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361.
Erik J. Bonten Ida Annunziata Alessandra d’Azzo 《Cellular and molecular life sciences : CMLS》2014,71(11):2017-2032
The ubiquitous distribution of lysosomes and their heterogeneous protein composition reflects the versatility of these organelles in maintaining cell homeostasis and their importance in tissue differentiation and remodeling. In lysosomes, the degradation of complex, macromolecular substrates requires the synergistic action of multiple hydrolases that usually work in a stepwise fashion. This catalytic machinery explains the existence of lysosomal enzyme complexes that can be dynamically assembled and disassembled to efficiently and quickly adapt to the pool of substrates to be processed or degraded, adding extra tiers to the regulation of the individual protein components. An example of such a complex is the one composed of three hydrolases that are ubiquitously but differentially expressed: the serine carboxypeptidase, protective protein/cathepsin A (PPCA), the sialidase, neuraminidase-1 (NEU1), and the glycosidase β-galactosidase (β-GAL). Next to this ‘core’ complex, the existence of sub-complexes, which may contain additional components, and function at the cell surface or extracellularly, suggests as yet unexplored functions of these enzymes. Here we review how studies of basic biological processes in the mouse models of three lysosomal storage disorders, galactosialidosis, sialidosis, and GM1-gangliosidosis, revealed new and unexpected roles for the three respective affected enzymes, Ppca, Neu1, and β-Gal, that go beyond their canonical degradative activities. These findings have broadened our perspective on their functions and may pave the way for the development of new therapies for these lysosomal storage disorders. 相似文献
362.
Dealing with Distances and Transformations for Fuzzy C-Means Clustering of Compositional Data 总被引:1,自引:0,他引:1
Javier Palarea-Albaladejo Josep Antoni Martín-Fernández Jesús A. Soto 《Journal of Classification》2012,29(2):144-169
Clustering techniques are based upon a dissimilarity or distance measure between objects and clusters. This paper focuses on the simplex space, whose elements??compositions??are subject to non-negativity and constant-sum constraints. Any data analysis involving compositions should fulfill two main principles: scale invariance and subcompositional coherence. Among fuzzy clustering methods, the FCM algorithm is broadly applied in a variety of fields, but it is not well-behaved when dealing with compositions. Here, the adequacy of different dissimilarities in the simplex, together with the behavior of the common log-ratio transformations, is discussed in the basis of compositional principles. As a result, a well-founded strategy for FCM clustering of compositions is suggested. Theoretical findings are accompanied by numerical evidence, and a detailed account of our proposal is provided. Finally, a case study is illustrated using a nutritional data set known in the clustering literature. 相似文献
363.
天牛(鞘翅目Coleoptera:天牛科Cerambycidae)为树木重要的钻蛀性害虫.本文研究了伊朗西北部Arasbaran生物圈保护区及邻近地区的天牛种类,采集鉴定了5个亚科26属33种,提供了一些种类的植物寄主. 相似文献
364.
Otávio Bueno Steven French James Ladyman 《Studies in History and Philosophy of Science Part B: Studies in History and Philosophy of Modern Physics》2012,43(2):95-104
We offer a framework to represent the roles of empirical and theoretical factors in theory construction, and examine a case study to illustrate how the framework can be used to illuminate central features of scientific reasoning. The case study provides an extension of French and Ladyman's (1997) analysis of Fritz and Heinz London's model of superconductivity to accommodate the role of the analogy between superconductivity and diamagnetic phenomena in the development of the model between 1935 and 1937. We focus on this case since it allows us to separate the roles of empirical and theoretical factors, and so provides an example of the utility of the approach that we have adopted. We conclude the paper by drawing on the particular framework here developed to address a range of concerns. 相似文献
365.
On-chip natural assembly of silicon photonic bandgap crystals. 总被引:20,自引:0,他引:20
Photonic bandgap crystals can reflect light for any direction of propagation in specific wavelength ranges. This property, which can be used to confine, manipulate and guide photons, should allow the creation of all-optical integrated circuits. To achieve this goal, conventional semiconductor nanofabrication techniques have been adapted to make photonic crystals. A potentially simpler and cheaper approach for creating three-dimensional periodic structures is the natural assembly of colloidal microspheres. However, this approach yields irregular, polycrystalline photonic crystals that are difficult to incorporate into a device. More importantly, it leads to many structural defects that can destroy the photonic bandgap. Here we show that by assembling a thin layer of colloidal spheres on a silicon substrate, we can obtain planar, single-crystalline silicon photonic crystals that have defect densities sufficiently low that the bandgap survives. As expected from theory, we observe unity reflectance in two crystalline directions of our photonic crystals around a wavelength of 1.3 micrometres. We also show that additional fabrication steps, intentional doping and patterning, can be performed, so demonstrating the potential for specific device applications. 相似文献
366.
