937 research outputs found
X-linked lymphoproliferative disease (XLP)
Review on X-linked lymphoproliferative disease (XLP), with data on clinics, and the genes involved
Influence of geography on language competition
Competition between languages or cultural traits diffusing in the same
geographical area is studied combining the language competition model of Abrams
and Strogatz and a human dispersal model on an inhomogeneous substrate. Also,
the effect of population growth is discussed. It is shown through numerical
experiments that the final configuration of the surviving language can be
strongly affected by geographical and historical factors. These factors are not
related to the dynamics of culture transmission, but rather to initial
population distributions as well as geographical boundaries and
inhomogeneities, which modulate the diffusion process.Comment: typos in contact information have been corrected - text/figures not
change
Stepwise phosphorylation of p65 promotes NF-kappa B activation and NK cell responses during target cell recognition
NF-ÎșB is a key transcription factor that dictates the outcome of diverse immune responses. How NF-ÎșB is regulated by multiple activating receptors that are engaged during natural killer (NK)-target cell contact remains undefined. Here we show that sole engagement of NKG2D, 2B4 or DNAM-1 is insufficient for NF-ÎșB activation. Rather, cooperation between these receptors is required at the level of Vav1 for synergistic NF-ÎșB activation. Vav1-dependent synergistic signalling requires a separate PI3K-Akt signal, primarily mediated by NKG2D or DNAM-1, for optimal p65 phosphorylation and NF-ÎșB activation. Vav1 controls downstream p65 phosphorylation and NF-ÎșB activation. Synergistic signalling is defective in X-linked lymphoproliferative disease (XLP1) NK cells entailing 2B4 dysfunction and required for p65 phosphorylation by PI3K-Akt signal, suggesting stepwise signalling checkpoint for NF-ÎșB activation. Thus, our study provides a framework explaining how signals from different activating receptors are coordinated to determine specificity and magnitude of NF-ÎșB activation and NK cell responses
Drinfeld twist and symmetric Bethe vectors of the open XYZ chain with non-diagonal boundary terms
With the help of the Drinfeld twist or factorizing F-matrix for the
eight-vertex solid-on-solid (SOS) model, we find that in the F-basis provided
by the twist the two sets of pseudo-particle creation operators simultaneously
take completely symmetric and polarization free form. This allows us to obtain
the explicit and completely symmetric expressions of the two sets of Bethe
states of the model.Comment: Latex file, 25 page
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On the representation error in data assimilation
Representation, representativity, representativeness error, forward interpolation error, forward model error, observation operator error, aggregation error and sampling error are all terms used to refer to components of observation error in the context of data assimilation. This paper is an attempt to consolidate the terminology that has been used in the earth sciences literature and was suggested at a European Space Agency workshop held in Reading in April 2014. We review the state-of-the-art, and through examples, motivate the terminology. In addition to a theoretical framework, examples from application areas of satellite data assimilation, ocean reanalysis and atmospheric chemistry data assimilation are provided. Diagnosing representation error statistics
as well as their use in state-of-the-art data assimilation systems is discussed within a consistent framework
Incubation of ovine scrapie with environmental matrix results in biological and biochemical changes of PrPSc over time
Ovine scrapie can be transmitted via environmental reservoirs. A pool of ovine scrapie isolates were incubated on soil for one day or thirteen months and eluted prion was used to challenge tg338 mice transgenic for ovine PrP. After one-day incubation on soil, two PrPSc phenotypes were present: G338 or Apl338ii. Thirteen months later some divergent PrPSc phenotypes were seen: a mixture of Apl338ii with either G338 or P338, and a completely novel PrPSc deposition, designated Cag338. The data show that prolonged ageing of scrapie prions within an environmental matrix may result in changes in the dominant PrPSc biological/biochemical properties
Cassini observations of planetary-period oscillations of Saturn's magnetopause
Examination of Cassini magnetic field and plasma data in the outer boundary regions of Saturn's magnetosphere shows that magnetopause oscillations at the planetary period commonly occur, in phase with plasma pressure variations inside the magnetosphere. The peak-to-trough amplitude of the boundary oscillations mapped to the planet-Sun line is estimated to be typically similar to 2 R-S, corresponding to a similar to 10% change in the boundary radius. The change in internal pressure required to produce such motions is estimated to be similar to 40% of the background values. A qualitative physical picture is proposed in which a compressive wave propagates outward through the sub-corotating outer magnetospheric plasma, originating from a corotating source in the nearer-planet region
Diagnostic criteria and tumor screening for individuals with isolated hemihyperplasia
Isolated hemihyperplasia, formerly termed isolated hemihypertrophy, is a congenital overgrowth disorder associated with an increased risk for embryonal tumors, mainly Wilms tumor and hepatoblastoma. This practice guideline will set forth the diagnostic criteria and tumor screening recommendations for children with isolated hemihyperplasia, based on the best information available. There is clinical overlap between isolated hemihyperplasia with Beckwith-Wiedemann syndrome. The majority of Beckwith-Wiedemann syndrome patients have a molecular abnormality involving the imprinted cluster of genes at 11p15.5. In contrast, the preponderance of isolated hemihyperplasia patients studied have no identified etiology. Tumors have developed in isolated hemihyperplasia patients with and without molecular abnormalities. For this reason, molecular diagnostics are not helpful in identifying the subset of isolated hemihyperplasia patients with tumor risk and all isolated hemihyperplasia patients should undergo tumor screening
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