4 resultados para hyper-Dk material

em Duke University


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This paper focuses on the nature of jamming, as seen in two-dimensional frictional granular systems consisting of photoelastic particles. The photoelastic technique is unique at this time, in its capability to provide detailed particle-scale information on forces and kinematic quantities such as particle displacements and rotations. These experiments first explore isotropic stress states near point J through measurements of the mean contact number per particle, Z, and the pressure, P as functions of the packing fraction, . In this case, the experiments show some but not all aspects of jamming, as expected on the basis of simulations and models that typically assume conservative, hence frictionless, forces between particles. Specifically, there is a rapid growth in Z, at a reasonable which we identify with as c. It is possible to fit Z and P, to power law expressions in - c above c, and to obtain exponents that are in agreement with simulations and models. However, the experiments differ from theory on several points, as typified by the rounding that is observed in Z and P near c. The application of shear to these same 2D granular systems leads to phenomena that are qualitatively different from the standard picture of jamming. In particular, there is a range of packing fractions below c, where the application of shear strain at constant leads to jammed stress-anisotropic states, i.e. they have a non-zero shear stress, τ. The application of shear strain to an initially isotropically compressed (hence jammed) state, does not lead to an unjammed state per se. Rather, shear strain at constant first leads to an increase of both τ and P. Additional strain leads to a succession of jammed states interspersed with relatively localized failures of the force network leading to other stress-anisotropic states that are jammed at typically somewhat lower stress. The locus of jammed states requires a state space that involves not only and τ, but also P. P, τ, and Z are all hysteretic functions of shear strain for fixed . However, we find that both P and τ are roughly linear functions of Z for strains large enough to jam the system. This implies that these shear-jammed states satisfy a Coulomb like-relation, τ = μP. © 2010 The Royal Society of Chemistry.

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The purpose of this research was to use next generation sequencing to identify mutations in patients with primary immunodeficiency diseases whose pathogenic gene mutations had not been identified. Remarkably, four unrelated patients were found by next generation sequencing to have the same heterozygous mutation in an essential donor splice site of PIK3R1 (NM_181523.2:c.1425 + 1G > A) found in three prior reports. All four had the Hyper IgM syndrome, lymphadenopathy and short stature, and one also had SHORT syndrome. They were investigated with in vitro immune studies, RT-PCR, and immunoblotting studies of the mutation's effect on mTOR pathway signaling. All patients had very low percentages of memory B cells and class-switched memory B cells and reduced numbers of naïve CD4+ and CD8+ T cells. RT-PCR confirmed the presence of both an abnormal 273 base-pair (bp) size and a normal 399 bp size band in the patient and only the normal band was present in the parents. Following anti-CD40 stimulation, patient's EBV-B cells displayed higher levels of S6 phosphorylation (mTOR complex 1 dependent event), Akt phosphorylation at serine 473 (mTOR complex 2 dependent event), and Akt phosphorylation at threonine 308 (PI3K/PDK1 dependent event) than controls, suggesting elevated mTOR signaling downstream of CD40. These observations suggest that amino acids 435-474 in PIK3R1 are important for its stability and also its ability to restrain PI3K activity. Deletion of Exon 11 leads to constitutive activation of PI3K signaling. This is the first report of this mutation and immunologic abnormalities in SHORT syndrome.