Coexistence of protease sensitive and resistant prion protein in 129VV homozygous sporadic Creutzfeldt-Jakob disease: a case report

Introduction: The coexistence of different molecular types of classical protease-resistant prion protein in the same individual have been described, however, the simultaneous finding of these with the recently described protease-sensitive variant or variably protease-sensitive prionopathy has, to the best of our knowledge, not yet been reported. Case presentation: A 74-year-old Caucasian woman showed a sporadic Creutzfeldt-Jakob disease clinical phenotype with reactive depression, followed by cognitive impairment, akinetic-rigid Parkinsonism with pseudobulbar syndrome and gait impairment with motor apraxia, visuospatial disorientation, and evident frontal dysfunction features such as grasping, palmomental reflex and brisk perioral reflexes. She died at age 77. Neuropathological findings showed: spongiform change in the patient"s cerebral cortex, striatum, thalamus and molecular layer of the cerebellum with proteinase K-sensitive synaptic-like, dot-like or target-like prion protein deposition in the cortex, thalamus and striatum; proteinase K-resistant prion protein in the same regions; and elongated plaque-like proteinase K-resistant prion protein in the molecular layer of the cerebellum. Molecular analysis of prion protein after proteinase K digestion revealed decreased signal intensity in immunoblot, a ladder-like protein pattern, and a 71% reduction of PrPSc signal relative to non-digested material. Her cerebellum showed a 2A prion protein type largely resistant to proteinase K. Genotype of polymorphism at codon 129 was valine homozygous. Conclusion: Molecular typing of prion protein along with clinical and neuropathological data revealed, to the best of our knowledge, the first case of the coexistence of different protease-sensitive prion proteins in the same patient in a rare case that did not fulfill the current clinical diagnostic criteria for either probable or possible sporadic Creutzfeldt-Jakob disease. This highlights the importance of molecular analyses of several brain regions in order to correctly diagnose rare and atypical prionopathies

Dynamics of domain walls in pattern formation under traveling-wave forcing

We study dynamics of domain walls in pattern forming systems that are externally forced by a moving space-periodic modulation close to 2:1 spatial resonance. The motion of the forcing induces nongradient dynamics, while the wave number mismatch breaks explicitly the chiral symmetry of the domain walls. The combination of both effects yields an imperfect nonequilibrium Ising-Bloch bifurcation, where all kinks (including the Ising-like one) drift. Kink velocities and interactions are studied within the generic amplitude equation. For nonzero mismatch, a transition to traveling bound kink-antikink pairs and chaotic wave trains occurs.

Moths behaving like butterflies. Evolutionary loss of long range attractant pheromones in Castniid Moths: A Paysandisia archon model

Background: In the course of evolution butterflies and moths developed two different reproductive behaviors. Whereas butterflies rely on visual stimuli for mate location, moths use the"female calling plus male seduction" system, in which females release long-range sex pheromones to attract conspecific males. There are few exceptions from this pattern but in all cases known female moths possess sex pheromone glands which apparently have been lost in female butterflies. In the day-flying moth family Castniidae ("butterfly-moths"), which includes some important crop pests, no pheromones have been found so far. Methodology/Principal Findings: Using a multidisciplinary approach we described the steps involved in the courtship of P. archon, showing that visual cues are the only ones used for mate location; showed that the morphology and fine structure of the antennae of this moth are strikingly similar to those of butterflies, with male sensilla apparently not suited to detect female-released long range pheromones; showed that its females lack pheromone-producing glands, and identified three compounds as putative male sex pheromone (MSP) components of P. archon, released from the proximal halves of male forewings and hindwings. Conclusions/Significance: This study provides evidence for the first time in Lepidoptera that females of a moth do not produce any pheromone to attract males, and that mate location is achieved only visually by patrolling males, which may release a pheromone at short distance, putatively a mixture of Z,E-farnesal, E,E-farnesal, and (E,Z)-2,13-octadecadienol. The outlined behavior, long thought to be unique to butterflies, is likely to be widespread in Castniidae implying a novel, unparalleled butterfly-like reproductive behavior in moths. This will also have practical implications in applied entomology since it signifies that the monitoring/control of castniid pests should not be based on the use of female-produced pheromones, as it is usually done in many moths.

