Lack of GDAP1 induces neuronal calcium and mitochondrial defects in a knockout mouse model of charcot-marie-tooth neuropathy.

Mutations in GDAP1, which encodes protein located in the mitochondrial outer membrane, cause axonal recessive (AR-CMT2), axonal dominant (CMT2K) and demyelinating recessive (CMT4A) forms of Charcot-Marie-Tooth (CMT) neuropathy. Loss of function recessive mutations in GDAP1 are associated with decreased mitochondrial fission activity, while dominant mutations result in impairment of mitochondrial fusion with increased production of reactive oxygen species and susceptibility to apoptotic stimuli. GDAP1 silencing in vitro reduces Ca2+ inflow through store-operated Ca2+ entry (SOCE) upon mobilization of endoplasmic reticulum (ER) Ca2+, likely in association with an abnormal distribution of the mitochondrial network. To investigate the functional consequences of lack of GDAP1 in vivo, we generated a Gdap1 knockout mouse. The affected animals presented abnormal motor behavior starting at the age of 3 months. Electrophysiological and biochemical studies confirmed the axonal nature of the neuropathy whereas histopathological studies over time showed progressive loss of motor neurons (MNs) in the anterior horn of the spinal cord and defects in neuromuscular junctions. Analyses of cultured embryonic MNs and adult dorsal root ganglia neurons from affected animals demonstrated large and defective mitochondria, changes in the ER cisternae, reduced acetylation of cytoskeletal α-tubulin and increased autophagy vesicles. Importantly, MNs showed reduced cytosolic calcium and SOCE response. The development and characterization of the GDAP1 neuropathy mice model thus revealed that some of the pathophysiological changes present in axonal recessive form of the GDAP1-related CMT might be the consequence of changes in the mitochondrial network biology and mitochondria-endoplasmic reticulum interaction leading to abnormalities in calcium homeostasis.

Service Architecture in Mobile IP Telephony Network

Uudet palvelut ovat tarkeinta, mita asiakkaat odottavat uudelta teknologialta.Se on paaasiallinen syy siihen, etta asiakkaat ovat valmiita maksamaan uudesta teknologiasta ja kayttamaan sita. Sen vuoksi uuden verkon tuoma uusi palveluarkkitehtuuri on tarkea koko projektin onnistumiselle. Tama dokumentti keskittyy kolmannen sukupolven matkapuhelinverkkojen palveluarkkitehtuuriin, jonka viitemallista annetaan kuvaus. Verkon palvelut esitellaan ja kuvaillaan. Toteutukseen liittyvia asioita selostetaan. USA:n markkinoilla tarvittava WIN konsepti kuvataan ja sen toteutuksesta annetaan myos kuvaus. Lopussa kuvataan Pre-Paid tilaajien laskutustietojen kasittelya WIN konseptissa elvytystilanteessa.

Logistics in Pump Manufacturer's Spare Part Distribution Network

Tässä diplomityössä käsitellään pumppujen maailmanlaajuista varaosa-jakelua. Työn pääpaino on varaosien moniportaisen jakeluverkon toimitusketjun logistiikassa. Asiakaspalvelussa varaosien nopea saatavuus on ratkaisevassa asemassa, jotta asiakkaiden tehtaiden käynnissäpito voidaan turvata. Työn tavoitteena oli kehittää Ahlström Pumpuille uusi globaali toimintamalli varaosien jakeluun. Tämä uusi varaosien jakelumalli on kehitetty pääasiassa sopimusasiakkaille, jotka ovat halukkaita ulkoistamaan varaosatoimintonsa. Mallia sovelletaan käytäntöön varaosien jakelussa ensiksi Suomessa ja Ruotsissa. Myöhemmin on tarkoitus kopioida mallia myös muualle. Malli on pyritty rakentamaan siten, että siihen voidaan helposti liittää muita Ahlström Pumppujen tarjoamia palveluita. Työssä koottu materiaali perustuu pääasiassa haastatteluihin, palavereihin, erilaisiin sisäisiin raportteihin, kirjallisuuteen ja itsenäiseen selvitystyöhön. Työssä on myös tutustuttu kolmen eri yrityksen after-sales- ja varaosatoimintaan.

