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De la protección a la inclusión : las personas con experiencias de adopción, acogimiento familiar y residencial en los centros educativos

Repositorio: Dipòsit Digital de Documents de la UAB
Clemente Martínez, Chandra Kala, Urrea Monclús, Aida, Cordova, Federica de, Selmi, Giulia, Sità, Chiara, López López, Mónica, ten Brumelaar, Mijntje, Zijlstra, Elianne, Linares, Ana Mª, Ruibal, Águeda, Vázquez, Chus, Ferrero, Barbara, Patt, Sandra, Wilkins, Rebecca, Mitchell, Jo, de Haan, Anna, Keij, Marjolein

Este manual ha sido desarrollado en el contexto del proyecto "BRIGTHER FUTURE: Innovative tools for developing full potential after early adversity", financiado por el Programa Erasmus+ de la Comisión Europea y cuyo equipo de trabajo está integrado por las siguientes entidades: Comune di Torino (Italia); CORA (España); PAC UK/ Family Action (Reino Unido); Pharos Expertise Center on Health Disparities (Holanda); Universitat Autònoma de Barcelona (España); Università di Verona (Italia…, Comprender las diferentes trayectorias de vida y situaciones familiares es clave para maestras, maestros, educadoras y educadores cuando los niñas y niños que viven en acogimiento (incluidos las y los migrantes no acompañadas) y las niñas y niños adoptadas. Este manual proporciona información y herramientas útiles para hacer de la escuela un lugar seguro para todas las niñas y niños, independientemente de su situación familiar.



Een portret van Spinoza geschilderd door Sorolla [Un retrato de Spinoza pintado por Sorolla]

Repositorio: E-Prints Complutense. Archivo Institucional de la UCM
Campos Bueno, José Javier

It is reviewed the iconography of Dutch-Sephardic philosopher Baruch Spinoza. It reports on the recent identification of the image of the philosopher found in an anonymous portrait deposited at the Universidad Complutense coming from Simarro Legacy Foundation. Documentation is provided showing that the portrait was painted by Sorolla for the library of Dr. Simarro. Repasa la iconografía del filosofo holandés de origen español-sefardí Baruch Spinoza. Da cuenta de la reciente identificación de…



‘HIJ’ VERSUS ‘ZIJ’: CONTRASTEN, PARALLELLEN EN BEELDSPRAAK IN LAIA FÀBREGAS’ LANDEN (2010), ‘HIM’ VERSUS ‘HER’: CONTRASTS, PARALLELS AND IMAGERY IN LAIA FÀBREGAS’ LANDEN (2010)

Repositorio: RCUB. Revistas Científicas de la Universidad de Barcelona
van Raemdonck, Anne

In this article I shall show how deftly Fàbregas creates in her novel Landen two separate but thematically connected stories and leads them to a very satisfying and totally unexpected ending. Landen is an intriguing psychological novel where the two main characters, Him and Her, tell, in alternating chapters, their own story in the first person. They only meet once, in the first chapter, where the Him character dies. I shall first look at the main characters’ life experiences, i.e. the car…, In this article I shall show how deftly Fàbregas creates in her novel Landen two separate but thematically connected stories and leads them to a very satisfying and totally unexpected ending. Landen is an intriguing psychological novel where the two main characters, Him and Her, tell, in alternating chapters, their own story in the first person. They only meet once, in the first chapter, where the Him character dies. I shall first look at the main characters’ life experiences, i.e. the car…



Lezers, boeken en lectuur van een humanistenkring in de zestiende eeuw: Boekbestellingen door Arias Montano en zijn Sevilliaanse vrienden

Repositorio: RODIN. Repositorio de Objetos de Docencia e Investigación de la Universidad de Cádiz
Dávila Pérez, Antonio

Después de su salida de Amberes, Benito Arias Montano (ca. 1525/27-1598) mantuvo una frecuente correspondencia con la oficina tipográfica de Cristóbal Plantino. Las cartas no se limitaron a intercambiar noticias públicas y privadas; el teólogo español envió muy a menudo listas de títulos de libros que él o su cenáculo de humanistas sevillanos estaban interesados en comprar. Este artículo se centra en quince listas enviadas entre 1585 y 1596 y conservadas en el Museo Plantin-Moretus de Amberes.…



