＺＹＳ—２阻燃不饱和聚酯树脂的研制，该树脂采用溴素阻燃剂，并利用阻燃剂之间的协同作用，减少了阻燃剂添加量，并且提高了阻燃效果和阻燃材料的物理机械性能．     

Multiple ionization of atom clusters by intense soft X-rays from a free-electron laser

Intense radiation from lasers has opened up many new areas of research in physics and chemistry, and has revolutionized optical technology. So far, most work in the field of nonlinear processes has been restricted to infrared, visible and ultraviolet light, although progress in the development of X-ray lasers has been made recently. With the advent of a free-electron laser in the soft-X-ray regime below 100 nm wavelength, a new light source is now available for experiments with intense, short-wavelength radiation that could be used to obtain deeper insights into the structure of matter. Other free-electron sources with even shorter wavelengths are planned for the future. Here we present initial results from a study of the interaction of soft X-ray radiation, generated by a free-electron laser, with Xe atoms and clusters. We find that, whereas Xe atoms become only singly ionized by the absorption of single photons, absorption in clusters is strongly enhanced. On average, each atom in large clusters absorbs up to 400 eV, corresponding to 30 photons. We suggest that the clusters are heated up and electrons are emitted after acquiring sufficient energy. The clusters finally disintegrate completely by Coulomb explosion.     

新疆西天山西段金铜成矿带——中亚成矿域东延

新疆西天山西段位于哈萨克斯坦 -伊犁板块的东南部边缘 ,包括伊犁石炭纪裂谷、那拉提地块、哈尔克早古生代被动陆缘褶皱带。主要已知矿床 (点 )按其容矿围岩可分为镁铁 -超镁铁岩型、斑岩型、花岗岩型、矽卡岩型、陆相火山岩型、陆相沉积岩型、浅变质含碳碎屑岩型。根据区内成矿地质条件、矿化特征 ,区内以找金矿主 ,兼顾铜镍矿等。金矿以穆龙套型和火山岩型金矿为主 ,同时注意卡林型和中 -酸性侵入岩内外接触带型 ;铜矿以镁铁 -超镁铁岩型和火山岩型为主。地质与成矿条件与中亚成矿域具有可比性 ,因此 ,新疆西天山西段金铜成矿带应为中亚成矿域东延部分     

新疆昭苏水磨沟铜金矿特征

新疆西天山下石炭统大哈拉军山组地层中产有众多的金矿体。通过对昭苏水磨沟铜金矿床矿化特征的研究 ,总结出这类矿床的成矿规律是 :下石炭统大哈拉军山组中酸性火山岩是矿源层 ;区域性大断裂是矿质运移通道 ,其中的低级别、低序次构造为容矿空间 ;华力西期钾长花岗岩是形成铜金矿的能量来源 ;绿片岩相以下的动力变质作用是矿化富集的条件。矿床成因应属构造破碎蚀变岩型铜金矿床     

中国南方海相震旦系-中三叠统层序划分与对比

在对中国南方不同地区海相震旦系-中三叠统基干剖面和辅助剖面详细观测研究的基础上,对震旦系-中三叠统进行了系统的层序划分对比,共识别出13个超层序和99个三级层序,讨论了13个超层序的特征,建立了中国南方海相震旦系-中三叠统的层序格架.总体上看各超层序在整个中国南方不同盆地、不同相带的剖面上可以完全对比,但同一个超层序内所包含的三级层序个数及完整性有所不同.对于三级沉积层序来说,半数以上可以得到化石带较为准确的年代控制并在同一个构造分区甚至整个中国南方进行对比,部分层序甚至还可以在三级层序的体系域层次进行对比.表明从整个中国南方来看二级相对海平面升降变化更具有一致性,而三级相对海平面的变化规律在不同盆地、不同相带有所差异.