293 resultados para Xeroderma pigmentoso (XP)
Resumo:
The gap junction protein connexin37 (Cx37) plays an important role in cell-cell communication in the vasculature. Cx37 is expressed in endothelial cells, platelets and megakaryocytes. We have recently shown that Cx37 limits thrombus propensity by permitting intercellular signaling between aggregating platelets. Here, we have performed high throughput phage display to identify potential binding partners for the regulatory intracellular C-terminus of Cx37 (Cx37CT). We retrieved 2 consensus binding motifs for Cx37CT: WHK...[K,R]XP... and FH-K...[K,R]XXP.... Sequence alignment against the NCBI protein database indicated 66% homology of one the selected peptides with FVIII B-domain. We performed cross-linking reactions using BS3 and confirmed that an 11-mer peptide of the FVIII B-domain sequence linked to recombinant Cx37CT. In vitro binding of this peptide to Cx37CT was also confirmed by surface plasmon resonance. The dissociation constant of FVIII B-domain peptides to Cx37CT was ~20 uM. Other peptide sequences, designed upstream or downstream of the FVIII B-domain sequence, showed very low or no affinity for Cx37CT. Finally, in vivo studies revealed that thrombin generation in platelet-poor plasma from Cx37-/- mice (endogenous thrombin potential: 634±11 nM min, mean±SEM) was increased compared to Cx37+/+ mice (427±12, P<0.001). Moreover, partial activated thromboplastin time (aPTT) was shorter in Cx37-/- (39.7±1.5 s) than in Cx37+/+ mice (45.9±1.8, P=0.03), whereas prothrombin time was comparable. The shorter aPTT in Cx37-/- mice correlated with higher circulating FVIII activity (46.0±0.7 vs. 53.5±2.7 s for Cx37+/+, P=0.03). Overall, our data show for the first time a functional interaction between FVIII and Cx37. This interaction may be relevant for the control of FVIII secretion and, thereby, in the regulation of levels of FVIII circulating in blood. In addition, these results may open new perspectives to improve the efficiency of recombinant FVIII manufacturing.
Resumo:
La aplicación CABIRA (Calidad Biológica de los Ríos Altoandinos) está diseñada para el cálculo de métricas representativas de la calidad biológica de la comunidad de los macroinvertebrados acuáticos que viven en los ríos altoandinos (por encima de los 2000 m.s.n.m.) y muy especialmente para el cálculo del índice multimétrico IMEERA (Índice Multimétrico de Estado Ecológico de Ríos Altoandinos) (Villamarín et al., 2013). Esta aplicación informática ha sido desarrollada por el grupo de investigación F.E.M. (Freshwater Ecology and Management) del Departamento de Ecología de la Universitat de Barcelona (España) bajo la dirección del profesor Narcís Prat.
Resumo:
Two portable Radio Frequency IDentification (RFID) systems (made by Texas Instruments and HiTAG) were developed and tested for bridge scour monitoring by the Department of Civil and Environmental Engineering at the University of Iowa (UI). Both systems consist of three similar components: 1) a passive cylindrical transponder of 2.2 cm in length (derived from transmitter/responder); 2) a low frequency reader (~134.2 kHz frequency); and 3) an antenna (of rectangular or hexagonal loop). The Texas Instruments system can only read one smart particle per time, while the HiTAG system was successfully modified here at UI by adding the anti-collision feature. The HiTAG system was equipped with four antennas and could simultaneously detect 1,000s of smart particles located in a close proximity. A computer code was written in C++ at the UI for the HiTAG system to allow simultaneous, multiple readouts of smart particles under different flow conditions. The code is written for the Windows XP operational system which has a user-friendly windows interface that provides detailed information regarding the smart particle that includes: identification number, location (orientation in x,y,z), and the instance the particle was detected.. These systems were examined within the context of this innovative research in order to identify the best suited RFID system for performing autonomous bridge scour monitoring. A comprehensive laboratory study that included 142 experimental runs and limited field testing was performed to test the code and determine the performance of each system in terms of transponder orientation, transponder housing material, maximum antenna-transponder detection distance, minimum inter-particle distance and antenna sweep angle. The two RFID systems capabilities to predict scour depth were also examined using pier models. The findings can be summarized as follows: 1) The first system (Texas Instruments) read one smart particle per time, and its effective read range was about 3ft (~1m). The second system (HiTAG) had similar detection ranges but permitted the addition of an anti-collision system to facilitate the simultaneous identification of multiple smart particles (transponders placed into marbles). Therefore, it was sought that the HiTAG system, with the anti-collision feature (or a system with similar features), would be preferable when compared to a single-read-out system for bridge scour monitoring, as the former could provide repetitive readings at multiple locations, which could help in predicting the scour-hole bathymetry along with maximum scour depth. 