Molecular genetics and control of iron metabolism in hemochromatosis

Background and Objectives. Hereditary hemochromatosis (HC) is an inborn error of iron metabolism that leads to progressive iron overload. Considerable advances in the knowledge of molecular events in iron metabolism have been recently obtained. These molecular findings, the cloning of the gene responsible for HC (HFE gene) and the results of preliminary studies on the HFE protein prompted us to review this topic. information Sources. The material examined in this review article includes papers and abstracts published in the Journals covered by the Science Citation Index(C) and Medline(C). The authors have been working in the field of HC for several years and have contributed eleven of the quoted papers. State of Art and Perspective. HC is now recognized as the genetic disease characterized by the homozygosity for the Cys --> Tyr substitution at position 282 (C282Y) of the HFE protein. The mutation abolishes the association of the HFE protein with beta(2)-microglobulin (beta(2)M), making the complex unable to gain the cell surface. Thus HC is an example of abnormal trafficking of the corresponding proteins. It Is clear by the analysis of its structure that HFE protein is not an iron transporter itself, but has a regulatory role in iron metabolism. Its peculiar localization in the crypt cells of the small intestine suggests an important role in iron trafficking at this level. However, other proteins are involved in iron uptake, as the recently cloned Nramp2, the first iron transporter discovered in mammalians. Nramp2 has a recognized role both in the intestinal iron uptake and in the iron transport within the erythroblast. The relationships between HFE and Nramp2 are still unexplored. The recent association of HFE gene with transferrin receptor (TfR) in trophoblast cells opens new possibilities on its role in cellular iron uptake. The existence of other forms of genetic iron overload suggests that the scenario of iron proteins is not yet fully defined. Further studies in this field will contribute to our knowledge of iron metabolism regulation in humans. (C)1998, Ferrata Storti Foundation

On the key role of droughts in the dynamics of summer fires in Mediterranean Europe

Summer fires frequently rage across Mediterranean Europe, often intensified by high temperatures and droughts. According to the state-of-the-art regional fire risk projections, in forthcoming decades climate effects are expected to become stronger and possibly overcome fire prevention efforts. However, significant uncertainties exist and the direct effect of climate change in regulating fuel moisture (e.g. warmer conditions increasing fuel dryness) could be counterbalanced by the indirect effects on fuel structure (e.g. warmer conditions limiting fuel amount), affecting the transition between climate-driven and fuel-limited fire regimes as temperatures increase. Here we analyse and model the impact of coincident drought and antecedent wet conditions (proxy for the climatic factor influencing total fuel and fine fuel structure) on the summer Burned Area (BA) across all eco-regions in Mediterranean Europe. This approach allows BA to be linked to the key drivers of fire in the region. We show a statistically significant relationship between fire and same-summer droughts in most regions, while antecedent climate conditions play a relatively minor role, except in few specific eco-regions. The presented models for individual eco-regions provide insights on the impacts of climate variability on BA, and appear to be promising for developing a seasonal forecast system supporting fire management strategies.We thank the European Forest Fire Information System-EFFIS (http://effis.jrc.ec.europa.eu) of the European Commission Joint Research Centre for the fire data. We acknowledge the SPEI data providers (http://sac.csic. es/spei/database.html). Special thanks to Joaquín Bedia, Esteve Canyameras, Xavier Castro and Andrej Ceglar for helpful discussions on the study. This work was partially funded by the Project of Interest “NextData” of the Italian Ministry for Education, University and Research and by the EU H2020 Project 641762 “ECOPOTENTIAL: Improving Future Ecosystem Benefits through Earth Observations”. Ricardo Trigo was supported by IMDROFLOOD funded by Portuguese FCT (WaterJPI/0004/2014).Peer ReviewedPostprint (published version

Search for low instability strip variables in the young open cluster NGC 2516

In this paper we revise and complete the photometric survey of the instability strip of the southern open cluster NGC 2516 published by Antonello and Mantegazza (1986). No variable stars with amplitudes larger than $0^m.02$ were found. However by means of an accurate analysis based on a new statistical method two groups of small amplitude variables have been disentangled: one with periods $< 0^d.25$ (probably $\delta$ Scuti stars) and one with periods $>0^d.025$. The position in the HR diagram and the apparent time-scale may suggest that the stars of the second group belong to a recently discovered new class of variables, named $\gamma$ Dor variables. They certainly deserve further study. We also present a comparison between the results of the photometric survey and the available pointed ROSAT observations of this cluster.Comment: 7 pages, 2 ps figures. Accepted for P.A.S.

