Human autopsy or biopsy tissue samples, mouse models and cell cultures of various types represent the most common materials in the investigation of cell pathogenesis of inherited diseases. This dissertation is devoted to all these approaches in the study of two X-linked lysosomal storage diseases, Fabry disease (FD,α-galactosidase A (AGAL) deficiency) and mucopolysaccharidosis type II (MPSII, idunorate-2- sulfatase (IDS) deficiency). The primary goal of the work was analysis of lipid blood group B antigens with terminal α-galactose (B-GSL) in the pancreas of FD patients with blood group B (FD-B).,In addition to the main glycosphingolipid (GSL) substrate, globotriaosylceramide (Gb3Cer), B-GSLs represent another minor substrate of AGAL. The deposition of undegraded B-GSL has been demonstrated in FD-B pancreas where it was significantly higher than in other organs such as the kidneys and lungs which accumulate mainly Gb3Cer. High concentration of lipid and non-lipid B-antigens was primarily confirmed in exocrine acinar epithelial cells of FD-B, accompanied by massive accumulation of ceroid (secondary sign of lysosomal storage). Unlike acini, the endocrine portion of the pancreas remained unaffected by accumulation of AGAL substrates. This interesting phenomenon of cell biology shows how a specific...

Made available in DSpace on 2014-12-17T14:54:58Z (GMT). No. of bitstreams: 1 DaviBB_DISSERT_1-70.pdf: 4567680 bytes, checksum: 150ff5afc1806ca374278b4c00a1f5a3 (MD5) Previous issue date: 2010-12-06 / Coordena??o de Aperfei?oamento de Pessoal de N?vel Superior / Metamaterials exhibiting negative refraction have attracted a great amount of attention in recent years mostly due to their exquisite electromagnetic properties. These materials are artificial structures that exhibit characteristics not found in nature. It is possible to obtain a metamaterial by combining artificial structures periodically. We investigated the unique properties of Split Ring Resonators, High impedance Surfaces and Frequency Selective Surfaces and composite metamaterials. We have successfully demonstrated the practical use of these structures in antennas and circuits. We experimentally confirmed that composite metamaterial can improve the performance of the structures considered in this thesis, at the frequencies where electromagnetic band gap transmission takes place

No description available.

Fabry Disease is an X-­‐linked lysosomal storage disorder characterized by a variety of symptoms including hypohydrosis, seizures, cardiac abnormalities, skin lesions, and chronic pain. These symptoms stem from a lack of functional endogenous α-­‐ Galactosidase A (α-­GAL), which leads to an accrual of its natural substrate. The severity of the disease symptoms can be directly correlated with the amount of residual enzyme activity. It has been shown that an imino sugar, 1-deoxygalactonojirimycin (DGJ), can increase enzymatic activity and clear excess substrate. This pH-­‐dependent chaperoning phenomenon is believed to arise from the presence of aspartic acid 170 in the active site. This key residue may become protonated at lower pH, preventing a buried salt bridge from being formed. We mutated this residue to an alanine, abolishing activity, and making traditional assays impractical. We have measured the KD of chaperone for this modified active site through crystallography. Previous crystallographic studies on this enzyme have also shown a preliminary second binding site on the surface of α-­Galactosidase that prefers the β-­Galactose anomer. When β-­Galactose binds it buries a greater surface area than when α‐Galactose binds to the active site. Binding of this site by a small molecule should stabilize the native state of the enzyme, but would be sterically occluded from inhibiting active site. We have probed this second site by soaking crystals of α‐Galactosidase with a small library of compounds.

No description available.

