Permanent correction of an inherited ectodermal dysplasia with recombinant EDA.

X-linked hypohidrotic ectodermal dysplasia (XLHED; OMIM 305100) is a genetic disorder characterized by absence or deficient function of hair, teeth and sweat glands. Affected children may experience life-threatening high fever resulting from reduced ability to sweat. Mice with the Tabby phenotype share many symptoms with human XLHED patients because both phenotypes are caused by mutations of the syntenic ectodysplasin A gene (Eda) on the X chromosome. Two main splice variants of Eda, encoding EDA1 and EDA2, engage the tumor necrosis factor (TNF) family receptors EDAR and XEDAR, respectively. The EDA1 protein, acting through EDAR, is essential for proper formation of skin appendages; the functions of EDA2 and XEDAR are not known. EDA1 must be proteolytically processed to a soluble form to be active. Here, we show that treatment of pregnant Tabby mice with a recombinant form of EDA1, engineered to cross the placental barrier, permanently rescues the Tabby phenotype in the offspring. Notably, sweat glands can also be induced by EDA1 after birth. This is the first example of a developmental genetic defect that can be permanently corrected by short-term treatment with a recombinant protein.

Natural development of stand structure in peatland Scots pine following drainage : results based on long-term monitoring of permanent sample plots

Abstract

Le projet PERMANENT.PLOT.CH demande votre collaboration

Un nouveau projet, baptisé PERMANENT.PLOT.CH, démarre cette année à l'Université de Lausanne. Le but est de répertorier tous les carrés permanents de végétation en Suisse et de les archiver dans une base de donnée informatique. Un appel est lancé à toute personne connaissant l'existence de carrés permanents de végétation, sur le terrain ou dans la litérature, de nous transmettre cette précieuse information.

Influence of the implanted pulse generator as reference electrode in finite element model of monopolar deep brain stimulation.

Electrical deep brain stimulation (DBS) is an efficient method to treat movement disorders. Many models of DBS, based mostly on finite elements, have recently been proposed to better understand the interaction between the electrical stimulation and the brain tissues. In monopolar DBS, clinically widely used, the implanted pulse generator (IPG) is used as reference electrode (RE). In this paper, the influence of the RE model of monopolar DBS is investigated. For that purpose, a finite element model of the full electric loop including the head, the neck and the superior chest is used. Head, neck and superior chest are made of simple structures such as parallelepipeds and cylinders. The tissues surrounding the electrode are accurately modelled from data provided by the diffusion tensor magnetic resonance imaging (DT-MRI). Three different configurations of RE are compared with a commonly used model of reduced size. The electrical impedance seen by the DBS system and the potential distribution are computed for each model. Moreover, axons are modelled to compute the area of tissue activated by stimulation. Results show that these indicators are influenced by the surface and position of the RE. The use of a RE model corresponding to the implanted device rather than the usually simplified model leads to an increase of the system impedance (+48%) and a reduction of the area of activated tissue (-15%).

Toward Synchronous Extensible Dependency Grammar

Extensible Dependency Grammar (XDG; Debusmann, 2007) is a flexible, modular dependency grammarframework in which sentence analyses consist of multigraphs and processing takes the form of constraint satisfaction. This paper shows how XDGlends itself to grammar-driven machine translation and introduces the machinery necessary for synchronous XDG. Since the approach relies on a shared semantics, it resembles interlingua MT.It differs in that there are no separateanalysis and generation phases. Rather, translation consists of the simultaneousanalysis and generation of a single source-target sentence.

Smooth transition or permanent exit? Evidence on job prospects of displaced industrial workers

This article examines the job prospects of displaced industrial workers in Switzerland. Based on a survey of 1,203 workers who were dismissed after their manufacturing plants closed down, we analyse the determinants of re-employment, the sector of re-employment and the change in wages. Two years after displacement, a majority of workers were back in employment: 69% were re-employed, 17% un-employed and 11% retired. Amongst re-employed workers, two thirds found a job in manufacturing and one third in services. Contrary to a common belief, low-end services are not the collecting vessel of redundant industrial workers. Displaced workers aged 55 and older seem particularly vulnerable after a plant closes down: over 30% were long-term unemployed, and those older workers who found a new job suffered disproportionate wage losses. Advanced age-and not low education-appears as the primary handicap after mass redundancy.