Evidence for a relationship between mitochondrial Complex I activity and mitochondrial aldehyde dehydrogenase during nitroglycerin tolerance: Effects of mitochondrial antioxidants

Repositorio: r-FISABIO. Repositorio Institucional de Producción Científica
Garcia-Bou R, Rocha M, Apostolova N, Herance R, Hernandez-Mijares A, Victor V

The medical use of nitroglycerin (GTN) is limited by patient tolerance. The present study evaluated the role of mitochondrial Complex I in GIN biotransformation and the therapeutic effect of mitochondrial antioxidants. The development of GIN tolerance (in rat and human vessels) produced a decrease in mitochondrial 02 consumption. Co-incubation with the mitochondria-targeted antioxidant mitoquinone (MQ 10(-6) mol/L) or with glutathione ester (GEE, 10(-4) mol/L) blocked GTN tolerance and…



The C-terminal region of the Hot1 transcription factor binds GGGACAAA-related sequences in the promoter of its target genes.

Repositorio: r-FISABIO. Repositorio Institucional de Producción Científica
Gomar-Alba M, Amaral C, Artacho A, D'Auria G, Pimentel C, Rodrigues-Pousada C, lí del Olmo M

Response to hyperosmotic stress in the yeast Saccharomyces cerevisiae involves the participation of the general stress response mediated by Msn2/4 transcription factors and the HOG pathway. One of the transcription factors activated through this pathway is Hot1, which contributes to the control of the expression of several genes involved in glycerol synthesis and flux, or in other functions related to adaptation to adverse conditions. This work provides new data about the interaction mechanism…



BCL(X)L and BCL2 increase mitochondrial dynamics in breast cancer cell: Evidence from functional and genetic studies

Repositorio: r-FISABIO. Repositorio Institucional de Producción Científica
Lucantoni F, Salvucci M, Dussmann H, Prehn J

BCL2 family proteins are important regulators of mitochondrial outer membrane permeabilization (MOMP). In recent years, BCL2 family proteins have also been linked to the regulation of mitochondrial bioenergetics and dynamics. Given their overexpression in breast cancer cells, we sought to explore whether two key members of this family, BCL2 and BCL(X)L impacted on mitochondrial fusion/fission processes. By employing a single cell imaging and RNA sequencing we found that overexpression of BCL2…



Human importin alpha 3 and its N-terminal truncated form, without the importin-beta-binding domain, are oligomeric species with a low conformational stability in solution

Repositorio: r-FISABIO. Repositorio Institucional de Producción Científica
Diaz-Garcia C, Hornos F, Giudici A, Camara-Artigas A, Luque-Ortega J, Arbe A, Rizzuti B, Alfonso C, Forwood J, Iovanna J, Gomez J, Prieto M, Coutinho A, Neira J

Background: Eukaryotic cells have a continuous transit of macromolecules between the cytoplasm and the nucleus. Several carrier proteins are involved in this transport. One of them is importin alpha, which must form a complex with importin beta to accomplish its function, by domain-swapping its 60-residue-long N terminus. There are several human isoforms of importin alpha; among them, importin alpha 3 has a particularly high flexibility. Methods: We studied the conformational stability of…



Dynamics of the intrinsically disordered protein NUPR1 in isolation and in its fuzzy complexes with DNA and prothymosin alpha

Repositorio: r-FISABIO. Repositorio Institucional de Producción Científica
Neira J, Palomino-Schatzlein M, Ricci C, Ortore M, Rizzuti B, Iovanna J

Intrinsically disordered proteins (IDPs) explore diverse conformations in their free states and, a few of them, also in their molecular complexes. This functional plasticity is essential for the function of IDPs, although their dynamics in both free and bound states is poorly understood. NUPR1 is a protumoral multifunctional IDP, activated during the acute phases of pancreatitis. It interacts with DNA and other IDPs, such as prothymosin alpha (ProT alpha), with dissociation constants of similar…



The C-terminal SAM domain of p73 binds to the N terminus of MDM2

Repositorio: r-FISABIO. Repositorio Institucional de Producción Científica
Neira J, Diaz-Garcia C, Prieto M, Coutinho A

Background: The p53, p63 and p73 proteins belong to the p53 family of transcription factors, playing key roles in tumour suppression. The alpha-splice variant of p73 (p73 alpha) has at its C terminus a sterile alpha motif (SAM); this domain, SAMp73, formed by five helices (alpha 1 to alpha 5), is thought to mediate in protein-protein interactions. The E3-ligase MDM2 binds to p73 at its N terminus transactivation domain (TA), but it does not promote its degradation via ubiquitination; however,…



