: The palmoplantar keratodermas (PPK) are a heterogeneous group of conditions, most frequently inherited in autosomal dominant fashion. A few are well‐documented autosomal recessive disorders; other ...are acquired in association with certain metabolic disorders and malignancies. Recently different point mutations of the keratin 9 (K9) gene have been identified in unrelated families with epidermolytic palmoplantar keratoderma (EPPK). We investigated two unrelated Hungarian families with EPPK. In one, a mutation consisting of a G→A transversion at nucleotide position 551, which changes codon arginine to glutamine at codon 162 (R162Q), was found. In the other, we observed a novel mutation at nucleotide position 571, which changes codon 169 lysine (AAG) into the amber stop codon (TAG) (K169X). Each found mutation is present in the highly conserved coil 1A region of the rod domain. In the case of a stop codon type of mutation, it is questionable whether it really results in a clinical phenotype, but segregation analysis revealed cosegregation of the PPK phenotype with the mutant allele.
The ESA-JAXA BepiColombo mission to Mercury will provide simultaneous measurements from two spacecraft, offering an unprecedented opportunity to investigate magnetospheric and exospheric particle ...dynamics at Mercury as well as their interactions with solar wind, solar radiation, and interplanetary dust. The particle instrument suite SER ENA (Search for Exospheric Refilling and Emitted Natural Abundances) is flying in space on-board the BepiColombo Mercury Planetary Orbiter (MPO) and is the only instrument for ion and neutral particle detection aboard the MPO. It comprises four independent sensors: ELENA for neutral particle flow detection, Strofio for neutral gas detection, PICAM for planetary ions observations, and MIPA, mostly for solar wind ion measurements. SERENA is managed by a System Control Unit located inside the ELENA box. In the present paper the scientific goals of this suite are described, and then the four units are detailed, as well as their major features and calibration results. Finally, the SERENA operational activities are shown during the orbital path around Mercury, with also some reference to the activities planned during the long cruise phase.
The Federal Arbitration Act (FAA) is the primary federal statute governing millions of arbitration agreements that have mushroomed in every nook and cranny of modern American society. In its most ...recent FAA decision from Dec 2015, DIRECTV, Inc v. Imburgia, the Supreme Court continued its awkward tradition of issuing preposterous FAA rulings. This article explores the Supreme Court's tortured and strained interpretation of the FAA in its recent DIRECTV decision.
In this paper we present the so-called standardized pricking probe surveying technique and demonstrate its usefulness in an archaeological study. The buried target is a Palaeochristian sepulchral ...chapel, which had already been excavated 82 years ago, then re-buried and forgotten.
By applying this technique, it was possible to locate the buried remnants of the chapel in a large field, in spite of the dense undergrowth, where classical geophysical methods could have hardly been applied. When the area was mopped-up, a detailed and systematic pricking probe surveying was carried out, and completed with geoelectric, magnetic and georadar mapping. The standardized pricking probe images, at least in this field experiment, proved to be competitive to the geophysical maps.
The optimum pricking probe parameters such as horizontal interval, pricking depth, observable quantity and the way of presentation were optimized through field experiments. For a detailed investigation a rectangular grid with an interval of 50
cm (i.e. a grid interval, corresponding to the wall thickness) is recommended, while for reconnaissance measurements a two times larger horizontal interval (1
m in this case) proved to be sufficient. In this case study the optimum pricking depth was 20–30
cm; in general it depends on the burial depth of the investigated object. For the presentation of the results a suitable running average of a two-valued observable quantity is defined.
The merits of the standardized pricking probe technique are as follows: its field procedure and data processing are simple, it is cheap and relatively quick; it does not need any electronic instrument, therefore there are no investment costs and there is no risk of technical failures; the technique can be applied even among the most unfavourable field conditions like e.g. bad weather, extreme topography, dense undergrowth, etc. At the same time, the standardized pricking probe method should be applied only in areas, where the possible damaging of the buried structures is excluded.
The paper focuses on the basic mathematical and theoretical questions of spatial interpolation of meteorological elements. Nowadays, in meteorology the most often applied procedures for spatial ...interpolation are the geostatistical interpolation methods built also in GIS software. The mathematical basis of these methods is the geostatistics that is an exact but special part of the mathematical statistics. However, special meteorological spatial interpolation methods for climate elements also exist, such as Gandin optimum interpolation as well as the MISH method developed at the Hungarian Meteorological Service in the last few years. These meteorological interpolation methods are also based on the mathematical statistical theory. Therefore, the basic type of the interpolation formulas applied by the geostatistical and meteorological methods are similar. One of our intentions is to present some comparison of the various kriging formulas, such as ordinary, universal, regression, residual, detrended, etc., ones. In general, these formulas can be derived from the multiple linear regression formula by using the generalized-least-squares estimation for certain unknown parameters. But the main difference between the geostatistical and meteorological interpolation methods can be found in the amount of information used for modeling the necessary statistical parameters. In geostatistics, the usable information or the sample for modeling is only the system of predictors, which is a single realization in time, while in meteorology we have spatiotemporal data, namely the long data series which form a sample in time and space as well. The long data series is such a speciality of the meteorology that makes possible to model efficiently the statistical parameters in question.
‘Search for Exospheric Refilling and Emitted Natural Abundances’ (SERENA) is an instrument package that will fly on board the BepiColombo/Mercury Planetary Orbiter (MPO). It will investigate ...Mercury's complex particle environment that is composed of thermal and directional neutral atoms (exosphere) caused by surface release and charge-exchange processes, and of ionized particles caused by photo-ionization of neutrals as well by charge exchange and surface release processes. In order to investigate the structure and dynamics of the environment, an in-situ analysis of the key neutral and charged components is necessary, and for this purpose the SERENA instrument shall include four units: two neutral particle analyzers (Emitted Low Energy Neutral Atoms (ELENA) sensor and Start from a Rotating FIeld mass spectrometer (STROFIO)) and two ion spectrometers (Miniature Ion Precipitation Analyzer (MIPA) and Planetary Ion Camera (PICAM)). The scientific merits of SERENA are presented, and the basic characteristics of the four units are described, with a focus on novel technological aspects.
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