Structural studies of the hipersaline adaptation of proteins belonging to halophilic archaea

183 p.

Two Nuclear Localization Signals in USP1 Mediate Nuclear Import of the USP1/UAF1 Complex

9 p. : il.

DNA Damage and Transcriptional Changes in the Gills of Mytilus galloprovincialis Exposed to Nanomolar Doses of Combined Metal Salts (Cd, Cu, Hg)

[ENG]Aiming at an integrated and mechanistic view of the early biological effects of selected metals in the marine sentinel organism Mytilus galloprovincialis, we exposed mussels for 48 hours to 50, 100 and 200 nM solutions of equimolar Cd, Cu and Hg salts and measured cytological and molecular biomarkers in parallel. Focusing on the mussel gills, first target of toxic water contaminants and actively proliferating tissue, we detected significant dose-related increases of cells with micronuclei and other nuclear abnormalities in the treated mussels, with differences in the bioconcentration of the three metals determined in the mussel flesh by atomic absorption spectrometry. Gene expression profiles, determined in the same individual gills in parallel, revealed some transcriptional changes at the 50 nM dose, and substantial increases of differentially expressed genes at the 100 and 200 nM doses, with roughly similar amounts of up- and down-regulated genes. The functional annotation of gill transcripts with consistent expression trends and significantly altered at least in one dose point disclosed the complexity of the induced cell response. The most evident transcriptional changes concerned protein synthesis and turnover, ion homeostasis, cell cycle regulation and apoptosis, and intracellular trafficking (transcript sequences denoting heat shock proteins, metal binding thioneins, sequestosome 1 and proteasome subunits, and GADD45 exemplify up-regulated genes while transcript sequences denoting actin, tubulins and the apoptosis inhibitor 1 exemplify down-regulated genes). Overall, nanomolar doses of co-occurring free metal ions have induced significant structural and functional changes in the mussel gills: the intensity of response to the stimulus measured in laboratory supports the additional validation of molecular markers of metal exposure to be used in Mussel Watch programs

Design,optimization and in vivo evaluation of non-viral vectors for gene therapy. Applications in ocular disorders

227 págs.

Analysis of SUMOylated proteins using SUMO-traps

6 p. [+ 7 p. Supplementary Information]

Validación de la cuantificación de la concentración hepática de hierro mediante resonancia magnética nuclear de 1-Tesla

Introducción: La medición de la concentración de hierro hepática (CHH) por RM es una técnica no invasiva de gran utilidad en el diagnóstico de los pacientes con sospecha de sobrecarga férrica en hígado. Objetivo:Validar la eficacia de la RM 1 Tesla en la determinación de la (CHH) en pacientes con sospecha de sobrecarga férrica. Validar su capacidad para diagnosticar o descartar la presencia de una CHH sugestiva de hemocromatosis. Pacientes y métodos:Estudio observacional, transversal, con inclusión prospectiva de pacientes consecutivos. De 2002 a 2010 hemos obtenido la CHH estimada mediante RM 1 Tesla (método Gandon) y de RM 1,5 Tesla (método Alústiza), y mediante BH, en 56 pacientes consecutivos (58RM:35/23). Resultados:Grupo RM 1 Tesla: de acuerdo con CHH en BH, 15 pacientes clasificados como normales (<36µmol/g)-la RM valoró correctamente 7; sobreestimó 8-; 15 grupo hemosiderosis (36-80 µmol/g)-RM valoró correctamente 5, sobreestimó 10-; 5 grupo hemocromatosis (>80 µmol/g)-valoró correctamente las 5-. Existió una correlación entre la determinación de la CHH por BH y RM 1 Tesla con r=0.619. Existieron diferencias estadísticamente significativas (p<0.05) entre CHH media por biopsia (53.43/DE45.67/IC95%37.74 a 69.12) y por RM 1 Tesla (76.14/DE47.31/IC95% 60.46 a 92.97), con sobrevaloración por parte de la RM. Grupo RM 1,5 Tesla: de acuerdo con CHH en BH, normal en 14, hemosiderosis en 6 y hemocromatosis en 3. La RM valoró correctamente 6 y sobreestimó 8 en grupo normal; grupo hemosiderosis, 3 correctamente, 3 sobrevalorados; grupo hemocromatosis, valoró correctamente los 3. La correlación entre CHH por BH y RM 1,5 Tesla fue de r=0.815. La CHH media obtenida por BH (69,34/DE152.1/IC95% 3.57 a 135.1 ) y RM 1,5 Tesla (70.43/DE 57.63/IC95% 45.51 a 95.36) no demostraron diferencias significativas (p>0.05). Conclusiones: La determinación de CHH por RM 1 Tesla (método Gandon) es útil para diagnosticar o descartar hemocromatosis y para diagnosticar CHH normal. Existe una importante tendencia a la sobreestimación en pacientes sin y con sobrecarga férrica en la CHH obtenida por RM 1 Tesla. La determinación de CHH por RM 1,5 Tesla (método Alústiza) es superior a la de RM 1 Tesla, aunque también existe una tendencia a sobreestimar. La CHH media obtenida por BH o RM 1,5 Tesla no tuvieron diferencias significativas. En cambio si existieron entre BH y RM 1 Tesla.

Effects of Climate Change on the European Nuclear Power Sector

4 p.

