The search of 'novel' mtDNA mutations in hypertrophic cardiomyopathy: MITOMAPping as a risk factor

MITOMAP is by far the most frequently cited Web resource that is referred to in substantiating novelty of an mtDNA mutation. This database, as is now known, has quite an incomplete coverage of the mtDNA mutations from the literature. This circumstance has

Modelling information requirements in complex engineering services

Services based around complex engineering equipment and systems provide substantial challenges in both the long-term management of the equipment and the need for guaranteed delivery of the related service. One of the challenges for an organisation providing these services is the management of the information that is required to design, deliver and subsequently assess the success of the service. To assist in this process this paper develops a model for capturing, organising and assessing information requirements for these Complex Engineering Services in which information required to support key decisions in the life cycle of the service is identified. The model – referred to as The 12-Box Model for Service Information Requirements – is embedded in a three-phase procedure for providing an assessment of information requirements of a service contract which also provides insight into the capabilities of available information systems in supporting the contract. An illustrative example examining service information in an aircraft availability contract is used to demonstrate the use of the 12-Box Model and associated assessment procedure.

Entrepreneurial innovation problems associated with the dynamic growth of university spin-outs in China: A capabilities perspective

This research aims to develop a capabilities-based conceptual framework in order to study the stage-specific innovation problems associated with the dynamic growth process of university spin-outs (hereafter referred to as USOs) in China. Based on the existing literature, pilot cases and five critical cases, this study attempts to explore the interconnections between the entrepreneurial innovation problems and the configuration of innovative capabilities (that acquire, mobilise and re-configure the key resources) throughout the lifecycle of a firm in four growth phases. This paper aims to contribute to the literature in a holistic manner by providing a theoretical discussion of USOs' development through adding evidence from a rapid growth emerging economy. To date, studies that have investigated the development of USOs in China recognised the heterogeneity of USOs in terms of capabilities still remain sparse. Addressing this research gap will be of great interest to entrepreneurs, policy makers and venture investors. © Copyright 2010 Inderscience Enterprises Ltd.

Thermal stratification produced by plumes and jets in enclosed spaces

The airflow and thermal stratification produced by a localised heat source located at floor level in a closed room is of considerable practical interest and is commonly referred to as a 'filling box'. In rooms with low aspect ratios H/R ≲ 1 (room height H to characteristic horizontal dimension R) the thermal plume spreads laterally on reaching the ceiling and a descending horizontal 'front' forms separating a stably stratified, warm upper region from cooler air below. The stratification is well predicted for H/R ≲ 1 by the original filling box model of Baines and Turner (J. Fluid. Mech. 37 (1968) 51). This model represents a somewhat idealised situation of a plume rising from a point source of buoyancy alone-in particular the momentum flux at the source is zero. In practical situations, real sources of heating and cooling in a ventilation system often include initial fluxes of both buoyancy and momentum, e.g. where a heating system vents warm air into a space. This paper describes laboratory experiments to determine the dependence of the 'front' formation and stratification on the source momentum and buoyancy fluxes of a single source, and on the location and relative strengths of two sources from which momentum and buoyancy fluxes were supplied separately. For a single source with a non-zero input of momentum, the rate of descent of the front is more rapid than for the case of zero source momentum flux and increases with increasing momentum input. Increasing the source momentum flux effectively increases the height of the enclosure, and leads to enhanced overturning motions and finally to complete mixing for highly momentum-driven flows. Stratified flows may be maintained by reducing the aspect ratio of the enclosure. At these low aspect ratios different long-time behaviour is observed depending on the nature of the heat input. A constant heat flux always produces a stratified interior at large times. On the other hand, a constant temperature supply ultimately produces a well-mixed space at the supply temperature. For separate sources of momentum and buoyancy, the developing stratification is shown to be strongly dependent on the separation of the sources and their relative strengths. Even at small separation distances the stratification initially exhibits horizontal inhomogeneity with localised regions of warm fluid (from the buoyancy source) and cool fluid. This inhomogeneity is less pronounced as the strength of one source is increased relative to the other. Regardless of the strengths of the sources, a constant buoyancy flux source dominates after sufficiently large times, although the strength of the momentum source determines whether the enclosure is initially well mixed (strong momentum source) or stably stratified (weak momentum source). © 2001 Elsevier Science Ltd. All rights reserved.