Amphipathic helical peptides hamper protein-protein interactions of the intrinsically disordered chromatin nuclear protein 1 (NUPR1)

Repositorio: r-FISABIO. Repositorio Institucional de Producción Científica
Santofimia-Castano P, Rizzuti B, Abian O, Velazquez-Campoy A, Iovanna J, Neira J

Background: NUPR1 is a multifunctional intrinsically disordered protein (IDP) involved, among other functions, in chromatin remodelling, and development of pancreatic ductal adenocarcinoma (PDAC). It interacts with several biomolecules through hydrophobic patches around residues A1a33 and Thr68. The drug trifluoperazine (TFP), which hampers PDAC development in xenografted mice, also binds to those regions. Because of the large size of the hot-spot interface of NUPR1, small molecules could not…



Small heterodimer partner (SHP) contributes to insulin resistance in cardiomyocytes.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Rodríguez-Calvo R, Chanda D, Oligschlaeger Y, Miglianico M, Coumans WA, Barroso E, Tajes M, Luiken JJ, Glatz JF, Vazquez M, Neumann D

Small heterodimer partner (SHP) is an atypical nuclear receptor expressed in heart that has been shown to inhibit the hypertrophic response. Here, we assessed the role of SHP in cardiac metabolism and inflammation. Mice fed a high-fat diet (HFD) displayed glucose intolerance accompanied by increased cardiac mRNA levels of Shp. In HL-1 cardiomyocytes, SHP overexpression inhibited both basal and insulin-stimulated glucose uptake and impaired the insulin signalling pathway (evidenced by reduced…



High-fat diet-induced deregulation of hippocampal insulin signaling and mitochondrial homeostasis deficiences contribute to Alzheimer disease pathology in rodents.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Petrov D, Pedrós I, Artiach G, Sureda FX, Barroso E, Pallàs M, Casadesús G, Beas-Zarate C, Carro E, Ferrer I, Vazquez M, Folch J, Camins A

Global obesity is a pandemic status, estimated to affect over 2 billion people, that has resulted in an enormous strain on healthcare systems worldwide. The situation is compounded by the fact that apart from the direct costs associated with overweight pathology, obesity presents itself with a number of comorbidities, including an increased risk for the development of neurodegenerative disorders. Alzheimer disease (AD), the main cause of senile dementia, is no exception. Spectacular failure of…



PPARß/d ameliorates fructose-induced insulin resistance in adipocytes by preventing Nrf2 activation.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Barroso E, Rodríguez-Rodríguez R, Chacón MR, Maymó-Masip E, Ferrer L, Salvadó L, Salmerón E, Wabistch M, Palomer FX, Vendrell J, Wahli W, Vazquez M

We studied whether PPARß/d deficiency modifies the effects of high fructose intake (30% fructose in drinking water) on glucose tolerance and adipose tissue dysfunction, focusing on the CD36-dependent pathway that enhances adipose tissue inflammation and impairs insulin signaling. Fructose intake for 8 weeks significantly increased body and liver weight, and hepatic triglyceride accumulation in PPARß/d-deficient mice but not in wild-type mice. Feeding PPARß/d-deficient mice with fructose…



Interrelation of attention and prediction in visual processing: Effects of task-relevance and stimulus probability.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Marzecová A, Widmann A, San-Miguel I, Kotz SA, Schröger E

The potentially interactive influence of attention and prediction was investigated by measuring event-related potentials (ERPs) in a spatial cueing task with attention (task-relevant) and prediction (probabilistic) cues. We identified distinct processing stages of this interactive influence. Firstly, in line with the attentional gain hypothesis, a larger amplitude response of the contralateral N1, and Nd1 for attended gratings was observed. Secondly, conforming to the attenuation-by-…



Early indices of deviance detection in humans and animal models.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Grimm S, Escera C, Nelken I

Detecting unexpected stimuli in the environment is a critical function of the auditory system. Responses to unexpected "deviant" sounds are enhanced compared to responses to expected stimuli. At the human scalp, deviance detection is reflected in the mismatch negativity (MMN) and in an enhancement of the middle-latency response (MLR). Single neurons often respond more strongly to a stimulus when rare than when common, a phenomenon termed stimulus-specific adaptation (SSA). Here we…