Error probability in synchronous digital circuits due to power supply noise

This paper presents a probabilistic approach to model the problem of power supply voltage fluctuations. Error probability calculations are shown for some 90-nm technology digital circuits.The analysis here considered gives the timing violation error probability as a new design quality factor in front of conventional techniques that assume the full perfection of the circuit. The evaluation of the error bound can be useful for new design paradigms where retry and self-recoveringtechniques are being applied to the design of high performance processors. The method here described allows to evaluate the performance of these techniques by means of calculating the expected error probability in terms of power supply distribution quality.

Synchronous Digital Hierarchy implementation to a Radio Transmission Node

Kiristyville markkinoille on aina tuotava jotain uutta tarjottavaa ja SDH-kortti on yksi sellainen tuote jonka Nokia Networks julkistaa osaksi uutta Nokia Flexihub Nodea. Tavoitteena on suunnitella VC-12kanavoitu STM-1 kortti yhdistämään ylemmän tason tietoliikenneverkko suuren kapasiteetin radioon. Ennen kuin markkinoilla on valmis tuote, on sen takana valtaisa määrä työtä. Erilaisia dokumentteja on pitänyt tuottaa ja sopimuksia tehdä. Esimerkiksi vaatimusmäärittelyt on oltava selvät, jotta tiedetään mitä tuotteelta halutaan. Tätä ennen on kuitenkin pitänyt ymmärtää miten SDHtoimii ja miten otsikkotavuja käsitellään. Myös erilaiset piirivalinnat aiheuttavat miettimistä, sillä markkinoilla on runsaasti valmiita piirejä SDH signaalinkäsittelyyn. Varma tiedonsiirto on tärkeää puhelinoperaattorille ja siksi joudutaan miettimään varmennuksia ja niiden toteuttamista. Myös synkronointi on tärkeä osa SDH järjestelmää ja sen toteuttaminen hyvin on tärkeää. Hälytykset on otettava huomioon ja mietittävä, miten niiden käsittely saadaan hoidettua järkevästi, ilman että mikään järjestelmän osa ruuhkautuu kohtuuttomasti. Tässä Diplomityössä on tutustuttu SDH-järjestelmään, otsikkotavujen käsittelyyn ja vaatimusmäärittelyihin.

Measuring the effects through time of the influence of visuomotor and visuotactile synchronous stimulation on a virtual body ownership illusion

Previous studies have examined the experience of owning a virtual surrogate body or body part through specific combinations of cross-modal multisensory stimulation. Both visuomotor (VM) and visuotactile (VT) synchronous stimulation have been shown to be important for inducing a body ownership illusion, each tested separately or both in combination. In this study we compared the relative importance of these two cross-modal correlations, when both are provided in the same immersive virtual reality setup and the same experiment. We systematically manipulated VT and VM contingencies in order to assess their relative role and mutual interaction. Moreover, we present a new method for measuring the induced body ownership illusion through time, by recording reports of breaks in the illusion of ownership ("breaks") throughout the experimental phase. The balance of the evidence, from both questionnaires and analysis of the breaks, suggests that while VM synchronous stimulation contributes the greatest to the attainment of the illusion, a disruption of either (through asynchronous stimulation) contributes equally to the probability of a break in the illusion.