<p>The fiber-to-the-x (FTTX) has been widely investigated as a leading access technology to meet the ever growing demand for bandwidth in the last mile. The passive optical network (PON) provides a cost-effective and durable solution. In this thesis, we investigate different aspects of the PON, in the search for cost-effective and high-performance designs of link system and devices.</p> <p>In Chapter 2, we propose a novel upstream link scheme for optical orthogonal frequency division multiplexing (OOFDM)-PON. The colorless laser diodes are used at the optical network units (ONUs), and the overlapped channel spectrum of orthogonal subcarrier multiplexing provides high spectral efficiency. At the optical line terminal (OLT), optical switch and all optical fast Fourier transform (OFFT) are adopted for high speed demultiplexing. The deterioration caused by the laser perturbation is also investigated.</p> <p>In Chapter 3, we design a novel polarization beam splitter (PBS), which is one of the most important components in polarization-controlled optical systems, e.g. the next-generation PON utilizing polarization multiplexing. Our PBS is built on a slab waveguide platform where the light is vertically confined. Planar lenses are formed to collimate and refocus light beam by converting the phase front of the beam. A planar subwavelength grating of a wedge shape induces the form birefringence, where the transverse electric (TE) and transverse magnetic (TM) waves have different effective refractive indices, and are steered to distinct directions. This design provides low insertion loss (< 0.9 dB) and low crosstalk (< -30 dB) for a bandwidth of 100 nm in a compact size, and can be realized by different material systems for easy fabrication and/or monolithic integration with other optical components.</p> <p>In Chapter 4, we study the mode partition noise (MPN) characteristics of the Fabry-Perot (FP) laser diode using the time-domain simulation of noise-driven multi-mode laser rate equation. FP laser is cheaper than the widely used distributed feedback (DFB) laser diode in PON, but its MPN is the major limiting factor in an optical transmission system. We calculate the probability density functions for each longitudinal mode. We also investigate the k-factor, which is a simple yet important measure of MPN. The sources of the k-factor are studied with simulation, including the intrinsic source of the laser Langevin noise, and the extrinsic source of the bit pattern.</p> / Doctor of Philosophy (PhD)

[EN] The aim of this work is to demonstrate theoretically and experimentally how acoustic wave propagation can be controlled by temperature gradients. Starting with the simplest case of two hot wires in air the study extends over periodic structures known as Sonic Crystals (SCs). The Finite Elements Method (FEM) has been employed to perform numerical simulations in order to demonstrate collimation and focusing effect of acoustic waves in two-dimensional (2D) SC whose filling fraction is adjusted by temperature gradients. As a part of the research, Bragg reflection and Fabry-Perot type of acoustic effects are investigated for the proposed type of SC. As example, a SC with desired transmittance can be tailored. Also, gradient index (GRIN) 2D sonic lenses are studied. Using parallel rows of heated wires whose temperatures vary according to a prefixed gradient index law a GRIN lens can be designed with a given performance. Moreover, by changing the temperature of the wires a change in the filling fraction inside the GRIN SC can be achieved. Thus, the local refraction index, which is directly related to the filling fraction, is changed too and an index gradient variation inside the GRIN SC is obtained. This GRIN SC is a direct analogy of gradient media observed in nature. Like their optical counterparts, the investigated 2D GRIN SC lenses have flat surfaces and are easier for fabrication than curved SC lenses. The bending of sound waves obtained by GRIN acoustics structures can be used to focusing and collimating acoustic waves. Another aspect of this work is about tuning some SC properties as effective refractive index, effective mass density, etc. in order to obtain a SC with prefixed properties. Since active tuning of the phononic band gaps is certainly desirable for future applications with enhanced functionalities, few attempts have been made to develop tunable SCs thus far. By controlling the incident angle or operating frequency, a GRIN SC can dynamically adjust the curved trajectory of acoustic wave propagation inside the SC structure. Among the last studies of tunable SCs, the filling fractions were tuned either by direct physical