Search results for "ML"

A germline mutation in the platelet-derived growth factor receptor beta gene may be implicated in hereditary progressive mucinous histiocytosis.

2020

Epistemic Relativity: An Experimental Approach to Physics

2019

The recent concept of relativistic positioning system (RPS) has opened the possibility of making Relativity the general standard frame in which to state any physical problem, theoretical or experimental. Because the velocity of propagation of the information is finite, epistemic relativity proposes to integrate the physicist as a truly component of every physical problem, taking into account explicitly what information, when and where, the physicist is able to know. This leads naturally to the concept of relativistic stereometric system (RSS), allowing to measure the intrinsic properties of physical systems. Together, RPSs and RSSs complete the notion of laboratory in general relativity, al…

Congenital secretory diarrhoea caused by activating germline mutations in GUCY2C

2016

Objective Congenital sodium diarrhoea (CSD) refers to a form of secretory diarrhoea with intrauterine onset and high faecal losses of sodium without congenital malformations. The molecular basis for CSD remains unknown. We clinically characterised a cohort of infants with CSD and set out to identify disease-causing mutations by genome-wide genetic testing. Design We performed whole-exome sequencing and chromosomal microarray analyses in 4 unrelated patients, followed by confirmatory Sanger sequencing of the likely disease-causing mutations in patients and in their family members, followed by functional studies. Results We identified novel de novo missense mutations in GUCY2C, the gene encod…

Synchrotron diffraction study of the isothermal oxidation of uranium dioxide at 250°C

2003

ABSTRACTThe structural evolution of UO2 during its oxidation to U3O8 at 250°C in air was studied by in-situ synchrotron X-ray diffraction on the D2AM-CRG beamline at ESRF. The aim of this study is to determine the phases that are likely to appear during the long-term storage of spent nuclear fuel. Our results are in disagreement with the literature in which the existence of an intermediate cubic phase is not reported. Instead, an α-U3O7 tetragonal phase (c/a < 1) was mentioned but not definitively observed. These previous interpretations may have been the result of poor instrumental resolution.

High-energy X-ray diffraction and topography investigation of CdZnTe

2005

High-energy transmission x-ray diffraction techniques have been applied to investigate the crystal quality of CdZnTe (CZT). CdZnTe has shown excellent performance in hard x-ray and gamma detection; unfortunately, bulk nonuniformities still limit spectroscopic properties of CZT detectors. Collimated high-energy x-rays, produced by a superconducting wiggler at the National Synchrotron Light Source’s X17B1 beamline, allow for a nondestructive characterization of thick CZT samples (2–3 mm). In order to have complete information about the defect distribution and strains in the crystals, two series of experiments have been performed. First, a monochromatic 67 keV x-ray beam with the size of 300×3…

Photoemission from Pt(111)-(hex)-Rb and Pt(111)-(4×1)-RbO using polarised synchrotron radiation

2001

Abstract In various experiments it has been demonstrated that the circular dichroism in the angular distribution of photoelectrons (CDAD) is not only observed from oriented initial states (aligned adsorbed molecules or magnetised samples), but also arises as a consequence of the scattering of photoelectrons from the surrounding atoms in a solid or an adsorbate. In this work we will show first measurements performed at the SGM beamline of the Brazilian Synchrotron Light Source (LNLS) on a (4×1) superstructure of 1 ML RbO adsorbed on Pt(111). Measurements from the 4s core levels of Rb adsorbed at Pt(111) were also performed at the PM-III beamline at BESSY. The measured variations of the inten…

Contribution of the synchrotron diffraction study of the oxidation of uranium dioxide at 250○C

2004

The structural evolution of UO 2 during its oxidation into U 3 O 8 at 250°C in air was studied by in-situ synchrotron X-ray diffraction on the D2AM-CRG beamline at ESRF. The aim of this study is to determine the phases which are likely to appear during a long term storage of used nuclear fuel. Our results are in disagreement with the literature where the existence of the secondary cubic phase is not reported, and an α-U 3 O 7 tetragonal phase (c/a < 1) is also mentioned but definitely not observed. These previous interpretations are possibly due to a poor instrumental resolution, inducing a sensible broadening of the diffraction peaks. Particularly, the fact that the instrumental resolution…

Forme del testo digitale

2017

L'articolo costituisce una riflessione sulla modellizzazione dei testi appartenenti alla nostra tradizione culturale in questa fase aurorale della Filologia Digitale e parte dalla domanda: che "forma" stiamo dando ai nostri testi? Vengono asplorati tre aspetti per cui i nostri modelli testuali riflettono i nostri presupposti culturali e tecnologici: in primo luogo, il modello gerarchico OHCO che sta alla base di TEI XML; in secondo luogo, il modello sequenziale alfabetico basato sulla stampa delle lingue occidentali, che sta alla base di Unicode. Un terzo aspetto, poi, è il meno esplorato nella ricerca: protocolli per la citazione testuale come CITE/CTS sono basati su corpora specifici. Per…

Using Search Algorithms for Modeling Economic Processes

2013

Abstract Economic issues are placed in formal practice, when is desired a modelling of the economic process, a manufacturing process, a device, etc. Each share of that economic process is denoted by a, b, c, d, these actions with defined time periods and action pairs are formed strings of the form, ab * cab * bc ., ab, bb, bc. so for them there are no other restrictions. If the graph is viewed as a system image, nodes representing components, then an immediate interpretation of an arc (xi, xj) are the component xi that is said to directly influence component xj. If nodes have the significance of possible states of a system when a spring (xi.xj) means that, the system can jump from state xi …

Banach spaces which are somewhat uniformly noncreasy

2003

AbstractWe consider a family of spaces wider than r-UNC spaces and we give some fixed point results in the setting of these spaces.