Social network extraction and analysis based on multimodal dyadic interaction

Social interactions are a very important component in people"s lives. Social network analysis has become a common technique used to model and quantify the properties of social interactions. In this paper, we propose an integrated framework to explore the characteristics of a social network extracted from multimodal dyadic interactions. For our study, we used a set of videos belonging to New York Times" Blogging Heads opinion blog. The Social Network is represented as an oriented graph, whose directed links are determined by the Influence Model. The links" weights are a measure of the"influence" a person has over the other. The states of the Influence Model encode automatically extracted audio/visual features from our videos using state-of-the art algorithms. Our results are reported in terms of accuracy of audio/visual data fusion for speaker segmentation and centrality measures used to characterize the extracted social network.

Effect of radar rainfall time resolution on the predictive capability of a distributed hydrologic model

The performance of a hydrologic model depends on the rainfall input data, both spatially and temporally. As the spatial distribution of rainfall exerts a great influence on both runoff volumes and peak flows, the use of a distributed hydrologic model can improve the results in the case of convective rainfall in a basin where the storm area is smaller than the basin area. The aim of this study was to perform a sensitivity analysis of the rainfall time resolution on the results of a distributed hydrologic model in a flash-flood prone basin. Within such a catchment, floods are produced by heavy rainfall events with a large convective component. A second objective of the current paper is the proposal of a methodology that improves the radar rainfall estimation at a higher spatial and temporal resolution. Composite radar data from a network of three C-band radars with 6-min temporal and 2 × 2 km2 spatial resolution were used to feed the RIBS distributed hydrological model. A modification of the Window Probability Matching Method (gauge-adjustment method) was applied to four cases of heavy rainfall to improve the observed rainfall sub-estimation by computing new Z/R relationships for both convective and stratiform reflectivities. An advection correction technique based on the cross-correlation between two consecutive images was introduced to obtain several time resolutions from 1 min to 30 min. The RIBS hydrologic model was calibrated using a probabilistic approach based on a multiobjective methodology for each time resolution. A sensitivity analysis of rainfall time resolution was conducted to find the resolution that best represents the hydrological basin behaviour.

Sisäinen tulosraportointi tietojohtamisen näkökulmasta. Case: Elcoteq Network Oyj

Tutkielman tarkoituksena oli tutkia, mitkä tekijät vaikuttavat sisäisen laskennan tulostiedon oikeellisuuteen ja miten tietojohtamisen keinoin voidaan taata yhteensopivuus ulkoisen ja sisäisen laskentatiedon välillä. Tekijöiden tunnistamisen ja huomioonottamisen kautta voidaan kehittää organisaation tietämystä ja suorituskykyä. Tutkimus oli empiirinen ja kuvaileva tapaustutkimus. Kvalitatiivista aineistoa kerättiin pääasiassa kyselylomakkeen, havainto -ja dokumenttiaineiston avulla. Aineisto analysoitiin teemoittelun avulla. Laskentaorganisaatioissa havaittiin mekaanisen ja orgaanisen tietopääomaympäristön piirteitä, jotka asettavat tiedon välitykselle,vuorovaikutukselle ja tietämyksen kehittymiselle omat rajoitteensa. Laskentaorganisaatioiden toimintaa ohjasivat mekaaniselle ympäristölle tyypilliset vastuu - ja raportointisuhteet, aikataulut ja raportointikäytännöt. Raportointijärjestelmään tarvittaisiin lisäkoulutusta, tiedon vertailtavuuteen ja tiedon oikeellisuuden takaamiseen lisää työvälineitä. Tiedon välittämistä ja kommunikointia pitäisi tehostaa aktiivisemmalla sähköisellä viestinnällä, mutta myös monipuolisemmalla viestintäkanavien käytöllä. Laskentaorganisaatioiden toimintaympäristössä havaittiin orgaanisen tietopääomaympäristön piirteitä, mikä edellyttäisi dialogia tukevien teknologioiden ja toimintatapojen tehokkaampaa käyttöä. Haasteena on maantieteellisen etäisyyden aiheuttamat rajoitteet sekä toiminnan mekaaninen luonne. Uutta konseptia käyttöönotettaessa keskustelut ja vuorovaikutus tukivat mallin kehittämistä ja käytännön sisäistämistä osaksi organisaation tietämystä. Viestintäkanavien käytön tehostamisella voitaisiin edesauttaa tiedon välittymistä. Tietämyksen ja suorituskyvyn kehittäminen laskentaorganisaatioissa edellyttää kuitenkin vuorovaikutteisuuden ja dialogin tukemista.