Recursos de combustible en central nuclear de fusión en el País Vasco

[ES]Este trabajo surge de la inquietud del alumno sobre la energía nuclear y mas concretamente sobre la energía de fusión. Esta inquietud se plasma en una recopilación de información sobre los combustibles necesarios , su abundancia y su forma de obtención actual. Analizaremos la posibilidad de implantar alguna de estas plantas de combustible en el País Vasco, junto con un estudio de una posible central nuclear de fusión con la suma de la potencia de cada uno de los reactores de fisión que se encuentran actualmente en funcionamiento en el estado Español. Compararemos las cantidades de combustible necesarias en un año de uranio y el combustible de fusión, así como el coste de construcción de cada una.

Evaluación de dosis de radiación producida por una pérdida de refrigerante en reactor nuclear.

EN]This is a document done to try to see the effect of a nuclear power plant accident. In particular, it studies what would be the radiation doses delivered to the people in the close areas around the power plant of Garoña, if it had suffered from a loss of coolant accident during its normal functioning.

Muscle wasting in myotonic dystrophies: a model of premature aging

Myotonic dystrophy type 1 (DM1 or Steinert's disease) and type 2 (DM2) are multisystem disorders of genetic origin. Progressive muscular weakness, atrophy and myotonia are the most prominent neuromuscular features of these diseases, while other clinical manifestations such as cardiomyopathy, insulin resistance and cataracts are also common. From a clinical perspective, most DM symptoms are interpreted as a result of an accelerated aging (cataracts, muscular weakness and atrophy, cognitive decline, metabolic dysfunction, etc.), including an increased risk of developing tumors. From this point of view, DM1 could be described as a progeroid syndrome since a notable age dependent dysfunction of all systems occurs. The underlying molecular disorder in DM1 consists of the existence of a pathological (CTG) triplet expansion in the 3' untranslated region (UTR) of the Dystrophia ll/Iyotonica Protein Kinase (DMPK) gene, whereas (CCTG)n repeats in the first intron of the Cellular Nucleic acid Binding Protein/Zinc Finger Protein 9 (CNBP/ZNF9) gene cause DM2. The expansions are transcribed into (CUG)n and (CCUG)n-containing RNA, respectively, which form secondary structures and sequester RNA binding proteins, such as the splicing factor muscleblind-like protein (MBNL), forming nuclear aggregates known as foci. Other splicing factors, such as CUGBP, are also disrupted, leading to a spliceopathy of a large number of downstream genes linked to the clinical features of these diseases. Skeletal muscle regeneration relies on muscle progenitor cells, known as satellite cells, which are activated after muscle damage, and which proliferate and differentiate to muscle cells, thus regenerating the damaged tissue. Satellite cell dysfunction seems to be a common feature of both age-dependent muscle degeneration (sarcopenia) and muscle wasting in DM and other muscle degenerative diseases. This review aims to describe the cellular, molecular and macrostructural processes involved in the muscular degeneration seen in DM patients, highlighting the similarities found with muscle aging.

New Nuclear SNP Markers Unravel the Genetic Structure and Effective Population Size of Albacore Tuna (Thunnus alalunga)

In the present study we have investigated the population genetic structure of albacore (Thunnus alalunga, Bonnaterre 1788) and assessed the loss of genetic diversity, likely due to overfishing, of albacore population in the North Atlantic Ocean. For this purpose, 1,331 individuals from 26 worldwide locations were analyzed by genotyping 75 novel nuclear SNPs. Our results indicated the existence of four genetically homogeneous populations delimited within the Mediterranean Sea, the Atlantic Ocean, the Indian Ocean and the Pacific Ocean. Current definition of stocks allows the sustainable management of albacore since no stock includes more than one genetic entity. In addition, short-and long-term effective population sizes were estimated for the North Atlantic Ocean albacore population, and results showed no historical decline for this population. Therefore, the genetic diversity and, consequently, the adaptive potential of this population have not been significantly affected by overfishing.

Low Effort L-i Nuclear Fusion Plasma Control Using Model Predictive Control Laws

One of the main problems of fusion energy is to achieve longer pulse duration by avoiding the premature reaction decay due to plasma instabilities. The control of the plasma inductance arises as an essential tool for the successful operation of tokamak fusion reactors in order to overcome stability issues as well as the new challenges specific to advanced scenarios operation. In this sense, given that advanced tokamaks will suffer from limited power available from noninductive current drive actuators, the transformer primary coil could assist in reducing the power requirements of the noninductive current drive sources needed for current profile control. Therefore, tokamak operation may benefit from advanced control laws beyond the traditionally used PID schemes by reducing instabilities while guaranteeing the tokamak integrity. In this paper, a novel model predictive control (MPC) scheme has been developed and successfully employed to optimize both current and internal inductance of the plasma, which influences the L-H transition timing, the density peaking, and pedestal pressure. Results show that the internal inductance and current profiles can be adequately controlled while maintaining the minimal control action required in tokamak operation.

Efficient Synthesis of Single-Chain Polymer Nanoparticles via Amide Formation

Single-chain technology (SCT) allows the transformation of individual polymer chains to folded/collapsed unimolecular soft nanoparticles. In this work we contribute to the enlargement of the SCT toolbox by demonstrating the efficient synthesis of single-chain polymer nanoparticles (SCNPs) via intrachain amide formation. In particular, we exploit cross-linking between active methylene groups and isocyanate moieties as powerful "click" chemistry driving force for SCNP construction. By employing poly(methyl methacrylate)- (PMMA-) based copolymers bearing beta-ketoester units distributed randomly along the copolymer chains and bifunctional isocyanate cross-linkers, SCNPs were successfully synthesized at r.t. under appropriate reaction conditions. Characterization of the resulting SCNPs was carried out by means of a combination of techniques including size exclusion chromatography (SEC), infrared (IR) spectroscopy, proton nuclear magnetic resonance (H-1 NMR) spectroscopy, dynamic light scattering (DLS), and elemental analysis (EA).