The mediterranean diet pattern and its main components are associated with lower plasma concentrations of tumor necrosis factor receptor 60 in patients at high risk for cardiovascular disease

Adherence to aMediterranean diet (MD) is associated with a reduced risk of coronary heart disease. However, themolecular mechanisms involved are not fully understood. The aim of this studywas to compare the effects of 2MD with those of a lowfat- diet (LFD) on circulating inflammatory biomarkers related to atherogenesis. A total of 516 participants included in the PreventionwithMediterraneanDiet Studywere randomized into 3 intervention groups [MD supplementedwith virgin olive oil (MD-VOO); MD supplemented with mixed nuts (MD-Nuts); and LFD]. At baseline and after 1 y, participants completed FFQ and adherence to MD questionnaires, and plasma concentrations of inflammatory markers including intercellular adhesion molecule-1(ICAM-1), IL-6, and 2 TNF receptors (TNFR60 and TNFR80) were measured by ELISA. At 1 y, the MD groups had lower plasma concentrations of IL-6, TNFR60, and TNFR80 (P , 0.05), whereas ICAM-1, TNFR60, and TNFR80 concentrations increased in the LFD group (P , 0.002). Due to between-group differences, participants in the 2 MD groups had lower plasma concentrations of ICAM-1, IL-6, TNFR60, and TNFR80 compared to those in the LFD group (P # 0.028). When participants were categorized in tertiles of 1-y changes in the consumption of selected foods, those in the highest tertile of virgin olive oil (VOO) and vegetable consumption had a lower plasma TNFR60 concentration compared with those in tertile 1 (P,0.02).Moreover, the only changes in consumption thatwere associated with 1-y changes in the geometricmean TNFR60 concentrations were those of VOO and vegetables (P = 0.01). This study suggests that a MD reduces TNFR concentrations in patients at high cardiovascular risk.

Functional and evolutionary analysis of DXL1, a non-essential gene encoding a 1-deoxy-D-xylulose 5-phosphate synthase like protein in Arabidopsis thaliana

The synthesis of 1-deoxy-D-xylulose 5-phosphate (DXP), catalyzed by the enzyme DXP synthase (DXS), represents a key regulatory step of the 2-C-methyl-D-erythritol 4-phosphate (MEP) pathway for isoprenoid biosynthesis. In plants DXS is encoded by small multigene families that can be classified into, at least, three specialized subfamilies. Arabidopsis thaliana contains three genes encoding proteins with similarity to DXS, including the well-known DXS1/CLA1 gene, which clusters within subfamily I. The remaining proteins, initially named DXS2 and DXS3, have not yet been characterized. Here we report the expression and functional analysis of A. thaliana DXS2. Unexpectedly, the expression of DXS2 failed to rescue Escherichia coli and A. thaliana mutants defective in DXS activity. Coherently, we found that DXS activity was negligible in vitro, being renamed as DXL1 following recent nomenclature recommendation. DXL1 is targeted to plastids as DXS1, but shows a distinct expression pattern. The phenotypic analysis of a DXL1 defective mutant revealed that the function of the encoded protein is not essential for growth and development. Evolutionary analyses indicated that DXL1 emerged from DXS1 through a recent duplication apparently specific of the Brassicaceae lineage. Divergent selective constraints would have affected a significant fraction of sites after diversification of the paralogues. Furthermore, amino acids subjected to divergent selection and likely critical for functional divergence through the acquisition of a novel, although not yet known, biochemical function, were identified. Our results provide with the first evidences of functional specialization at both the regulatory and biochemical level within the plant DXS family.

Nontypable Haemophilus influenzae displays a prevalent surface structure molecular pattern in clinical isolates.

Non-typable Haemophilus influenzae (NTHi) is a Gram negative pathogen that causes acute respiratory infections and is associated with the progression of chronic respiratory diseases. Previous studies have established the existence of a remarkable genetic variability among NTHi strains. In this study we show that, in spite of a high level of genetic heterogeneity, NTHi clinical isolates display a prevalent molecular feature, which could confer fitness during infectious processes. A total of 111 non-isogenic NTHi strains from an identical number of patients, isolated in two distinct geographical locations in the same period of time, were used to analyse nine genes encoding bacterial surface molecules, and revealed the existence of one highly prevalent molecular pattern (lgtF+, lic2A+, lic1D+, lic3A+, lic3B+, siaA−, lic2C+, ompP5+, oapA+) displayed by 94.6% of isolates. Such a genetic profile was associated with a higher bacterial resistance to serum mediated killing and enhanced adherence to human respiratory epithelial cells.

Capitalism, unemployment and the transition to the contemporary pattern of growth

A new model of unemployment based on an idea of Marx is presented and used to interpret the development of the British economy from the beginning of capitalism to the present. It is shown that unemployment may be created purposely by capitalists in order to weaken the bargaining position of the workers. This mechanism leads to complex temporal pattern of unemployment and can explain why wages took almost a century and a half to react to the growing capital to labour ratio that characterised early British capitalism.