A System-Level Simulation Model for a Protocol Processor

As the development of integrated circuit technology continues to follow Moore’s law the complexity of circuits increases exponentially. Traditional hardware description languages such as VHDL and Verilog are no longer powerful enough to cope with this level of complexity and do not provide facilities for hardware/software codesign. Languages such as SystemC are intended to solve these problems by combining the powerful expression of high level programming languages and hardware oriented facilities of hardware description languages. To fully replace older languages in the desing flow of digital systems SystemC should also be synthesizable. The devices required by modern high speed networks often share the same tight constraints for e.g. size, power consumption and price with embedded systems but have also very demanding real time and quality of service requirements that are difficult to satisfy with general purpose processors. Dedicated hardware blocks of an application specific instruction set processor are one way to combine fast processing speed, energy efficiency, flexibility and relatively low time-to-market. Common features can be identified in the network processing domain making it possible to develop specialized but configurable processor architectures. One such architecture is the TACO which is based on transport triggered architecture. The architecture offers a high degree of parallelism and modularity and greatly simplified instruction decoding. For this M.Sc.(Tech) thesis, a simulation environment for the TACO architecture was developed with SystemC 2.2 using an old version written with SystemC 1.0 as a starting point. The environment enables rapid design space exploration by providing facilities for hw/sw codesign and simulation and an extendable library of automatically configured reusable hardware blocks. Other topics that are covered are the differences between SystemC 1.0 and 2.2 from the viewpoint of hardware modeling, and compilation of a SystemC model into synthesizable VHDL with Celoxica Agility SystemC Compiler. A simulation model for a processor for TCP/IP packet validation was designed and tested as a test case for the environment.

Considering tourist perceptions and network configuration in destination management

The competitiveness of tourism destinations is a relevant issue for tourism studies, moreso, is a key element on the daily basis of tourism destinations. In this sense, the management of tourism destinations is essential to maintain competitive advantages. In this article tourism destination is considered as a relational network, where interaction and cooperation is needed among tourist agents, to achieve major levels of competitive advantage and a more effective destination management system. In addition, the perceptions of tourists are obtained from two main sources. The first one is the social construction of a tourism destination previous to the visit and the second one is obtained from the interaction between tourists and tourism destination agents during the visit. In this sense, the management of tourism destination to emit a homogenous and collective image is a factor that can reduce the gap if dissatisfaction from the previous and real tourist perception. The discussion is centered on the relationship within a destination, between the supply network and the targeted demand, considering these two approaches jointly, to benefit destination management. The main result is a conceptual model that shows how tourism agents and tourists in the tourism destination interact to improve the destination competitiveness

Customer driven business model – connecting customer value to firm resources in ICT value networks

The objective of this thesis is to provide a business model framework that connects customer value to firm resources and explains the change logic of the business model. Strategic supply management and especially dynamic value network management as its scope, the dissertation is based on basic economic theories, transaction cost economics and the resource-based view. The main research question is how the changing customer values should be taken into account when planning business in a networked environment. The main question is divided into questions that form the basic research problems for the separate case studies presented in the five Publications. This research adopts the case study strategy, and the constructive research approach within it. The material consists of data from several Delphi panels and expert workshops, software pilot documents, company financial statements and information on investor relations on the companies’ web sites. The cases used in this study are a mobile multi-player game value network, smart phone and “Skype mobile” services, the business models of AOL, eBay, Google, Amazon and a telecom operator, a virtual city portal business system and a multi-play offering. The main contribution of this dissertation is bridging the gap between firm resources and customer value. This has been done by theorizing the business model concept and connecting it to both the resource-based view and customer value. This thesis contributes to the resource-based view, which deals with customer value and firm resources needed to deliver the value but has a gap in explaining how the customer value changes should be connected to the changes in key resources. This dissertation also provides tools and processes for analyzing the customer value preferences of ICT services, constructing and analyzing business models and business concept innovation and conducting resource analysis.