deformation of the structure or external stimuli. The former is impractical for most applications and the latter often requires very strong stimuli to produce only modest adjustment. In this work another way to tune the SC properties is proposed. Hot and cold media have different density, speed of sound, refractive index, etc. in comparison with the same properties at normal conditions, so inserting temperature gradients inside the medium can be used to tune the SC properties in certain limits. The proposed way to obtain temperature gradients inside SC is by wires made of Nicrom which are heated by electrical currents. There are some important advantages of this method. First, changing the electrical current intensity through the wires the SC properties can be changed dynamically. Second, it is relatively easier to change the filling fraction simply by adjusting the current intensity than physically changing the structure or applying strong electric or magnetic fields. In conclusion, the method proposed in this thesis allows us, in principle, to get materials and structures with dynamically adjustable acoustic properties using the temperature control through electric current in the wires, within certain limits. Thus, it is easy to carry out experiments of wave propagation phenomena in a macroscopic scale similar to those that occur in microscopic structures for the propagation of electromagnetic waves of high frequency (microwaves and light). / [ES] El objetivo de este trabajo es demostrar teoréticamente y experimentalmente como la propagación de ondas acústicas puede ser controlada por gradientes de temperatura. Empezando con el caso más simple de dos hilos calientes en aire, el estudio se extiende sobre estructuras periódicas conocidas como cristales sónicos (CS). Se ha utilizado el Método de Elementos Finitos (FEM) para realizar simulaciones numéricas con el objetivo de demonstrar la colimación y focalización de ondas acústicas en CS bidimensionales (2D) cuya fracción de llenado es ajustable mediante gradientes de temperatura. Como parte de la investigación se ha analizado la reflexión de Bragg y el efecto de tipo Fabry-Perot asociados con los CSs estudiados. Entre los ejemplos tratados figuran un CS con una transmitancia ajustable a voluntad, dentro de ciertos límites. También se han estudiado lentes acústicas bidimensionales de gradiente de índice, basadas en gradiente de temperatura. Utilizando cortinas paralelas de hilos calientes cuya temperatura varía según una ley dada se puede diseñar una lente GRIN con propiedades determinadas. Por otra parte, cambiando la temperatura de los hilos se puede lograr un cambio en la fracción de llenado dentro del GRIN CS. Así, el índice de refracción local, que está directamente relacionado con la fracción de llenado, se cambia también y se obtiene una variación de gradiente de índice dentro del GRIN CS. Este GRIN CS es una analogía directa de medios con gradiente, observados en la naturaleza. Otro aspecto de este trabajo trata sobre el ajuste de algunas propiedades de un SC como el índice de refracción efectivo o la densidad efectiva con el objetivo de obtener unas propiedades deseadas del cristal. Como el ajuste activo de los bandgaps fonónicos es ciertamente deseado para futuras aplicaciones con funcionalidades mejoradas, hasta ahora se han hecho varios intentos de desarrollar CSs de características ajustables. Controlando el ángulo de incidencia o la frecuencia de funcionamiento, un GRIN CS puede ajustar dinámicamente la curvatura de la trayectoria de propagación dentro de la estructura CS. Entre los últimos estudios de CSs las fracciones de llenado se ajustaron mediante una deformación física directa de la estructura o mediante estímulos externos (por ejemplo campos eléctricos o magnéticos). El primero es poco práctico para una gran parte de las aplicaciones y el segundo a menudo requiere estímulos muy fuertes para ajustes modestos. En este trabajo se propone otra forma de ajustar las propiedades de un CS. Las propiedades acústicas del medio de propagación (densidad, índice de refracción) dependen de la temperatura, por tanto, introduciendo gradientes de temperatura dentro de dicho medio pueden ajustarse a voluntad las propiedades del CS dentro de ciertos límites. La manera de obtener gradientes de temperatura dentro del CS, propuesta en este estudio, es mediante hilos de nicrom calentados con corrientes eléctricas. Hay algunas ventajas importantes de este método. En primer lugar, cambiando la intensidad de corriente eléctrica que circula por los hilos se puede conseguir cambiar dinámicamente las propiedades del CS. En segundo lugar, es relativamente más fácil de cambiar la