2) The HiTAG system provided reliable measures of the scour depth (z-direction) and the locations of the smart particles on the x-y plane within a distance of about 3ft (~1m) from the 4 antennas. A Multiplexer HTM4-I allowed the simultaneous use of four antennas for the HiTAG system. The four Hexagonal Loop antennas permitted the complete identification of the smart particles in an x, y, z orthogonal system as function of time. The HiTAG system can be also used to measure the rate of sediment movement (in kg/s or tones/hr). 3) The maximum detection distance of the antenna did not change significantly for the buried particles compared to the particles tested in the air. Thus, the low frequency RFID systems (~134.2 kHz) are appropriate for monitoring bridge scour because their waves can penetrate water and sand bodies without significant loss of their signal strength. 4) The pier model experiments in a flume with first RFID system showed that the system was able to successfully predict the maximum scour depth when the system was used with a single particle in the vicinity of pier model where scour-hole was expected. The pier model experiments with the second RFID system, performed in a sandbox, showed that system was able to successfully predict the maximum scour depth when two scour balls were used in the vicinity of the pier model where scour-hole was developed. 5) The preliminary field experiments with the second RFID system, at the Raccoon River, IA near the Railroad Bridge (located upstream of 360th street Bridge, near Booneville), showed that the RFID technology is transferable to the field. A practical method would be developed for facilitating the placement of the smart particles within the river bed. This method needs to be straightforward for the Department of Transportation (DOT) and county road working crews so it can be easily implemented at different locations. 6) Since the inception of this project, further research showed that there is significant progress in RFID technology. This includes the availability of waterproof RFID systems with passive or active transponders of detection ranges up to 60 ft (~20 m) within the water–sediment column. These systems do have anti-collision and can facilitate up to 8 powerful antennas which can significantly increase the detection range. Such systems need to be further considered and modified for performing automatic bridge scour monitoring. The knowledge gained from the two systems, including the software, needs to be adapted to the new systems.
Resumo:
This manual describes how to use the Iowa Bridge Backwater software. It also documents the methods and equations used for the calculations. The main body describes how to use the software and the appendices cover technical aspects. The Bridge Backwater software performs 5 main tasks: Design Discharge Estimation; Stream Rating Curves; Floodway Encroachment; Bridge Backwater; and Bridge Scour. The intent of this program is to provide a simplified method for analysis of bridge backwater for rural structures located in areas with low flood damage potential. The software is written in Microsoft Visual Basic 6.0. It will run under Windows 95 or newer versions (i.e. Windows 98, NT, 2000, XP and later).
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Photons participate in many atomic and molecular interactions and changes. Recent biophysical research has shown the induction of ultraweak photons in biological tissue. It is now established that plants, animal and human cells emit a very weak radiation which can be readily detected with an appropriate photomultiplier system. Although the emission is extremely low in mammalian cells, it can be efficiently induced by ultraviolet light. In our studies, we used the differentiation system of human skin fibroblasts from a patient with Xeroderma Pigmentosum of complementation group A in order to test the growth stimulation efficiency of various bone growth factors at concentrations as low as 5 ng/ml of cell culture medium. In additional experiments, the cells were irradiated with a moderate fluence of ultraviolet A. The different batches of growth factors showed various proliferation of skin fibroblasts in culture which could be correlated with the ultraweak photon emission. The growth factors reduced the acceleration of the fibroblast differentiation induced by mitomycin C by a factor of 10-30%. In view that fibroblasts play an essential role in skin aging and wound healing, the fibroblast differentiation system is a very useful tool in order to elucidate the efficacy of growth factors.
