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941.
Peutz-Jeghers syndrome (PJS, OMIM 175200) is an unusual inherited intestinal polyposis syndrome associated with distinct peri-oral
blue/black freckling [1–9]. Variable penetrance and clinical heterogeneity make it difficult to determine the exact frequency
of PJS [4]. PJS is a cancer predisposition syndrome. Affected individuals are at high risk for intestinal and extra-intestinal
cancers. In 1997, linkage studies mapped PJS to chromosome 19p [10, 11], and subsequently a serine/threonine kinase gene defect
(LKB1) was noted in a majority of PJS cases [12, 13]. A phenotypically similar syndrome has been produced in an LKB1 mouse
knockout model [14–18]. Several PJS kindred without LKB1 mutations have been described, suggesting other PJS loci [19–22].
The management of PJS is complex and evolving. New endoscopic technologies may improve management of intestinal polyposis.
Identification of specific genetic mutations and their targets will more accurately assess the clinical course, and help gage
the magnitude of cancer risk for affected individuals.
Received 20 February 2006; received after revision 5 May 2006; accepted 15 June 2006 相似文献
942.
The macromolecular peptide-loading complex in MHC class I-dependent antigen presentation 总被引:5,自引:1,他引:4
A challenging task for the adaptive immune system of vertebrates is to identify and eliminate intracellular antigens. Therefore
a highly specialized antigen presentation machinery has evolved to display fragments of newly synthesized proteins to effector
cells of the immune system at the cell surface. After proteasomal degradation of unwanted proteins or defective ribosome products,
resulting peptides are translocated into the endoplasmic reticulum by the transporter associated with antigen processing and
loaded onto major histocompatibility complex (MHC) class I molecules. Peptide-MHC I complexes are transported via the secretory
pathway to the cell surface where they are then inspected by cytotoxic T lymphocytes, which can trigger an immune response.
This review summarizes the current view of the intracellular machinery of antigen processing and of viral immune escape mechanisms
to circumvent destruction by the host.
Received 4 October 2005; received after revision 19 November 2005; accepted 24 November 2005 相似文献
943.
The mammalian olfactory system is not uniformly organized but consists of several subsystems each of which probably serves
distinct functions. Not only are the two major nasal chemosensory systems, the vomeronasal organ and the main olfactory epithelium,
structurally and functionally separate entities, but the latter is further subcompartimentalized into overlapping expression
zones and projection-related subzones. Moreover, the populations of ‘OR37’ neurons not only express a unique type of olfactory
receptors but also are segregated in a cluster-like manner and generally project to only one receptor-specific glomerulus.
The septal organ is an island of sensory epithelium on the nasal septum positioned at the nasoplatine duct; it is considered
as a ‘mini-nose’ with dual function. A specific chemosensory function of the most recently discovered subsystem, the so-called
Grueneberg ganglion, is based on the expression of olfactory marker protein and the axonal projections to defined glomeruli
within the olfactory bulb. This complexity of distinct olfactory subsystems may be one of the features determining the enormous
chemosensory capacity of the sense of smell. 相似文献
944.
Blasig IE Winkler L Lassowski B Mueller SL Zuleger N Krause E Krause G Gast K Kolbe M Piontek J 《Cellular and molecular life sciences : CMLS》2006,63(4):505-514
Tight junctions seal intercellular clefts via membrane-related strands, hence, maintaining important organ functions. We investigated
the self-association of strand-forming transmembrane tight junction proteins. The regulatory tight junction protein occludin
was differently tagged and cotransfected in eucaryotic cells. These occludins colocalized within the plasma membrane of the
same cell, coprecipitated and exhibited fluorescence resonance energy transfer. Differently tagged strand-forming claudin-5
also colocalized in the plasma membrane of the same cell and showed fluorescence resonance energy transfer. This demonstrates
self-association in intact cells both of occludin and claudin-5 in one plasma membrane. In search of dimerizing regions of
occludin, dimerization of its cytosolic C-terminal coiledcoil domain was identified. In claudin-5, the second extracellular
loop was detected as a dimer. Since the transmembrane junctional adhesion molecule also is known to dimerize, the assumption
that homodimerization of transmembrane tight junction proteins may serve as a common structural feature in tight junction
assembly is supported.