Values and actions in aversion

Model-based and model-free controllers can, in principle, learn arbitrary actions to optimize their behavior, at least those actions that can be expressed and explored. Indeed, these are often referred to as instrumental controllers because their choices are learned to be instrumental for the delivery of desired outcomes. Although this flexibility is very powerful, it comes with an attendant cost of learning. Evolution appears to have endowed everything from the simplest organisms to us with powerful, pre-specified, but inflexible alternatives. These responses are termed Pavlovian, after the famous Russian physiologist and psychologist Pavlov. The responses of the Pavlovian controller are determined by evolutionary (phylogenetic) considerations rather than (ontogenetic) aspects of the contingent development or learning of an individual. These responses directly interact with instrumental choices arising from goal-directed and habitual controllers. This interaction has been studied in a wealth of animal paradigms, and can be helpful, neutral, or harmful, according to circumstance. Although there has been less careful or analytical study of it in humans, it can be interpreted as underpinning a wealth of behavioral aberrations. © 2009 Elsevier Inc. All rights reserved.

Corrugated all-composite sandwich structures. Part 2: Failure mechanisms and experimental programme

A novel corrugated composite core, referred to as a hierarchical corrugation, has been developed and tested experimentally. Hierarchical corrugations exhibit a range of different failure modes depending on the geometrical properties and the material properties of the structures. In order to understand the different failure modes the analytical strength model, developed in part 1 of this paper, was used to make collapse mechanism maps for the different corrugation configurations. If designed correctly, the hierarchical structures can have more than 7 times higher weight specific strength compared to its monolithic counter part. The difference in strength arises mainly from the increase in buckling resistance of the sandwich core members compared to the monolithic version. The highest difference in strength is seen for core configurations with low overall density. As the density of the core increases, the monolithic core members get stockier and more resistant to buckling and thus the benefits of the hierarchical structure reduces. © 2008 Elsevier Ltd. All rights reserved.

Electronic states of a two-dimensional electron system in a lateral superlattice and a perpendicular magnetic field

The electronic state of a two-dimensional electron system (2DES) in the presence of a perpendicular uniform magnetic field and a lateral superlattice (LS) is investigated theoretically. A comparative study is made between a LS induced by a spatial electrostatic potential modulation (referred to as a PMLS) and that induced by a spatial magnetic-field modulation (referred ro asa MMLS). By utilizing a finite-temperature self-consistent Hartree-Fock approximation scheme; the dependence of the electronic state on different system parameters (e.g., the modulation period, the modulation strength, the effective electron-electron interaction strength, the averaged electron density, and the system temperature) is studied in detail. The inclusion of exchange effect is found to bring qualitative changes to the electronic state of a PMLS, leading generally to a nonuniform spin splitting, and consequently the behavior of the electronic state becomes similar to that of a MMLS. The Landau-level coupling is taken into account, and is found to introduce some interesting features not observed before. It is also found that, even in the regime of intermediate modulation strength, the density dependence of the spin splitting of energy levels, either for a PMLS or a MMLS, can be qualitatively understood within the picture of a 2DES in a perpendicular magnetic field with the modulation viewed as a perturbation. [S0163-1829(97)02248-0].

Loess magnetic properties in the Ili Basin and their correlation with the Chinese Loess Plateau

     Over the past two decades, magnetoclimatological studies of loess-paleosol sequences in the Chinese Loess Plateau (CLP) have made outstanding achievements, which greatly promote the understanding of East Asian paleomonsoon evolution, inland aridification of Asia, and past global climate changes. Loess magnetic properties of the CLP have been well studied. In contrast, loess magnetic properties from outside the CLP in China have not been fully understood. We have little knowledge about the magnetic properties of loess in the Ili Basin, an intermontane depression of the Tianshan (or Tien Shan) Mountains. Here, we present the results of rock magnetic measurements of the Ili loess including mass magnetic susceptibility (χ) and anhysteretic remanent magnetization (ARM), high/low temperature dependence of susceptibility (TDS) and hysteresis, as well as X-ray diffraction (XRD) for mineral analysis. Based on the comparison with loess-paleosol sequences in the CLP (hereafter referred to as the Chinese loess), we discuss the possible magnetic susceptibility enhancement mechanism of the Ili loess. The results show that 1) the total magnetic mineral concentration of the Ili loess is far lower than that of the Chinese loess, though they have similar magnetic mineral compositions. The ferrimagnetic minerals in the Ili loess are magnetite and maghemite, and the antiferromagnetic mineral is hematite; XRD analysis also identifies the presence of ilmenite. The ratio of maghemite is lower in the Ili loess than in the Chinese loess, but the ratios of magnetite and hematite are higher in the Ili loess than in the Chinese loess. 2) The granularity of magnetic minerals in the Ili loess, dominated by pseudo-single domain (PSD) and multi-domain (MD) grains, is generally much coarser than that of the Chinese loess. Ultrafine pedogenically-produced magnetic grains have a very limited contribution to the susceptibility enhancement. Rather, PSD and MD particles of magnetite and maghemite are the main contributors to the enhancement of susceptibility in the Ili loess. 3) The susceptibility enhancement mechanism for the Ili loess is complicated and superimposes both a wind velocity/vigor model (Alaskan or Siberian model) and the in situ ultrafine grain pedogenic model; the former might play an important role in the Ili loess. 4) Magnetic susceptibility enhancements of the Ili loess are related not only to the eolian input of the source area, but also to the local climate, landform, and geological background. Therefore, great care should be taken when reconstructing paleoclimate using magnetic susceptibility data from the Ili loess.