Fabricació de Lents Zonals de Fresnel per aplicació als raigs X tous

Estudi elaborat a partir d’una estada al Paul Scherrer Institut del Maig a l’Octubre del 2006 amb l’ajuda i supervisió dels Dr. Konstantins Jefimovs i Dr. Christian David. Focalitzar raigs X tous és una necessitat essencial per al microanàlisis, la microscopia, i fer imatges en moltes Instal·lacions de Radiació Sincrotró. Les Lents Zonals de Fresnel (FZP, de la denominació anglesa “Fresnel Zone Plates”) han demostrat donar uns punts focals amb una resolució espacial destacada i una baixa il·luminació de fons. Tanmateix, la fabricació de FZP és complexa i no totalment reproduïble. A més a més, el temps de vida de les FZP és força curt, ja que estant situades sobre membranes de nitrur de silici molt fines i altament absorbents. Per tant, hem fet esforços per implementar FZP de silici, que s’espera que siguin més resistents. L’element està fet d’una oblia de cristall de silici poc absorbent, i no presenta cap interfase entre materials. Així doncs, aquestes lents són especialment adequades per a aguantar les extremes càrregues de radiació de les fonts de raigs X més brillants. Particularment, això és molt important per a les aplicacions a les pròximes generacions de fonts de raigs X, com els Làsers d’Electrons Lliures (FEL, de la denominació anglesa “Free Electron Laser”). El silici també garanteix que no hi hagi cap banda d’absorció en el rang d’energies de la finestra de l’aigua (200-520 eV), fent aquestes lents ideals per a fer imatges de mostres biològiques. En aquest informe, hi ha una descripció detallada de tots els passos involucrats en la fabricació de les Lents Zonals de Fresnel de silici. En resum, les estructures de FZP es modelen sobre una resina utilitzant litografia per feix d’electrons i llavors el patró es transmet al silici mitjançant un gravat d’ions reactius (RIE, de la denominació anglesa ‘Reactive Ion Etching’) utilitzant una fina (20 nm) màscara de Crintermitja. Les membranes de silici es poden aprimar després de la fabricació de les estructures per a garantir una transmissió suficient fins i tot a baixes energies. Aquest informe també inclou l’anàlisi i la discussió d’alguns experiments preliminars per avaluar el rendiment de les Si FZPs fets a la línia de llum PolLux del Swiss Ligth Source amb l’ajuda dels Dr. Jörg Raabe i Dr. George Tzvetkov.

New FFT/IFFT Factorizations with Regular Interconnection Pattern Stage-to-Stage Subblocks

Les factoritzacions de la FFT (Fast Fourier Transform) que presenten un patró d’interconnexió regular entre factors o etapes son conegudes com algorismes paral·lels, o algorismes de Pease, ja que foren originalment proposats per Pease. En aquesta contribució s’han desenvolupat noves factoritzacions amb blocs que presenten el patró d’interconnexió regular de Pease. S’ha mostrat com aquests blocs poden ser obtinguts a una escala prèviament seleccionada. Les noves factoritzacions per ambdues FFT i IFFT (Inverse FFT) tenen dues classes de factors: uns pocs factors del tipus Cooley-Tukey i els nous factors que proporcionen la mateix patró d’interconnexió de Pease en blocs. Per a una factorització donada, els blocs comparteixen dimensions, el patró d’interconnexió etapa a etapa i a més cada un d’ells pot ser calculat independentment dels altres.

Algebraic properties of isochronous centers of polynomial quadratic-like Hamiltonian systems

Projecte de recerca elaborat a partir d’una estada a la School of Mathematics and Statistics de la University of Plymouth, United Kingdom, entre abril juliol del 2007.Aquesta investigació és encara oberta i la memòria que presento constitueix un informe de la recerca que estem duent a terme actualment. En aquesta nota estudiem els centres isòcrons dels sistemes Hamiltonians analítics, parant especial atenció en el cas polinomial. Ens centrem en els anomenats quadratic-like Hamiltonian systems. Diverses propietats dels centres isòcrons d'aquest tipus de sistemes van ser donades a [A. Cima, F. Mañosas and J. Villadelprat, Isochronicity for several classes of Hamiltonian systems, J. Di®erential Equations 157 (1999) 373{413]. Aquell article estava centrat principalment en el cas en que A; B i C fossin funcions analítiques. El nostre objectiu amb l'estudi que estem duent a terme és investigar el cas en el que aquestes funcions són polinomis. En aquesta nota formulem una conjectura concreta sobre les propietats algebraiques que venen forçades per la isocronia del centre i provem alguns resultats parcials.