Phase re-setting of gamma neural oscillations during novelty processing in an appetitive context.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Domínguez-Borràs J, Garcia-Garcia M, Escera C

Based on the previous study where phase-synchronization (PS) of gamma-band responses (GBRs) proved a reliable cerebral correlate of involuntary attention and its enhancement under threat, we measured gamma-PS elicited by novel sounds from human electroencephalogram (EEG) scalp-recordings when participants responded to visual stimuli displaying either highly motivational or neutral sceneries. We then tested the modulatory effect of the emotional conditions on auditory responses. Novel distractor…



Ultrafast tracking of sound location changes as revealed by human auditory evoked potentials.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Grimm S, Recasens M, Althen H, Escera C

The rapid discrimination of auditory location information enables grouping and selectively attending to specific sound sources. The typical indicator of auditory change detection is the mismatch negativity (MMN) occurring at a latency of about 100-250 ms. However, recent studies have revealed the existence of earlier markers of frequency deviance detection in the middle-latency response (MLR). Here, we measured the MLR and MMN to changes in sound location. Clicks were presented in either the…



A risk variant for alcoholism in the NMDA receptor affects amygdala activity during fear conditioning in humans.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Cacciaglia R, Nees F, Pohlack ST, Ruttorf M, Winkelmann T, Witt SH, Nieratschker V, Rietschel M, Flor H

People at high risk for alcoholism show deficits in aversive learning, as indicated by impaired electrodermal responses during fear conditioning, a basic form of associative learning that depends on the amygdala. A positive family history of alcohol dependence has also been related to decreased amygdala responses during emotional processing. In the present study we report reduced amygdala activity during the acquisition of conditioned fear in healthy carriers of a risk variant for alcoholism (…



Mathematical anxiety effects on simple arithmetic processing efficiency: an event-related potential study.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Suárez-Pellicioni M, Nunez M, Colomé A

This study uses event-related brain potentials to investigate the difficulties that high math anxious individuals face when processing dramatically incorrect solutions to simple arithmetical problems. To this end, thirteen high math-anxious (HMA) and thirteen low math-anxious (LMA) individuals were presented with simple addition problems in a verification task. The proposed solution could be correct, incorrect but very close to the correct one (small-split), or dramatically incorrect (large-…



Less precise representation of numerical magnitude in high math-anxious individuals: an ERP study of the size and distance effects.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Nunez M, Suárez-Pellicioni M

Numerical comparison tasks are widely used to study the mental representation of numerical magnitude. In study, event-related brain potentials (ERPs) were recorded while 26 high math-anxious (HMA) and 27 low math-anxious (LMA) individuals were presented with pairs of single-digit Arabic numbers and were asked to decide which one had the larger numerical magnitude. The size of the numbers and the distance between them were manipulated in order to study the size and the distance effects. The…



Phasic boosting of auditory perception by visual emotion.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Selinger L, Domínguez-Borràs J, Escera C

Emotionally negative stimuli boost perceptual processes. There is little known, however, about the timing of this modulation. The present study aims at elucidating the phasic effects of, emotional processing on auditory processing within subsequent time-windows of visual emotional, processing in humans. We recorded the electroencephalogram (EEG) while participants responded to a, discrimination task of faces with neutral or fearful expressions. A brief complex tone, which subjects, were…



Mitochondrial response to the BCKDK-deficiency: Some clues to understand the positive dietary response in this form of autism

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
De Oyarzabal-Sanz AL, Bravo-Alonso I, Sánchez-Aragó M, Rejas MT, Merinero B, Garcia-Cazorla A, Artuch-Iriberri R, Ugarte M, Rodríguez-Pombo P

Mutations on the mitochondrial-expressed Branched Chain of-Keto acid Dehydrogenase Kinase (BCKDK) gene have been recently associated with a novel dietary-treatable form of autism. But, being a mitochondrial metabolism disease, little is known about the impact on mitochondrial performance. Here, we analyze the mitochondrial response to the BCKDK-deficiency in patient's primary fibroblasts by measuring bioenergetics, ultra structural and dynamic parameters. A two-fold increase in superoxide…