Resumo:
Aquest projecte es va realitzar per a un client que disposa d’un conjunt d’uns 100 ordinadors per a accés a internet amb accés temporitzat per monedes, ubicats en diferents locals, I havia observat una creixent demanda del servei d’impressió, entre d'altres per a fer el checkin per a ryanair.L'objecte d'aquest projecte és crear un sistema que permeti realitzar el cobrament de les impressions de forma prèvia i automàtica, alhora que clara per al client final. Aquest sistema ha de funcionar de forma autònoma, alliberant així al personal del local de les tasques de gestió de la impressora.Cal tenir en compte que aquest sistema ha de funcionar tant per a Linux com per a Windows XP o superior.Es dissenyarà l’electrònica i el software corresponent al servidor d’ impressió,així com les comunicacions entre el servidor d’impressió i els ordinadors per monedes. Quant als ordinadors controlats per monedes, s'implantarà la comunicació amb el moneder, per tal de controlar el crèdit disponible i descomptar el temps corresponent a les impressions. Per altre banda, es realitzarà una interfície d'usuari on es comunicarà a l’usuari el preu de les impressions, el temps que se li restarà, el que té disponible i el que li restaràdesprés de realitzar la impressió. En aquesta mateixa pantalla se li donarà la opció d'acceptar o rebutjar la impressió abans que li sigui descomptada del temps disponible
Resumo:
The software development industry is constantly evolving. The rise of the agile methodologies in the late 1990s, and new development tools and technologies require growing attention for everybody working within this industry. The organizations have, however, had a mixture of various processes and different process languages since a standard software development process language has not been available. A promising process meta-model called Software & Systems Process Engineering Meta- Model (SPEM) 2.0 has been released recently. This is applied by tools such as Eclipse Process Framework Composer, which is designed for implementing and maintaining processes and method content. Its aim is to support a broad variety of project types and development styles. This thesis presents the concepts of software processes, models, traditional and agile approaches, method engineering, and software process improvement. Some of the most well-known methodologies (RUP, OpenUP, OpenMethod, XP and Scrum) are also introduced with a comparison provided between them. The main focus is on the Eclipse Process Framework and SPEM 2.0, their capabilities, usage and modeling. As a proof of concept, I present a case study of modeling OpenMethod with EPF Composer and SPEM 2.0. The results show that the new meta-model and tool have made it possible to easily manage method content, publish versions with customized content, and connect project tools (such as MS Project) with the process content. The software process modeling also acts as a process improvement activity.
Resumo:
Yrityksillä on toimipisteissään käytössä lähiverkkoja, joiden toivotaan olevan yhtey-dessä toisiinsa, jostoimipisteitä on useita. Työntekijöiden etätyömahdollisuudet tai ainakin järjestelmien ylläpito edellyttävät toimivia etäkäyttöyhteyksiä näihin järjes-telmiin.Työn teoriaosassa tutustutaan lähiverkkojen yhdistämis- ja etäkäyttötapoihin. Käytännön osassa toteutetaan toimipisteiden lähiverkkojen yhdistäminen Internetinkautta VPN-yhteyksillä sekä työntekijöiden etäkäyttöyhteydet Microsoft Windows 2003 Serverin RRAS-palvelulla ja Windows XP:n asiakasohjelmalla. Erityispiirteenä toteutuksessa oli pienen noin kymmenen hengen yrityksen useat toi-mipisteet, joiden lukumäärä ja sijainti vaihtelevat jonkin verran vuosittain. Lisäksi osassatoimipisteistä oli jo valmiiksi lähiverkkojen yhdistämispalvelu pitkällä sopi-muksella. Toteutettu järjestelmä on kuitenkin sellaisenaan sovelias niin pienemmän kuin isommankin kokonaisuuden toteuttamiseen myös ilman näitä erityispiirteitä.