A feed-forward spiking model of shape-coding by IT cells

The ability to recognize a shape is linked to figure-ground (FG) organization. Cell preferences appear to be correlated across contrast-polarity reversals and mirror reversals of polygon displays, but not so much across FG reversals. Here we present a network structure which explains both shape-coding by simulated IT cells and suppression of responses to FG reversed stimuli. In our model FG segregation is achieved before shape discrimination, which is itself evidenced by the difference in spiking onsets of a pair of output cells. The studied example also includes feature extraction and illustrates a classification of binary images depending on the dominance of vertical or horizontal borders.

An Experimental Study on Peer Selection in a P2P Network over PlanetLab

Peer-reviewed

El Turisme de memòria a Catalunya: realitat i perspectives d'un model incipient

Catalunya disposa a l’actualitat de nombrosos equipaments culturals destinats a la difusió memorial mitjançant la pràctica turística creant-se, tanmateix, les primeres estructures en xarxa per tal de fer que aquests espais disposin d’un relat conjunt, element indispensable en el qual recolzar-se a l’hora d’emprendre qualsevol estratègia de difusió. El model català, però, pot dir-se que està en fase embrionària, i des d’aquest moment en endavant ens correspon als professionals i acadèmics treballar de cara a la seva consolidació. Aquest estudi posa les bases per a la investigació en clau turística sobre els Espais de Memòria

Developing a new service business model for an industrial supplier

Project business companies are moving towards solution offering in order to avoid discontinuity of project business and to gain other advantages. An option to implement solution business could be BOOT (Build-Own-Operate-Transfer) business model where a company is given the responsibilities to design, finance, build, own, operate and maintain for example production facilities of a client. The contract is made for 10-30 years, the client pays the solution during this period of time and after contract termination the facilities are transferred to the ownership of the client. The purpose of this study was to provide knowledge about BOOT business model for the company in question and its employees and to create a settlement of the advantages, disadvantages and risks of it. Furthermore, one of the main objectives was to create a description of the network needed to run a BOOT project. The objectives were met through a literature study and an explorative case study with appropriate interviews. Based on this study, the company should be able to evaluate the applicability of BOOT business model to their business environment better.

The maximum voltage drop in an on-chip power distribution network: analysis of square, triangular and hexagonal power pad arrangements

A mathematical model of the voltage drop which arises in on-chip power distribution networks is used to compare the maximum voltage drop in the case of different geometric arrangements of the pads supplying power to the chip. These include the square or Manhattan power pad arrangement, which currently predominates, as well as equilateral triangular and hexagonal arrangements. In agreement with the findings in the literature and with physical and SPICE models, the equilateral triangular power pad arrangement is found to minimize the maximum voltage drop. This headline finding is a consequence of relatively simple formulas for the voltage drop, with explicit error bounds, which are established using complex analysis techniques, and elliptic functions in particular.

DWI and complex brain network analysis predicts vascular cognitive impairment in spontaneous hypertensive rats undergoing executive function tests

The identification of biomarkers of vascular cognitive impairment is urgent for its early diagnosis. The aim of this study was to detect and monitor changes in brain structure and connectivity, and to correlate them with the decline in executive function. We examined the feasibility of early diagnostic magnetic resonance imaging (MRI) to predict cognitive impairment before onset in an animal model of chronic hypertension: Spontaneously Hypertensive Rats. Cognitive performance was tested in an operant conditioning paradigm that evaluated learning, memory, and behavioral flexibility skills. Behavioral tests were coupled with longitudinal diffusion weighted imaging acquired with 126 diffusion gradient directions and 0.3 mm(3) isometric resolution at 10, 14, 18, 22, 26, and 40 weeks after birth. Diffusion weighted imaging was analyzed in two different ways, by regional characterization of diffusion tensor imaging (DTI) indices, and by assessing changes in structural brain network organization based on Q-Ball tractography. Already at the first evaluated times, DTI scalar maps revealed significant differences in many regions, suggesting loss of integrity in white and gray matter of spontaneously hypertensive rats when compared to normotensive control rats. In addition, graph theory analysis of the structural brain network demonstrated a significant decrease of hierarchical modularity, global and local efficacy, with predictive value as shown by regional three-fold cross validation study. Moreover, these decreases were significantly correlated with the behavioral performance deficits observed at subsequent time points, suggesting that the diffusion weighted imaging and connectivity studies can unravel neuroimaging alterations even overt signs of cognitive impairment become apparent.