The QUAX proposal: a search of galactic axion with magnetic materials

Aim of the QUAX (QUaerere AXion) proposal is to exploit the interaction of cosmological axions with the spin of electrons in a magnetized sample. Their effect is equivalent to the application of an oscillating rf field with frequency and amplitude which are fixed by axion mass and coupling constant, respectively. The rf receiver module of the QUAX detector consists of magnetized samples with the Larmor resonance frequency tuned to the axion mass by a polarizing static magnetic field. The interaction of electrons with the axion-equivalent rf field produces oscillations in the total magnetization of the samples. To amplify such a tiny field, a pump field at the same frequency is applied in a direction orthogonal to the polarizing field. The induced oscillatory magnetization along the polarizing field is measured by a SQUID amplifier operated at its quantum noise level.Comment: 5 pages, Contribution for the proceedings of the TAUP2015, International Conference on Topics in Astroparticle and Underground Physics, 7-11 September 2015, Torino, Ital

A comprehensive study of Cepheid variables in the Andromeda galaxy. Period distribution, blending and distance determination

Extragalactic Cepheids are the basic rungs of the cosmic distance scale. They are excellent standard candles, although their luminosities and corresponding distance estimates can be affected by the particular properties of the host galaxy. Therefore, the accurate analysis of the Cepheid population in other galaxies, and notably in the Andromeda Galaxy (M31), is crucial to obtaining reliable distance determinations. We obtained accurate photometry (in B and V passbands) of 416 Cepheids in M31 over a five year campaign within a survey aimed at the detection of eclipsing binaries. The resulting Cepheid sample is the most complete in M31 and has almost the same period distribution as the David Dunlap Observatory sample in the Milky Way. The large number of epochs (~250 per filter) has permitted the characterisation of the pulsation modes of 356 Cepheids, with 281 of them pulsating in the fundamental mode and 75 in the first overtone. The period-luminosity relationship of the fundamental mode Cepheids has been studied and a new approach has been used to estimate the effect of blending. We find that the blending contribution is as important as the metallicity correction when computing Cepheid distance determinations to M31 (~0.1 mag). Since large amplitude Cepheids are less affected by blending, we have used those with an amplitude A_V>0.8 mag to derive a distance to M31 of (m-M)_0=24.32+/-0.12 mag.Comment: 10 pages, 9 figures; accepted for publication in A&

Energy consumption of electric vehicles: models' estimation using big data (FCD)

Abstract The paper presents a framework to estimate energy consumption of Electric Vehicles (EVs) by combining: (a) the use of models derived from traffic flow theory and from mechanics of locomotion and (b) the great amount of Floating Cara Data (FCD) from available Information and Communications Technology (ICT) devices. Existing energy consumption models may be classified into aggregate vs. disaggregate, according to the level of aggregation of variables related to driver, vehicle, and infrastructure. The proposed models have a hybrid nature: the aggregate component allows to estimating the values of vehicular speed and acceleration on a road link; the disaggregate one allows to estimating the discrete variability of EVs' energy consumption inside a spatial-temporal domain. The energy consumption models are estimated using traffic data extracted from FCD. The proposed framework is structured into four steps: FCD processing, estimation of vehicular speeds and accelerations, estimation of resistance/energy consumption. The framework is applied in a pilot study area, composed by the backward (sub-)urban area of the port of "Porto delle Grazie" of Roccella Jonica (South of Italy). The preliminary results show that the methodology allows relative inexpensive and accurate calculation of EVs' energy consumption and that it can be integrated into Intelligent Transportation System (ITS) applications

Smoke Alarm Ownership in Relation to Socio-Economic Factors

This study focuses on smoke alarm ownership within Christchurch. Two different areas were selected, based on income levels. These two areas, Holmwood and Avon Loop, were then compared to determine if socio-economic factors impact on smoke alarm ownership. This study focused primarily on the relationships between smoke alarm ownership and location, income, education, and home ownership. Other topics discussed include the number of alarms per household, escape plans, people who smoke in the household, heating methods, reasons for non-purchase and other specific relationships that were uncovered during the study. The results between the two areas are significant for various reasons explained in this study