fracción de llenado simplemente ajustando la intensidad de la corriente eléctrica que modificar físicamente la estructura o aplicar fuertes campos eléctricos o magnéticos. En conclusión, el método propuesto en esta tesis permite, en principio, conseguir materiales y estructuras con propiedades acústicas ajustables dinámicamente mediante el control de la temperatura a través de la corriente eléctrica en los hilos, dentro de ciertos límites. De esta forma se puede experimentar fácilmente a escala macroscópica fenómenos de propagación de ondas análogos a los que ocurren en estructuras microscópicas para la propagación de ondas electromagnéticas de alta frecuencia (microondas y l / [CA] L'objectiu d'este treball és demostrar teorèticament i experimentalment com la propagació d'ones acústiques pot ser controlada per gradients de temperatura. Començant amb el cas més simple de dos fils calents en aire, l'estudi s'estén sobre estructures periòdiques conegudes com a cristalls sónics (CS) . S'ha utilitzat el Mètode d'Elements Finits (FEM) per a realitzar simulacions numèriques amb l'objectiu de demonstrar la col¿limació i focalització d'ones acústiques en CS bidimensionals (2D) la fracció de omplit de la qual és ajustable per mitjà de gradients de temperatura. Com a part de la investigació s'ha analitzat la reflexió de Bragg i l'efecte de tipus Fabry-Perot associats amb els CSs estudiats. Entre els exemples tractats figuren un CS amb una transmitancia ajustable a voluntat, dins de certs límits. També s'han estudiat lents acústiques bidimensionals de gradient d'índex, basades en gradient de temperatura. Utilitzant cortines paral¿leles de fils calents la temperatura de la qual varia segons una llei donada es pot dissenyar una lent GRIN amb propietats determinades. D'altra banda, canviant la temperatura dels fils es pot aconseguir un canvi en la fracció d'ompliment dins del GRIN CS. Així, l'índex de refracció local, que està directament relacionat amb la fracció d'ompliment, es canvia també i s'obté una variació de gradient d'índex dins del GRIN CS. Este GRIN CS és una analogia directa de mitjans amb gradient, observats en la naturalesa. Com les seues analogies òptiques, les lents, estudiades en este treball, tenen les superfícies planes i són més fàcils de fabricar que les lents corbades. La deflexión de les ones acústiques obtinguda per mitjà d'una lent de gradient GRIN es pot utilitzar per a focalitzar o colimar feixos de so. Un altre aspecte d'este treball tracta sobre l'ajust d'algunes propietats d'un SC com l'índex de refracció efectiu o la densitat efectiva amb l'objectiu d'obtindre unes propietats desitjades del cristall. Com l'ajust actiu dels bandgaps fonónicos és certament desitjat per a futures aplicacions amb funcionalitats millorades, fins ara s'han fet diversos intents de desenrotllar CSs de característiques ajustables. Controlant l'angle d'incidència o la freqüència de funcionament, un GRIN CS pot ajustar dinàmicament la curvatura de la trajectòria de propagació dins de l'estructura CS. Entre els últims estudis de CSs les fraccions d'ompliment es van ajustar per mitjà d'una deformació física directa de l'estructura o per mitjà d'estímuls externs. El primer és poc pràctic per a una gran part de les aplicacions i el segon sovint requerix estímuls molt forts per a ajustos modestos. En este treball es proposa una altra forma d'ajustar les propietats d'un CS. Les propietats acústiques del mig de propagació (densitat, índex de refracció) depenen de la temperatura, per tant, introduint gradients de temperatura dins del dit mitjà poden ajustar-se a voluntat les propietats del CS dins de certs límits. La manera d'obtindre gradients de temperatura dins del CS, proposta en este estudi, és per mitjà de fils de Nicrom calfats amb corrents elèctrics. Hi ha alguns avantatges importants d'este mètode. En primer lloc, canviant la intensitat de corrent elèctric que circula pels fils es pot aconseguir canviar dinàmicament les propietats del CS. En segon lloc, és relativament més fàcil de canviar la fracció d'ompliment simplement ajustant la intensitat del corrent elèctric que modificar físicament l'estructura o aplicar forts camps elèctrics o magnètics. En conclusió, el mètode proposat en esta tesi permet, en principi, aconseguir materials i estructures amb propietats acústiques ajustables dinàmicament per mitjà del control de la temperatura a través del corrent elèctric en els fils, dins de certs límits. D'esta manera es pot experimentar fàcilment a escala macroscòpica fenòmens de propagació d'ones anàlegs a què ocorren e / Ivanov Angelov, M. (2016). Sound Scattering by Lattices of Heated Wires [Tesis doctoral]. Universitat Politècnica de València. https://doi.org/10.4995/Thesis/10251/63275

