Inherited factor VII (FVII) deficiency is a rare autosomal recessive disorder. Mutations and polymorphisms of the FVII gene were characterized in more than 40 unrelated patients with FVII deficiency. Among the 29 different mutations, the most frequent were Ala294 Val, Ala294Val;404delC, IVS7+7, and Val281 Phe. Four novel mutations (IVS2+1G>C, Arg247 Cys, Glu265 Lys, Asp343 His) were detected. The relationships between genotypes of mutations and polymorphisms of the FVII gene, FVII deficiency, and clinical phenotype were investigated. Homozygosity of the Phe4 Leu, IVS4+1G>A, Cys135 Arg, Ala244 Val, and Ala294 Val;404delC and the double heterozygosity of Tyr68 Cys / IVS3-1G>A, Val252 Met / IVS2+5G>T, Val281 Phe / Cys135 Arg, Ala294 Val / Val281 Phe, Ala294 Val;404delC / Val281Phe, Ala294 Val;404delC / Arg152 stop, Ala294Val;404delC / Gln(-35) stop, Ala294 Val / Val252 Met, Ala294 Val / Gly156 Asp, and Thr359 Met / Asp242 His were related to clinical symptoms. Double heterozygotes for Arg247 Cys / IVS2+1G>C, Ala206 Thr / Pro303 Arg, Leu(-20) Pro / Val252 Met as well as IVS7+7 /Ala294 Val, IVS7+7 /Ala206 Thr, and IVS7+7 / Met298 Ile were asymptomatic. The clinical symptomatology is rather poor in correlation with the FVII activity. Concerning the clinical phanotype, a correlation seems to exist between specific mutations and clinical symptoms.
Objective: To assess availability, variety, price and quality of different food products in a convenience sample of supermarkets in Germany and the USA. Design: Cross-sectional study using an adapted version of the Bridging the Gap Food Store Observation Form. Setting: Information on availability, quality, price and variety of selected food products in eight German and seven US supermarkets (discount and full service) was obtained and compared by country. Results: A general tendency for lower prices of fruits and vegetables in Germany was observed, while produce quality and variety did not seem to differ between countries, with the exception of the variety of some vegetables such as tomatoes. Chips and cereals did not differ significantly in variety nor price. In both countries, high energy-dense foods were lower in energy costs than lower energy-dense foods. Conclusions: The influence of food prices and availability on consumption should be further explored, including the impact of country differences.
S U M M A R YWe show the numerical applicability of a multiresolution method based on harmonic splines on the 3-D ball which allows the regularized recovery of the harmonic part of the Earth's mass density distribution out of different types of gravity data, for example, different radial derivatives of the potential, at various positions which need not be located on a common sphere. This approximated harmonic density can be combined with its orthogonal anharmonic complement, for example, determined out of the splitting function of free oscillations, to an approximation of the whole mass density function. The applicability of the presented tool is demonstrated by several test calculations based on simulated gravity values derived from EGM96. The method yields a multiresolution in the sense that the localization of the constructed spline basis functions can be increased which yields in combination with more data a higher resolution of the resulting spline. Moreover, we show that a locally improved data situation allows a highly resolved recovery in this particular area in combination with a coarse approximation elsewhere which is an essential advantage of this method, for example, compared to polynomial approximation. and the references therein). From the mathematical point of view, the inverse gravimetric problem is based on a Fredholm integral equation of the first kind involving Newton's law of gravitation. Thus, the relation between the gravitational potential V of the Earth and the density distribution ρ is given byIn reality, the gravitational potential V can be determined outside the Earth whereas the density ρ represents the unknown part. This means that we have to consider the inversion of the equation above. Problems of such kind are called inverse problems. By Hadamard's classification, that is the uniqueness, the existence and the stability of the solution, we divide inverse problems in ill-posed and well-posed problems. It is a well-known fact that the inverse gravimetric problem is ill-posed because each of these three criteria can be invalid.For finding an approximation for the solution of such an ill-posed inverse problem several different methods were developed. The classical approach, for example, is a method using a truncated singular value decomposition (see, e.g. Škorvanek 1981;Tscherning & Sünkel 1981;Tscherning & Strykowski 1988). This method has several well-known disadvantages like the non-localizing character of the used spherical C 2008 The Authors
Modular polyketide synthases (PKSs) from Streptomyces and related genera of bacteria produce many important pharmaceuticals. A program called CompGen was developed to carry out in silico homologous recombination between gene clusters encoding PKSs and determine whether recombinants have cluster architectures compatible with the production of polyketides. The chemical structure of recombinant polyketides was also predicted. In silico recombination was carried out for 47 well-characterised clusters. The predicted recombinants would produce 11,796 different polyketide structures. The molecular weights and average degree of reduction of the chemical structures are dispersed around the parental structures indicating that they are likely to include pharmaceutically interesting compounds. The details of the recombinants and the chemical structures were entered in a database called r-CSDB. The virtual compound library is a useful resource for computer-aided drug design and chemoinformatics strategies for finding pharmaceutically relevant chemical entities. A strategy to construct recombinant Streptomyces strains to produce these polyketides is described and the critical steps of mobilizing large biosynthetic clusters and producing new linear cloning vectors are illustrated by experimental data.
The purpose of this paper is the canonical connection of classical global gravity field determination following the concept of Stokes (1849), Bruns (1878), and Neumann (1887) on the one hand and modern locally oriented multiscale computation by use of adaptive locally supported wavelets on the other hand. Essential tools are regularization methods of the Green, Neumann, and Stokes integral representations. The multiscale approximation is guaranteed simply as linear difference scheme by use of Green, Neumann, and Stokes wavelets, respectively. As an application, gravity anomalies caused by plumes are investigated for the Hawaiian and Iceland areas.
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