Received 6 October 2005; received after revision 14 December 2005; accepted 27 December 2005
†These authors contributed equally to this work. 相似文献
945.
The serine/threonine protein phosphatase 2A (PP2A) represents a large family of highly conserved heterotrimeric enzymes. Their
critical importance in cell homeostasis is underlined by the fact that they are targets of natural toxins like the tumor promoter
okadaic acid, and of simian virus 40 small tumor antigen (SV40 small t), a viral protein known to promote cell transformation.
Furthermore, mutated or lower expression levels of PP2A subunits have been found in certain cancers. One major known event
in PP2A-dependent cell transformation is the alteration of key signaling pathways that control cell growth and survival. In
this review, we focus on how PP2A enzymes also affect cell adhesion and cytoskeletal dynamics, the disruption of which is
linked to loss of cell polarity, increased cell motility and invasiveness. We also examine how those various pathways participate
in the transforming activity of SV40 small t.
Received 29 June 2006; received after revision 3 August 2006; accepted 20 September 2006 相似文献
946.
The coagulum proteins of human semen, semenogelins I and II, are secreted in abundance by the seminal vesicles. Their function
in reproduction is poorly understood as they are rapidly degraded in ejaculated semen. However, more recent results indicate
that it is time to put the semenogelins in a broader physiological perspective that goes beyond reproduction and fertility.
Received 21 June 2006; received after revision 16 August 2006; accepted 28 September 2006 相似文献
947.
Lehmann F Tiralongo E Tiralongo J 《Cellular and molecular life sciences : CMLS》2006,63(12):1331-1354
Sialic acids consist of a family of acidic ninecarbon sugars that are typically located at the terminal positions of a variety
of glycoconjugates. Naturally occurring sialic acids show an immense diversity of structure, and this reflects their involvement
in a variety of biologically important processes. One such process involves the direct participation of sialic acids in recognition
events through specific interactions with lectins, a family of proteins that recognise and bind sugars. This review will present
a detailed overview of our current knowledge regarding the occurrence, specificity and function of sialic acid-specific lectins,
particularly those that occur in viruses, bacteria and non-vertebrate eukaryotes.
Received 13 December 2005; received after revision 9 February 2006; accepted 15 February 2006 相似文献
948.
如何优化分配,合理调运,及时准确地完成后勤保障任务,是现代战争中战区物资调运面临的重要问题之一.基于运输问题,就现代战争条件下战区物资调运问题的各种情况建立了数学模型.该模型可用于后勤物资保障的决策支持系统及指挥自动化系统,为解决战时后勤保障供应问题提供了新的理论方法和思路. 相似文献
949.
在分析了现有基线扣除算法的基础上,根据非基线对称类基线信号的特征提出了一种新的基线扣除算法--基于小波滤波和梯度直方图最佳分割的非基线对称类信号基线扣除新算法.考虑到信号总是存在着噪声,含噪的基线信号梯度值也较大,并且分布无规律,采用了多分辨率小波变换滤波技术.通过合理选择最佳分割阈值门限,算法可精确提取出该类信号的基线. 相似文献
950.
引信全电子安全系统程序控制组件设计 总被引:3,自引:0,他引:3
全电子安全系统是引信设计的发展方向,程序控制组件是实现引信安全控制的重要部件.介绍了一种引信全电子安全系统程序控制组件的技术要求、基本功能和工作顺序及原理,提出了提高系统可靠性的电路模式和设计原则,同时对程序控制组件的软件设计方法进行了介绍.采用先进的在系统可编程器件,实现系统逻辑功能的集成化、小型化,且具有易于功能扩展、设计升级等优点. 相似文献