Deconfinement phase Transition including perturbative QCD corrections in (proto)neutron star matter

Deconfinement phase transition and neutrino trapping in (proto)neutron star matter are investigated in a chiral hadronic model (also referred to as the FST model) for the hadronic phase (HP) and in the color-flavor-locked (CFL) quark model for the deconfined quark phase. We include a perturbative QCD correction parameter alpha(s) in the CFL quark matter equation of states. It is shown that the CFL quark core with K-0 condensation forms in neutron star matter with the large value of alpha(s). If the small value of alpha(s) is taken, hyperons suppress the CFL quark phase and the HP is dominant in the high-density region of (proto)neutron star matter. Neutrino trapping makes the fraction of the CFL quark matter decrease compared with those without neutrino trapping. Moreover, increasing the QCD correction parameter alpha(s) or decreasing the bag constant B and the strange quark mass m(s) can make the fraction of the CFL quark matter increase, simultaneously, the fraction of neutrino in protoneutron star matter increases, too. The maximum masses and the corresponding radii of (proto)neutron stars are not sensitive to the QCD correction parameter alpha(s).

Antikaon condensation and in-medium kaon and antikaon production in protoneutron stars

Antikaon condensation and kaon and antikaon production in protoneutron stars are investigated in a chiral hadronic model (also referred to as the FST model in this paper). The effects of neutrino trapping on protoneutron stars are analyzed systematically. It is shown that neutrino trapping makes the critical density of K- condensation delay to higher density and (K) over bar (0) condensation not occur. The equation of state (EOS) of (proto)neutron star matter with neutrino trapping is stiffer than that without neutrino trapping, As a result, the maximum masses of (proto)neutron stars with neutrino trapping are larger than those without neutrino trapping. If hyperons are taken into account, antikaon does not form a condensate in (Proto)neutron stars. Meanwhile, the corresponding EOS becomes much softer, and the maximum masses of (proto)neutron stars are smaller than those without hyprons. Finally, our results illustrate that the Q values for K+ and K- production in (proto)neutron stars are not sensitive to neutrino trapping and inclusion of hyperons.

Molecular biotechnology of marine algae in China

Molecular biotechnology of marine algae is referred to as the biotechnology on the identification, modification, production and utilization of marine algal molecules. It involves not only the manipulation of macromolecules such as DNA, RNA and proteins, but also deals with low molecular weight compounds such as secondary metabolites. In the last decade, molecular systematic researches to investigate the relationship and to examine the evolutionary divergence among Chinese marine algae have been carried out by Chinese scientists. For example, RAPD has been widely used in several laboratories to elucidate genetic variations of the reds, such as Porphyra, Gracilaria, Grateloupia and the greens such as Ulva and Enteromorpha. Some important data have been obtained. The study on molecular genetic markers for strain improvement is now in progress. In 1990s, genetic engineering of economic seaweeds such as Laminaria, Undaria, Porphyra, Gracilaria and Grateloupia has been studied in China. For Laminaria japonica, the successfully cultivated kelp in China, a model transformation system has been set up based on the application of plant genetic techniques and knowledge of the algal life history. Progress has been made recently in incorporating a vaccine gene into kelp genome. Evidence has been provided showing the expression of gene products as detectable vaccines. In the present paper, the progress of molecular biotechnological studies of marine algae in China, especially researches on elucidating and manipulating nucleic acids of marine algae, are reviewed.