Integrating testcase generator into Eclipse environment

Tämädiplomityö tutkii kuinka Eclipse -ympäristöä voidaan käyttää testitapausten generoinnissa. Eräs diplomityön pääaiheista on tutkia voidaanko olemassa olevilla Eclipsen komponenteilla parantaa symboolitietoutta, jotta testitapausten generointiin saataisiin lisää tietoa. Aluksi diplomityö antaa lyhyen katsauksen ohjelmistojentestaukseen, jotta lukija ymmärtää mitä ohjelmistotekniikan osa-aluetta diplomityö käsittelee. Tämän jälkeen kerrotaan lisää tietoa itse testitapausten generointiprosessista. Kun perusteet on käsitelty, tutustetaan lukija Eclipse -ympäristöön, mikä se on, mistä se koostuu ja mitä sillä voidaan tehdä. Tarkempaa tietoa kerrotaan Eclipsen komponenteista joita voidaan käyttää apuna testitapausten generoinnissa. Integrointi esimerkkinä diplomityössä esitellään valmiin testitapausgeneraattorin integrointi Eclipse -ympäristöön. Lopuksi Eclipse -pohjaista ratkaisua verrataan symboolitietouden sekä ajoajan kannalta aikaisempaan ratkaisuun. Diplomityön tuloksena syntyi prototyyppi jonka avulla todistettiin, että Eclipse - ympäristöön on mahdollista integroida testitapausgeneraattori ja että se voi lisätä symboolitietoutta. Tämätietouden lisäys kuitenkin lisäsi myös tarvittavaa ajoaikaa, joissakintapauksissa jopa merkittävästi. Samalla todettiin, että tällä hetkellä on menossa projekteja joiden tarkoituksena on parantaa käytettyjen Eclipse komponenttien suorituskykyä ja että tämä voi parantaa tuloksia tulevaisuudessa.

Analysis of Active Magnet Bearing Control Software

Problems of the designing active magnet bearingcontrol are developed. The estimation controller are designed and applied to a rigid rotor. The mathematical model of the active magnet bearing controller is developed. This mathematical model is realized on a DSP. The results of this realization are analyzed. The conclusions about the digital signal processing are made.

Exposició permanent Isaac Albéniz: l'últim músic del Romanticisme

Camprodon va rebre un llegat de més de 700 peces relacionades amb el músic Isaac Albéniz. L’any 1999, es decidí crear una fundació a Camprodon amb la intenció de difondre aquest llegat a través d’un museu. El museu no va acabar mai de funcionar per les limitacions d’espai i un projecte inadequat que va ser incapaç de despertar la curiositat dels visitants de Camprodon. Actualment, per manca de recursos econòmics, la rehabilitació del nou espai del Museu Isaac Albéniz es troba completament aturada. Aquest treball proposa la creació d’un projecte d’exposició permanent que es mantingui fins a l’obertura del museu definitiu

A comparative performance study of four-pole induction motors and synchronous reluctance motors in variable speed drives

Design aspects of the Transversally Laminated Anisotropic (TLA) Synchronous Reluctance Motor (SynRM) are studied and the machine performance analysis compared to the Induction Motor (IM) is done. The SynRM rotor structure is designed and manufactured for a30 kW, four-pole, three-phase squirrel cage induction motor stator. Both the IMand SynRM were supplied by a sensorless Direct Torque Controlled (DTC) variablespeed drive. Attention is also paid to the estimation of the power range where the SynRM may compete successfully with a same size induction motor. A technicalloss reduction comparison between the IM and SynRM in variable speed drives is done. The Finite Element Method (FEM) is used to analyse the number, location and width of flux barriers used in a multiple segment rotor. It is sought for a high saliency ratio and a high torque of the motor. It is given a comparison between different FEM calculations to analyse SynRM performance. The possibility to take into account the effect of iron losses with FEM is studied. Comparison between the calculated and measured values shows that the design methods are reliable. A new application of the IEEE 112 measurement method is developed and used especially for determination of stray load losses in laboratory measurements. The study shows that, with some special measures, the efficiency of the TLA SynRM is equivalent to that of a high efficiency IM. The power factor of the SynRM at rated load is smaller than that of the IM. However, at lower partial load this difference decreases and this, probably, brings that the SynRM gets a better power factor in comparison with the IM. The big rotor inductance ratio of the SynRM allows a good estimating of the rotor position. This appears to be very advantageous for the designing of the rotor position sensor-less motor drive. In using the FEM designed multi-layer transversally laminated rotor with damper windings it is possible to design a directly network driven motor without degrading the motorefficiency or power factor compared to the performance of the IM.