The large degeneracy of states resulting from the geometrical frustration of competing interactions is an essential ingredient of important problems in fields as diverse as magnetism, protein folding and neural networks. As first explained by Pauling, geometrical frustration of proton positions is also responsible for the unusual low-temperature thermodynamics of ice and its measured 'ground state' entropy. Recent work has shown that the geometrical frustration of ice is mimicked by Dy2Ti2O7, a site-ordered magnetic material in which the spins reside on a lattice of corner-sharing tetrahedra where they form an unusual magnetic ground state known as 'spin ice'. Here we identify a cooperative spin-freezing transition leading to the spin-ice ground state in Dy2Ti2O7. This transition is associated with a very narrow range of relaxation times, and represents a new form of spin-freezing. The dynamics are analogous to those associated with the freezing of protons in ice, and they provide a means through which to study glass-like behaviour and the consequences of frustration in the limit of low disorder. 相似文献
367.
Superconductivity in CaCuO2 as a result of field-effect doping. 总被引:2,自引:0,他引:2
J H Sch?n M Dorget F C Beuran X Z Zu E Arushanov C Deville Cavellin M Lagu?s 《Nature》2001,414(6862):434-436
Understanding the doping mechanisms in the simplest superconducting copper oxide-the infinite-layer compound ACuO2 (where A is an alkaline earth metal)-is an excellent way of investigating the pairing mechanism in high-transition-temperature (high-Tc) superconductors more generally. Gate-induced modulation of the carrier concentration to obtain superconductivity is a powerful means of achieving such understanding: it minimizes the effects of potential scattering by impurities, and of structural modifications arising from chemical dopants. Here we report the transport properties of thin films of the infinite-layer compound CaCuO2 using field-effect doping. At high hole- and electron-doping levels, superconductivity is induced in the nominally insulating material. Maximum values of Tc of 89 K and 34 K are observed respectively for hole- and electron-type doping of around 0.15 charge carriers per CuO2. We can explore the whole doping diagram of the CuO2 plane while changing only a single electric parameter, the gate voltage. 相似文献
368.
J. D. R. de Raadt 《Systemic Practice and Action Research》2011,24(6):509-521
Starting from the crisis in our communities—research has indicated it to be widely spread—the author explores the scientific
tradition preceding the rise of modernism in order to draw out a more humane way of thinking that may help our contemporary
societies. He discerns between two types of Renaissance humanism, one characterised by its cleverness, the other by its compassion
and desire to restore dignity to the lives of people struggling to escape the clutches of medievalism. The father of the latter
is Erasmus of Rotterdam and we follow the development of his conception of a social humanism as it branches out, through the
work of his successors, into every scientific discipline, both natural and human. These disciplines are united by an embracing
systemic idea of philosophy that unites the mind with the heart and which Erasmus called “Philosophia Christi”. We examine
the two main pillars upon which this philosophy is built, love for our neighbour and education as the only legitimate instrument
to change society, and the extraordinary impact it had on science and on communities living in the seventeenth century. We
contrast this with the idea of power, the chosen instrument of modernity to transform society, and trace some of its tragic
outcomes. We conclude by discussing the incorporation of an Erasmian type of social humanism into systems education and the
future development of such programmes. 相似文献
369.
Pastorello A Della Valle M Smartt SJ Zampieri L Benetti S Cappellaro E Mazzali PA Patat F Spiro S Turatto M Valenti S 《Nature》2007,449(7164):E1-E2
An anomalous transient in the early Hubble-type (S0) galaxy Messier 85 (M85) in the Virgo cluster was discovered by Kulkarni et al. on 7 January 2006 that had very low luminosity (peak absolute R-band magnitude M(R) of about -12) that was constant over more than 80 days, red colour and narrow spectral lines, which seem inconsistent with those observed in any known class of transient events. Kulkarni et al. suggest an exotic stellar merger as the possible origin. An alternative explanation is that the transient in M85 was a type II-plateau supernova of extremely low luminosity, exploding in a lenticular galaxy with residual star-forming activity. This intriguing transient might be the faintest supernova that has ever been discovered. 相似文献
370.
Byrne JA Pedersen DA Clepper LL Nelson M Sanger WG Gokhale S Wolf DP Mitalipov SM 《Nature》2007,450(7169):497-502