Origin and Pathway of Equatorial 13 degrees C Water in the Pacific Identified by a Simulated Passive Tracer and Its Adjoint

The origin and pathway of the thermostad water in the eastern equatorial Pacific Ocean, often referred to as the equatorial 13 degrees C Water, are investigated using a simulated passive tracer and its adjoint, based on circulation estimates of a global general circulation model. Results demonstrate that the source region of the 13 degrees C Water lies well outside the tropics. In the South Pacific, some 13 degrees C Water is formed northeast of New Zealand, confirming an earlier hypothesis on the water's origin. The South Pacific origin of the 13 degrees C Water is also related to the formation of the Eastern Subtropical Mode Water (ESTMW) and the Sub-Antarctic Mode Water (SAMW). The portion of the ESTMW and SAMW that eventually enters the density range of the 13 degrees C Water (25.8 < sigma(theta) < 26.6 kg m(-3)) does so largely by mixing. Water formed in the subtropics enters the equatorial region predominantly through the western boundary, while its interior transport is relatively small. The fresher North Pacific ESTMW and Central Mode Water (CMW) are also important sources of the 13 degrees C Water. The ratio of the southern versus the northern origins of the water mass is about 2 to 1 and tends to increase with time elapsed from its origin. Of the total volume of initially tracer-tagged water in the eastern equatorial Pacific, approximately 47.5% originates from depths above sigma(theta) = 25.8 kg m(-3) and 34.6% from depths below sigma(theta) = 26.6 kg m(-3), indicative of a dramatic impact of mixing on the route of subtropical water to becoming the 13 degrees C Water. Still only a small portion of the water formed in the subtropics reaches the equatorial region, because most of the water is trapped and recirculates in the subtropical gyre.

Applications of improved 2-D numerical wave tanks in wave breaking

Song and Banner (2002, henceforth referred to as SB02) used a numerical wave tank (developed by Drimer and Agnon, and further refined by Segre, henceforth referred to as DAS) to study the wave breaking in the deep water, and proposed a dimensionless breaking threshold that based on the behaviour of the wave energy modulation and focusing during the evolution of the wave group. In this paper, two modified DAS models are used to further test the SB02's results, the first one (referred to MDAS1) corrected many integral calculation errors appeared in the DAS code, and the second one (referred to MDAS2) replaced the linear boundary element approximation of DAS into the cubic element on the free surface. Researches show that the results of MDAS1 are the same with those of DAS for the simulations of deep water wave breaking, but, the different values of the wavemaker amplitude, the breaking time and the maximum local average energy growth rate delta(max) for the marginal breaking cases are founded by MDAS2 and MDAS1. However, MDAS2 still satisfies the SB02' s breaking threshold. Furthermore, MDAS1 is utilized to study the marginal breaking case in the intermediate water depth when wave passes over a submerged slope, where the slope is given by 1 : 500, 1 : 300, 1 : 150 or 1 : 100. It is found that the maximum local energy density U increases significantly if the slope becomes steeper, and the delta(max) decreases weakly and increases intensively for the marginal recurrence case and marginal breaking case respectively. SB02's breaking threshold is still valid for the wave passing over a submerged slope gentler than 1 : 100 in the intermediate water depth.

Antioxidant activity of different sulfate content derivatives of polysaccharide extracted from Ulva pertusa (Chlorophyta) in vitro

Polysaccharide extracted from Ulva pertusa (Chlorophyta) is a group of sulfated heteropolysaccharide; for simplicity, the sulfated polysaccharide is referred to as ulvan in this paper. In this study, different sulfate content ulvans were prepared with sulfur trioxide/N,N-diinethylformamide (SO3-DMF) in formamide, and their antioxidant activities were investigated including scavenging activity of superoxide and hydroxyl radicals, reducing Power and metal chelating ability. As expected, we obtained several satisfying results, as follows: firstly, high sulfate content ulvans had more effective scavenging activity on hydroxyl radical than natural ulvan. Secondly, comparing with natural ulvan, high sulfate content ulvans exhibited stronger reducing power. Thirdly, HU4 (sulfate content, 30.8%) and HU5 (sulfate content, 32.8%) showed more pronounce chelating ability on ferrous ion at high concentration than other samples. (c) 2005 Elsevier B.V. All rights reserved.

