A mutational hot spot in keratin 10 (KRT 10) in patients with epidermolytic hyperkeratosis

Epidermolytic hyperkeratosis (EHK), (bullous congenital ichthyosiform erythroderma), is an autosomal dominant human skin disorder. Recently, we and others have described mutations in keratins 1 and 10 (K1 and K10) in patients with this disease. Structure-function models predict that these mutations would impair normal filament assembly and function. We have extended our earlier studies to include 8 more incidences of EHK. In half of these families, we were unable to locate a mutation within the rod domains of either K1 or K10. However, polymorphic restriction site and sequence analysis of the other families revealed a mutational hot spot within the 1A alpha-helical segment of K10. These involve Arginine to Histidine, Arginine to Cysteine and Arginine to Leucine substitutions at residue 10 of the rod domain. Interestingly, mutations in the corresponding Arginine residue in keratin K14 have been identified in patients with epidermolysis bullosa simplex. The large number of mutations found at this position in both keratins K10 and K14 suggests that other epithelia cell disorders will be discovered that are caused by the corresponding mutation in related type I keratin gene

Персональная система управления вниманием в интеллектуальных видах деятельности

В данной работе описывается процесс разработки персональной системы управления вниманием в интеллектуальных видах деятельности. Объектом исследования является концентрация внимания, а также способы её улучшения реализуемые посредством объекта промышленного дизайна.This paper describes the process of developing a personal attention management system in intellectual activities. The object of research is the concentration of attention, as well as ways to improve it, implemented through the object of industrial design

Positive-P and Wigner representations for quantum-optical systems with nonorthogonal modes

We generalize the basic concepts of the positive-P and Wigner representations to unstable quantum-optical systems that are based on nonorthogonal quasimodes. This lays the foundation for a quantum description of such systems, such as, for example an unstable cavity laser. We compare both representations by calculating the tunneling times for an unstable resonator optical parametric oscillator

Friction of the surface plasmon by high-energy particle-hole pairs: Are memory effects important?

We show that the dynamics of the surface plasmon in metallic nanoparticles damped by its interaction with particle-hole excitations can be modelled by a single degree of freedom coupled to an environment. In this approach, the fast decrease of the dipole matrix elements that couple the plasmon to particle-hole pairs with the energy of the excitation allows a separation of the Hilbert space into low- and high-energy subspaces at a characteristic energy that we estimate. A picture of the spectrum consisting of a collective excitation built from low-energy excitations which interacts with high-energy particle-hole states can be formalised. The high-energy excitations yield an approximate description of a dissipative environment (or "bath") within a finite confined system. Estimates for the relevant timescales establish the Markovian character of the bath dynamics with respect to the surface plasmon evolution for nanoparticles with a radius larger than about 1 nm.Comment: 8 pages, 1 figure; see also cond-mat/070372

Elastography of vocal folds

Abstract The diagnosis of changes in the structure of the vocal folds is an important clinical task. There is no approach available, which is capable of getting synchronous spatial and biomechanical properties of them. Whereas biomechanical changes, particularly the elasticity, are a good maker for malignant and benign conditions. To gain the elastic characteristics of the vocal folds we are proposing an endoscopic approach using ultrasound elastography

Quantum Statistics of Surface Plasmon Polaritons in Metallic Stripe Waveguides

Single surface plasmon polaritons are excited using photons generated via spontaneous parametric down-conversion. The mean excitation rates, intensity correlations and Fock state populations are studied. The observed dependence of the second order coherence in our experiment is consistent with a linear uncorrelated Markovian environment in the quantum regime. Our results provide important information about the effect of loss for assessing the potential of plasmonic waveguides for future nanophotonic circuitry in the quantum regime.Comment: 21 pages, 6 figures, published in Nano Letters, publication date (web): March 27 (2012

A Comparative Study of the Formation of Aromatics in Rich Methane Flames Doped by Unsaturated Compounds

For a better modeling of the importance of the different channels leading to the first aromatic ring, we have compared the structures of laminar rich premixed methane flames doped with several unsaturated hydrocarbons: allene and propyne, because they are precursors of propargyl radicals which are well known as having an important role in forming benzene, 1,3-butadiene to put in evidence a possible production of benzene due to reactions of C4 compounds, and, finally, cyclopentene which is a source of cyclopentadienylmethylene radicals which in turn are expected to easily isomerizes to give benzene. These flames have been stabilized on a burner at a pressure of 6.7 kPa (50 Torr) using argon as dilutant, for equivalence ratios (?) from 1.55 to 1.79. A unique mechanism, including the formation and decomposition of benzene and toluene, has been used to model the oxidation of allene, propyne, 1,3 butadiene and cyclopentene. The main reaction pathways of aromatics formation have been derived from reaction rate and sensitivity analyses and have been compared for the three types of additives. These combined analyses and comparisons can only been performed when a unique mechanism is available for all the studied additives

Tourism policy and destination marketing in developing countries: the chain of influence

Tourism marketers including destination marketing organisations (DMOs) and international tour operators play a pivotal role in destination marketing, especially in creating destination images. These images, apparent in tourist brochures, are designed to influence tourist decision-making and behaviour. This paper proposes the concept of a “chain of influence” in destination marketing and image-making, suggesting that the content of marketing materials is influenced by the priorities of those who design these materials, e.g. tour operators and DMOs. A content analysis of 2,000 pictures from DMO and tour operator brochures revealed synergies and divergence between these marketers. The brochure content was then compared to the South African tourism policy, concluding that the dominant factor in the chain of influence in the South African context is in fact its organic image

The Trade-Off between Phosphorus Recycling and Health Protection during the BSE Crisis in Switzerland. A “Disposal Dilemma”

Phosphorus (P) is an essential, finite resource whose geological, economic, and geopolitical accessibility may become critical in the future. Because P losses from agriculture, sewage and waste have serious environmental impacts such as eutrophication, it would be desirable to close P cycles. However, there may arise dilemmas due to trade-offs with other issues. For instance, recycling of animal bones – containing relatively high amounts of P – was prohibited in Switzerland after the BSE crisis (1989 to 2000) due to perceived health risks. Our analysis indicates that the great- est potentials for closing the P cycle in Switzerland are to reduce P losses in agriculture and to increase P recovery from sewage and waste. Whereas in agriculture significant improvements have been made, the disposal dilemma of animal bones led to diminishing amounts of P being recycled from waste. Based on their risk perception, the involved stakeholders prioritized preventing potential harm to (human) health over maximizing P recycling