Resumo:
En esta memoria final se encuentra embebido la investigación realizada para poder generar una aplicación Web que permite registrar los procesos realizados para la producción de leche en el Cantón Cayambe de la provincia de Pichincha en Ecuador, el mismo que gracias a la ayuda del CILEC se pudo llevar a su culminación.En el primer capítulo de este documento se hace una breve introducción donde se profundiza la problemática del proyecto, así mismo se puntualiza los objetivos con los cuales se determina las directrices que dieron la guía al proyecto; en este capítulo también se topa brevemente sobre el estado del arte en el cual se puntualiza sobre los trabajos realizados hasta la actualidad.El segundo capítulo presenta el análisis realizado durante la recolección de requerimientos funcionales, deduciendo la automatización de los mismos, luego en mediante la aplicación de la metodología XP se pudo generar los diagramas que dieron el flujo del sistema. Aquí también se describe la estructura de la base de datos que se va a utilizar dentro de la aplicación. En consecución del diseño del sistema se procede a desarrollar la aplicación descrita en el tercer capítulo, donde se describe brevemente los paquetes creados y las configuraciones pertinentes, así mismo se plantea las pruebas de funcionamiento del sistema. En el cuarto capítulo se muestra los resultados de la aplicabilidad del sistema en función de los módulos determinados del sistema. Por último se expone las conclusiones como las referencias bibliográficas que se usó para el presente documento.
Resumo:
El presente documento recoge el trabajo llevado a cabo en el análisis e investigación de las principales metodologías ágiles que actualmente se usan en el desarrollo ágil del software (SCRUM, XP, Crystal Family y FDD), y del modelo de proceso centrado en el usuario MPIu+a, el cual ha sido desarrollado íntegramente en el grupo de investigación GRIHO como una de sus principales líneas de investigación. Posteriormente, se ha realizado una propuesta de integración entre SCRUM, XP y MPIu+a, con el objetivo de obtener un primer esquema de una única metodología, en la que se integren aquellas metodologías ágiles seleccionadas junto con un modelo de proceso concreto centrado en el usuario.
Resumo:
Tämä diplomityö tutkii turvallisia etäyhteystapoja yritysverkkoihin. Samanaikasesti kuin Internettiin liitetyt langattomat verkot, kuten langattomat lähiverkot ja pakettikytkentäiset matkapuhelinverkot, tulevat yhä yleisemmiksi, mahdollisuus etäyhteksien luomiseen näiden verkkojen kautta tulee yhä suositummaksi. Vaikka tietoverkot kehittyvät, pysyvät niitä uhkaavat yleisluontoiset uhat samoina. IP pohjaiset VPN-verkot ovat sopiva tapa suojata Internetin ylitse tapahtuvia etäyhteksiä. Eri VPN-ratkaisuja on kuitenkin tarjolla laaja valikoima. Oikean tyyppisen VPN-ratkaisun valinta on kriittistä, jotta yrityksen etäyhteystarpeet saadaan täytettyä. Diplomityö esittelee eräänä vaihtoehtona miten Pocket PC 2002 PDA laitetta ja Windows XP Professional käyttöjärjestelmää voidaan käyttää edullisen VPN ratkaisun toteuttamiseen.
Resumo:
Currently there is a vogue for Agile Software Development methods and many software development organizations have already implemented or they are planning to implement agile methods. Objective of this thesis is to define how agile software development methods are implemented in a small organization. Agile methods covered in this thesis are Scrum and XP. From both methods the key practices are analysed and compared to waterfall method. This thesis also defines implementation strategy and actions how agile methods are implemented in a small organization. In practice organization must prepare well and all needed meters are defined before the implementation starts. In this work three different sample projects are introduced where agile methods were implemented. Experiences from these projects were encouraging although sample set of projects were too small to get trustworthy results.