太阳辐射对热层和电离层变化性的影响

Solar ultraviolet (UV) radiation at wavelengths less than 400 nm is an important source of energy for aeronomic processes throughout the solar system. Solar UV photons are absorbed in planetary atmospheres, as well as throughout the heliosphere, via photodissociation of molecules, photoionization of molecules and atoms, and photoexcitation toexcitation including resonance scattering. In this paper, the solar irradiances data measured by TIMED SEE, as well as the solar proxies such as F10.7 and Mg II, thermosphere neutral density of CHAMP measurements and topside ionospheric plasmas densities from DMSP, are used to analyze solar irradiance effects on the variabilities of the thermosphere and the ionosphere. First, thermosphere densities near 410 km altitude are analyzed for solar irradiance variability effects during the period 2002-2004. Correlations between the densities and the solar irradiances for different spectral lines and wavelength ranges reveal significantly different characteristics. The density correlates remarkably well with all the selected solar irradiances except the lower chromospheric O I (130.4 nm) emission. Among the chosen solar proxies, the Mg II core-to-wing ratio index, EUV (30-120 nm) and F10.7 show the highest correlations with the density for short-term (< ~27 days) variations. For both long- (> ~27 days) and short-term variations, linear correlation coefficients exhibit a decreasing trend from low latitudes towards high latitudes. The density variability can be effectively modeled (capturing 71% of the variance) using multiple solar irradiance indices, including F10.7, SEUV (the EUV 30-120 nm index), and SFUV (the FUV 120-193 nm index), in which a lag time of 1 day was used for both F10.7 and SEUV, and 5 days for SFUV. In our regression formulation SEUV has the largest contribution to the density variation (40%), with the F10.7 having the next largest contribution (32%) and SFUV accounting for the rest (28%). Furthermore, a pronounced period of about 27.2 days (mean period of the Sun's rotation) is present in both density and solar irradiance data of 2003 and 2004, and a pronounced period of about 54.4 days (doubled period of the solar rotation) is also revealed in 2004. However, soft X-ray and FUV irradiances did not present a pronounced 54.4 day period in 2004, in spite of their high correlation with the densities. The Ap index also shows 54-day periodicities in 2004, and magnetic activity, together with solar irradiance, affects the 54-day variation in density significantly. In addition, NRLMSISE00, DTM-2000 and JB2006 model predictions are compared with density measurements from CHAMP to assess their accuracy, and the results show that these models underestimate the response of the thermosphere to variations induced by solar rotation. Next, the equatorial topside ionospheric plasmas densities Ni are analyzed for solar irradiance variability effects during the period 2002-2005. Linear correlations between Ni and the solar irradiances for different wavelength ranges reveal significantly different characteristics. XUV (0-35 nm) and EUV (115-130 nm) show higher correlation with Ni for the long-term variations, whereas EUV (35-115 nm) show higher correlation for the short-term variations. Moreover, partial correlation analysis shows that the long-term variations of Ni are affected by both XUV (0-35 nm) and EUV (35-115 nm), whereas XUV (0-35 nm) play a more important role; the short-term variations of Ni are mostly affected by EUV (35-115 nm). Furthermore, a pronounced period of about 27 days is present in both Ni and solar irradiance data of 2003 and 2004, and a pronounced period of about 54 days is also revealed in 2004. Finally, prompted by previous studies that have suggested solar EUV radiation as a means of driving the semiannual variation, we investigate the intra-annual variation in thermosphere neutral density near 400 km during 2002-2005. The intra-annual variation, commonly referred to as the ‘semiannual variation’, is characterized by significant latitude structure, hemispheric asymmetries, and inter-annual variability. The magnitude of the maximum yearly difference, from the yearly minimum to the yearly maximum, varies by as much as 60% from year to year, and the phases of the minima and maxima also change by 20-40 days from year to year. Each annual harmonic of the intra-annual variation, namely, annual, semiannual, ter-annual and quatra-annual, exhibits a decreasing trend from 2002 through 2005 that is correlated with the decline in solar activity. In addition, some variations in these harmonics are correlated with geomagnetic activity, as represented by the daily mean value of Kp. Recent empirical models of the thermosphere are found to be deficient in capturing most of the latitude dependencies discovered in our data. In addition, the solar flux and geomagnetic activity proxies that we have employed do not capture some latitude and inter-annual variations detected in our data. It is possible that these variations are partly due to other effects, such as seasonal-latitudinal variations in turbopause altitude (and hence O/N2 composition) and ionosphere coupling processes that remain to be discovered in the context of influencing the intra-annual variations depicted here. Our results provide a new dataset to challenge and validate thermosphere-ionosphere general circulation models that seek to delineate the thermosphere intra-annual variation and to understand the various competing mechanisms that may contribute to its existence and variability. We furthermore suggest that the term “intra-annual” variation be adopted to describe the variability in thermosphere and ionosphere parameters that is well-captured through a superposition of annual, semiannual, ter-annual, and quatra-annual harmonic terms, and that “semiannual’ be used strictly in reference to a pure 6-monthly sinusoidal variation. Moreover, we propose the term “intra-seasonal” to refer to those shorter-term variations that arise as residuals from the above Fourier representation.