Ultra-thin solar cells that incorporate earth-abundant and non-toxic materials are promising candidates in the endeavor toward sustainable energy harvesting. Methods to counteract the inevitable low absorption of thinner semiconductor layers are of high interest and have raised considerable attention in the research society. In an attempt to increase the absorption of these types of assemblies, optical coupling effects between the localized surface plasmon resonances (LSPR) of disordered Au nanostructures and a Fabry-Pérot cavity were studied using a previously established absorber/spacer/reflector stack. The disordered Au array was fabricated by evaporating a thin Au film on a substrate with a 55 nm SiO2 dielectric spacer and a 100 nm Al reflecting film, followed by thermal annealing. Nominal Au film thicknesses in the range of 5-25 Å and annealing temperatures of 200-500 oC were investigated. In situ spectroscopic ellipsometry measurements during the subsequent atomic layer deposition (ALD) of tin monosulfide (SnS) allowed analysis of how the optical properties of the SnS/Au absorber layer changed as a function of the growing SnS layer thickness. By employing the Transfer Matrix Method with the estimated optical properties from the in situ analysis, the absorptance of the absorber/spacer/reflector stacks was simulated as a function of the spacer thickness, revealing any signs of the characteristic anti-crossing behavior. It was discovered that a nominal Au film thickness of 25 Å, annealed at 450 oC, and coated with a SnS film of ∼13 nm primed toward the π-phase, resulted in strong optical coupling between the cavity mode and the LSPR. The energy difference at the avoided crossing in the specular reflectance measurement gave an estimated Rabi-splitting energy of 537 meV. This corresponded to about 40% of the original LSPR energy, placing itself within the ultra-strong coupling regime. To evaluate the relevance of the thin-layered structure in photovoltaic applications, more advanced computational methods are required to estimate the useful absorption that occurs in the SnS layer. Nevertheless, these results elucidate the realization of strong optical coupling effects between disordered Au nanostructures and a Fabry-Pérot cavity, and further the possibility of using scalable fabrication methods for this type of ultra-thin absorber/spacer/reflector stack.

Cette thèse s'inscrit dans le contexte de la forte évolution du réseau d'abonnées sur fibre optique (FTTH). Elle a l'objectif d'étudier les solutions d'émetteurs dits "achromatiques" à base de nouveaux composants optoélectroniques pour les réseaux d'accès optiques multiplexés en longueurs d'onde (WDM-PON). Ces émetteurs doivent être peu chers et identiques chez tout abonné quelque soit la longueur d'onde de fonctionnement. Deux types d'émetteurs achromatiques à base de modulateur réflectif sont étudiés dans cette thèse : le laser Fabry-Perot verrouillé par injection optique (IL-FP) et le modulateur électro-absorbant amplifié en réflexion (R-EAM-SOA). Les caractéristiques de l'IL-FP en régime de verrouillage sont évaluées en termes de bruit d'intensité, de modulation, de facteur de couplage phase-amplitude,... La caractérisation tant statique que dynamique est aussi effectuée pour le second composant R-EAM-SOA. D'autres fonctionnalités de ce composant comme la détection directe et la régénération promettent son grand potentiel pour la réalisation d'un dispositif multifonctionnel pour le réseau d'accès. Il est également proposé d'utiliser pour la première fois, le laser à bâtonnets quantiques à blocage de modes (QD-MLL) comme source d'injection multi-longueurs d'onde cohérentes pour l'accès optique WDM. Il est montré qu'il est possible de supprimer le bruit de partition de modes associé à ces structures à blocage de modes. Finalement l'intégration de ces trois composants dans des architectures WDM-PON est réalisée. La faisabilité des systèmes WDM-PON à 2,5 Gb/s à base des IL-FP injectés par une source QD-MLL est évaluée pour différentes configurations et architectures réseau. La montée en débit à 10 Gb/s est prouvée en utilisant le R-EAM-SOA. Un système WDM-PON bidirectionnel avec une capacité de 240 (24 x 10) Gb/s en voie descendante et 60 (24 x 2,5) Gb/s en voie montante a été démontré en utilisant ces solutions d'émetteurs achromatiques proposées. Une des principales sources de dégradation du système WDM-PON bidirectionnel est la rétrodiffusion de Rayleigh pour laquelle le bruit interférométrique produit est étudié. L'impact de ce bruit sur les performances en transmission du système WDM-PON est aussi analysé.