Resumo:
Aplicativo de RExcel que realiza cálculo de provisiones técnicas de prestaciones pendientes para el caso en que el modelo lineal generalizado tiene distribución de Poisson (sobredispersa) junto con función de enlace logarítmica y por lo tanto tiene como caso particular el método Chain-Ladder. Contempla el cálculo de reservas por años de origen, por años de calendario y total. Y permite aplicar fórmulas analíticas o distribuciones estimadas mediante bootstrap.
Resumo:
Background: Trichothiodistrophy (TTD) is a rare autosomal recessive condition that is characterized by a specific congenital hair shaft dysplasia caused by deficiency of sulfur associated with a wide spectrum of multisystem abnormalities. In this article, we study clinical, microscopic, and ultrastructural findings of 20 patients with TTD with the aim to add further insights regarding to this rare condition. Additionally, analyses of our results are compared with those extracted from the literature in order to enhance its comprehensibility. Materials and Methods: Twenty cases of TTD were included: 7 from Mexico and 14 from Spain. Clinical, microscopic, scanning electron microscopy (SEM) studies and X-ray microanalysis (XrMa) were carried out in all of them. Genetic studies were performed in all seven Mexican cases. Patients with xeroderma pigmentosum and xeroderma pigmentosum/TTD-complex were excluded. Results: Cuticular changes and longitudinal crests of the hair shaft were demonstrated. These crests were irregular, disorganized, following the hair longest axis. Hair shaft sulfur deficiency was disposed discontinuously and intermittently rather than uniformly. This severe decrease of sulfur contents was located close to the trichoschisis areas. Only five patients did not show related disturbances. Micro-dolichocephaly was observed in five cases and represented the most frequent facial dysmorphism found. It is also remarkable that all patients with urologic malformations also combined diverse neurologic disorders. Moreover, three Mexican sisters demonstrated the coexistence of scarce pubic vellus hair, developmental delay, onychodystrophy, and maxillar/mandibullar hypoplasia. Conclusions: TTD phenotype has greatly varied from very subtle forms to severe alterations such as neurologic abnormalities, blindness, lamellar ichthyosis and gonadal malformations. Herein, a multisystem study should be performed mandatorily in patients diagnosed with TTD.
Resumo:
Background: Trichothiodistrophy (TTD) is a rare autosomal recessive condition that is characterized by a specific congenital hair shaft dysplasia caused by deficiency of sulfur associated with a wide spectrum of multisystem abnormalities. In this article, we study clinical, microscopic, and ultrastructural findings of 20 patients with TTD with the aim to add further insights regarding to this rare condition. Additionally, analyses of our results are compared with those extracted from the literature in order to enhance its comprehensibility. Materials and Methods: Twenty cases of TTD were included: 7 from Mexico and 14 from Spain. Clinical, microscopic, scanning electron microscopy (SEM) studies and X-ray microanalysis (XrMa) were carried out in all of them. Genetic studies were performed in all seven Mexican cases. Patients with xeroderma pigmentosum and xeroderma pigmentosum/TTD-complex were excluded. Results: Cuticular changes and longitudinal crests of the hair shaft were demonstrated. These crests were irregular, disorganized, following the hair longest axis. Hair shaft sulfur deficiency was disposed discontinuously and intermittently rather than uniformly. This severe decrease of sulfur contents was located close to the trichoschisis areas. Only five patients did not show related disturbances. Micro-dolichocephaly was observed in five cases and represented the most frequent facial dysmorphism found. It is also remarkable that all patients with urologic malformations also combined diverse neurologic disorders. Moreover, three Mexican sisters demonstrated the coexistence of scarce pubic vellus hair, developmental delay, onychodystrophy, and maxillar/mandibullar hypoplasia. Conclusions: TTD phenotype has greatly varied from very subtle forms to severe alterations such as neurologic abnormalities, blindness, lamellar ichthyosis and gonadal malformations. Herein, a multisystem study should be performed mandatorily in patients diagnosed with TTD.