Applications of the MuSIASEM approach to study changes in the metabolic pattern of Catalonia

This document presents an integrated analysis of the performance of Catalonia based on an analysis of how the energy consumption (measured at the societal level for the Catalan Society) is used within both the productive sectors of the economy and the household, to generate added value, jobs, and to guarantee a given level of material standard of living to the population. The trends found in Catalonia are compared to the trends of other European Countries to contextualize the performance of Catalonia with respect to other societies that have followed different paths of economic development. The first part of the document consists of the Multi-Scale Integrated Analysis of Societal and Ecosystem Metabolism (MuSIASEM) approach that has been used to provide this integrated analysis of Catalan Society across different scales (starting from an analysis of the specific sectors of the Catalan economy as an Autonomous Community and scaling up to an intra-regional (European Union 14) comparison) and across different dimensions of analyses of energy consumption coupled with added value generation. Within the scope of this study, we observe the various trajectories of changes in the metabolic pattern for Catalonia and the EU14 countries in the Paid Work Sectors composed of namely, the Agricultural Sector, the Productive Sector and the Services and Government Sector also in comparison with the changes in the household sector. The flow intensities of the exosomatic energy and the added value generated for each specific sector are defined per hour of human activity, thus characterized as exosomatic energy (MJ/hour) (or Exosomatic Metabolic Rate) and added value (€/hour) (Economic Labour Productivity) across multiple levels. Within the second part of the document, the possible usage of the MuSIASEM approach to land use analyses (using a multi-level matrix of categories of land use) has been conducted.

Assessing the quality of alternative energy sources: Energy Return On the Investment (EROI), the metabolic pattern of societies and energy statistics

This paper presents an initial challenge to tackle the every so "tricky" points encountered when dealing with energy accounting, and thereafter illustrates how such a system of accounting can be used when assessing for the metabolic changes in societies. The paper is divided in four main sections. The first three, present a general discussion on the main issues encountered when conducting energy analyses. The last section, subsequently, combines this heuristic approach to the actual formalization of it, in quantitative terms, for the analysis of possible energy scenarios. Section one covers the broader issue of how to account for the relevant categories used when accounting for Joules of energy; emphasizing on the clear distinction between Primary Energy Sources (PES) (which are the physical exploited entities that are used to derive useable energy forms (energy carriers)) and Energy Carriers (EC) (the actual useful energy that is transmitted for the appropriate end uses within a society). Section two sheds light on the concept of Energy Return on Investment (EROI). Here, it is emphasized that, there must already be a certain amount of energy carriers available to be able to extract/exploit Primary Energy Sources to thereafter generate a net supply of energy carriers. It is pointed out that this current trend of intense energy supply has only been possible to the great use and dependence on fossil energy. Section three follows up on the discussion of EROI, indicating that a single numeric indicator such as an output/input ratio is not sufficient in assessing for the performance of energetic systems. Rather an integrated approach that incorporates (i) how big the net supply of Joules of EC can be, given an amount of extracted PES (the external constraints); (ii) how much EC needs to be invested to extract an amount of PES; and (iii) the power level that it takes for both processes to succeed, is underlined. Section four, ultimately, puts the theoretical concepts at play, assessing for how the metabolic performances of societies can be accounted for within this analytical framework.

Like milk or wine: Does firm performance improve with age?

Our empirical literature review shows that little is known about how firm performance changes with age, presumably because of the paucity of data on firm age. For Spanish manufacturing firms, we analyse the firm performance related to firm age between 1998 and 2006. We find evidence that firms improve with age, because ageing firms are observed to have steadily increasing levels of productivity, higher profits, larger size, lower debt ratios, and higher equity ratios. Furthermore, older firms are better able to convert sales growth into subsequent growth of profits and productivity. On the other hand, we also found evidence that firm performance deteriorates with age. Older firms have lower expected growth rates of sales, profits and productivity, they have lower profitability levels (when other variables such as size are controlled for), and also that they appear to be less capable to convert employment growth into growth of sales, profits and productivity.

Primordial Perturbations in Einstein-Aether theories

El nostre objectiu es l'estudi d'extensions de la Relativitat General i, en particular, estem interessats en les teories que continguin camps vectorials addicionals. En aquests tipus de teories es necessari imposar que el vector ha de tenir norma fixa per evitar la presència d'un fantasma o grau de llibertat amb terme cinètic negatiu, i això implica que la simetria Lorentz està trencada espontàniament. El camp del aether només interactua gravitatòriament i la seva presència es difícil de detectar, no obstant això, durant inflació les fluctuacions del buit a escales petites d'un camp lleuger pot deixar una empremta en observables com les anisotropies del fons de radiació de microones. Les fluctuacions del Einstein-aether es comporten com els camps sense massa i això fa que inflació generi modes de longitud de ona llarga en els sectors escalar i vectorial. Hem estudiat la signatura del Einstein-aether dins l'espectre de pertorbacions primordials lluny del límit de de Sitter de inflació. Aquests modes escalars i vectorials poden deixar una empremta significativa en la radiació de fons de microones en funció dels paràmetres del model. Les observacions del fons de radiació de microones imposen restriccions fenomenològiques que redueixen els límits existents per aquesta classe de teoria. Amb aquest estudi del aether també esperem millorar el coneixement que tenim de una classe més ampla de teories que exhibeixen el mateix tipus de trencament de simetria.