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Cyclase-associated proteins are highly conserved proteins that have a role in the regulation of actin dynamics. Higher eukaryotes have two isoforms, CAP1 and CAP2. To study the in vivo function of CAP2, we generated mice in which the CAP2 gene was inactivated by a gene-trap approach. Mutant mice showed a decrease in body weight and had a decreased survival rate. Further, they developed a severe cardiac defect marked by dilated cardiomyopathy (DCM) associated with drastic reduction in basal heart rate and prolongations in atrial and ventricular conduction times. Moreover, CAP2-deficient myofibrils exhibited reduced cooperativity of calcium-regulated force development. At the microscopic level, we observed disarrayed sarcomeres with development of fibrosis. We analyzed CAP2’s role in actin assembly and found that it sequesters G-actin and efficiently fragments filaments. This activity resides completely in its WASP homology domain. Thus CAP2 is an essential component of the myocardial sarcomere and is essential for physiological functioning of the cardiac system, and a deficiency leads to DCM and various cardiac defects.  相似文献   
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'Trapped rainbow' storage of light in metamaterials   总被引:1,自引:0,他引:1  
Tsakmakidis KL  Boardman AD  Hess O 《Nature》2007,450(7168):397-401
Light usually propagates inside transparent materials in well known ways. However, recent research has examined the possibility of modifying the way the light travels by taking a normal transparent dielectric and inserting tiny metallic inclusions of various shapes and arrangements. As light passes through these structures, oscillating electric currents are set up that generate electromagnetic field moments; these can lead to dramatic effects on the light propagation, such as negative refraction. Possible applications include lenses that break traditional diffraction limits and 'invisibility cloaks' (refs 5, 6). Significantly less research has focused on the potential of such structures for slowing, trapping and releasing light signals. Here we demonstrate theoretically that an axially varying heterostructure with a metamaterial core of negative refractive index can be used to efficiently and coherently bring light to a complete standstill. In contrast to previous approaches for decelerating and storing light, the present scheme simultaneously allows for high in-coupling efficiencies and broadband, room-temperature operation. Surprisingly, our analysis reveals a critical point at which the effective thickness of the waveguide is reduced to zero, preventing the light wave from propagating further. At this point, the light ray is permanently trapped, its trajectory forming a double light-cone that we call an 'optical clepsydra'. Each frequency component of the wave packet is stopped at a different guide thickness, leading to the spatial separation of its spectrum and the formation of a 'trapped rainbow'. Our results bridge the gap between two important contemporary realms of science-metamaterials and slow light. Combined investigations may lead to applications in optical data processing and storage or the realization of quantum optical memories.  相似文献   
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The different degrees of freedom of a given system are usually independent of each other but can in some materials be strongly coupled, giving rise to phase equilibria sensitively susceptible to external perturbations. Such systems often exhibit unusual physical properties that are difficult to treat theoretically, as exemplified by strongly correlated electron systems such as intermediate-valence rare-earth heavy fermions and Kondo insulators, colossal magnetoresistive manganites and high-transition temperature (high-T(c)) copper oxide superconductors. Metal fulleride salts-metal intercalation compounds of C60--and materials based on rare-earth metals also exhibit strong electronic correlations. Rare-earth fullerides thus constitute a particularly intriguing system--they contain highly correlated cation (rare-earth) and anion (C60) sublattices. Here we show, using high-resolution synchrotron X-ray diffraction and magnetic susceptibility measurements, that cooling the rare-earth fulleride Sm2.75C60 induces an isosymmetric phase transition near 32 K, accompanied by a dramatic isotropic volume increase and a samarium valence transition from (2 + epsilon) + to nearly 2 +. The negative thermal expansion--heating from 4.2 to 32 K leads to contraction rather than expansion--occurs at a rate about 40 times larger than in ternary metal oxides typically exhibiting such behaviour. We attribute the large negative thermal expansion, unprecedented in fullerene or other molecular systems, to a quasi-continuous valence transition from Sm(2+) towards the smaller Sm((2+epsilon)+), analogous to the valence or configuration transitions encountered in intermediate-valence Kondo insulators like SmS (ref. 3).  相似文献   
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