Made available in DSpace on 2016-12-12T20:27:37Z (GMT). No. of bitstreams: 1 Yujuan Wang.pdf: 1608760 bytes, checksum: 9cb82304a2c95c2b0e357dc7f8449815 (MD5) Previous issue date: 2012-12-17 / Coordenação de Aperfeiçoamento de Pessoal de Nível Superior / This work presents the development of an FPGA-based optical interrogation system of multiplexed FBG sensors. The Fabry-Perot filter is used to implement the tunable filter method, as a demodulator for FBG sensors. The tuning signal generation for Fabry-Perot filter and the data aquisition are implemented and syncronized em FPGA. Peak-detection algorithms, based on centroid and FIR filter, are implemented em FPGA. The peak displacement detected of FBG reflected spectrum is used to refer sensed elements, such as variation of temperature and refractive index. Besides, other auxiliary digital circuits are implemented for the system configuration, visulization of the detected peak information and the serial communication to monitor entire spectra in computer. All these digital circuits are working on their own duty at the same time, synchronised by the crystal clock of FPGA. The system was used to monitor the fabrication process of an refractive index sensor. The fabricated sensor was tested by measuring the refractive index of water and sucrose. In another experiment, the system was tested by measuring the temperature of a water sample. In addition, a method to calibrate the tuning signal generator is proposed, which can also be employed in other applications that uses DA converters. Finally, the developed system was packaged to facilitate its transportation. / Este trabalho descreve a implementação de um sistema de interrogação óptico para FBGs multiplexadas, com o controle dos processos implementado em FPGA. A interrogação das FBGs é realizada por meio do método de filtro sintonizável, com o uso de filtro Fabry-Perot. Um FPGA é utilizado para gerar sinal de sintonia para o filtro Fabry-Perot e realizar a leitura do sinal espectral refletido pelas FBGs. Algoritmos de detecção de pico (centroide e filtro FIR) foram implementados no FPGA para processar os sinais refletidos por cada FBG, com o objetivo de inferir as propriedades sensoreadas, como variação de temperatura ou índice de refração. Além destes circuitos digitais, são implementados outros circuitos digitais para a configuração do sistema, para a visualização do pico detectado do sinal espectral e para o monitoramento do espectro inteiro via comunicação serial. Todos circuitos cumprem a própria função paralelamente em virtude do paralelismo do FPGA, sincronizado pelo clock do oscilador cristal do FPGA. O sistema desenvolvido foi utilizado no monitoramento do processo de fabricação de sensor de índice de refração e então na leitura do índice de refração de amostras de sacarose e água. O sistema também foi utilizado para a leitura da temperatura de amostras de água, onde foi estimada a sensibilidade e a resolução do sistema. Também foi proposto um método para a calibração do sistema, que pode ser utilizado também em outros trabalhos. O sistema final foi colocado em uma maleta para facilitar o seu transporte para o local de uso.