Drug-induced lipotoxicity: lipodystrophy associated with HIV-1 infection and antiretroviral treatment.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Villarroya F, Domingo P, Giralt M

A subset of HIV-1-infected patients undergoing antiretroviral treatment develops a lipodystrophy syndrome. It is characterized by loss of peripheral subcutaneous adipose tissue (face, limbs, buttocks), visceral fat accumulation, and, in some cases, lipomatosis, especially in the dorsocervical area. In addition, these patients show metabolic alterations reminiscent of the metabolic syndrome, particularly dyslipidemia and insulin resistance. These alterations lead to enhanced cardiovascular risk…



Atorvastatin inhibits GSK-3beta phosphorylation by cardiac hypertrophic stimuli.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Planavila A, Rodríguez-Calvo R, Palomer FX, Coll T, Sánchez RM, Merlos M, Laguna JC, Vazquez M

In this study we examined the effect of the statin atorvastatin on the Akt/GSK-3beta pathway. Our findings indicate that atorvastatin treatment for 15 days inhibited pressure overload-induced cardiac hypertrophy and prevented nuclear translocation of GATA4 and c-Jun and AP-1 DNA-binding activity. In addition, atorvastatin treatment prevented the increase in the phosphorylation of Akt and GSK-3beta caused by cardiac hypertrophy, and this effect correlated with an increase in protein levels of…



Targeted knockdown of Cerkl, a retinal dystrophy gene, causes mild affectation of the retinal ganglion cell layer.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Garanto A, Vicente-Tejedor J, Riera M, de la Villa P, Gonzalez R, Blanco R, Marfany G

In order to approach the function of the retinal dystrophy CERKL gene we generated a novel knockout mouse model by cre-mediated targeted deletion of the Cerkl first exon and proximal promoter. The excised genomic region (2.3kb) encompassed the first Cerkl exon, upstream sequences including the proximal promoter and the initial segment of the first intron. The Cerkl-/- mice were viable and fertile. The targeted Cerkl deletion resulted in a knockdown more than a knockout model, given that…



Starvation enhances lipoprotein lipase activity in the liver of the newborn rat.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Grinberg-Vaisman DR, Ramírez I, Vilaró S, Reina M, Llobera M, Herrera E

To determine to what extent lipoprotein lipase activity in the liver of the newborn rat depends on milk ingestion, its changes were studied during different nutritional conditions. Newborns were placed with nurse rats with or without ligated nipples and they were killed at 0,8 or 24 h of life. Lipoprotein lipase in newborns liver was characterized by its inhibition in the presence of 1.0 M NaCl, its specific elution at 1.5 M NaCl on heparin-Sepharose 4B column and its requirement for serum…



Coordinate induction of Na(+)-dependent transport systems and Na+,K(+)-ATPase in the liver of obese Zucker rats.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Ruiz-Montasell B, Ferrer-Martinez A, Casado FJ, Felipe A, Pastor-Anglada M

Solute uptake into liver plasma membrane vesicles from either lean or obese Zucker rats was monitored. D-Glucose and L-leucine uptakes at physiological concentrations of the substrate were not different in lean and obese Zucker rats. In agreement with a previous report (Ruiz et al. (1991) Biochem. J. 280, 367-372) L-alanine uptake was significantly enhanced in those preparations from obese animals. Na(+)-coupled uridine transport was markedly enhanced also in obese rats. The effect was due…



Up-regulation of liver system A for neutral amino acid transport in euglycemic hyperinsulinemic rats.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Ferrer-Martínez A, Casado J, Leturque A, Felipe A, Pastor-Anglada M

To determine the role of insulin on the in vivo modulation of liver system A activity, we used the euglycemic hyperinsulinemic clamp coupled to the measurement of solute uptakes into plasma membrane vesicles partially purified from livers of hyperinsulinemic rats and their saline-infused controls. The clamp was performed in chronically catheterized rats, either in the fasted state, 24 h after surgery (Group I), or after 3 days of recovery (Group II). System A activity, measured as the…



Effect of epidermal growth factor (EGF) on gluconeogenesis in isolated rat hepatocytes. Dependency on the red-ox state of the substrate.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Soler C, Poveda B, Pastor-Anglada M, Soley M

It was found that EGF decreased both the basal- and the glucagon-stimulated gluconeogenesis from lactate alone or from a high lactate/pyruvate ratio and that it enhanced both the basal- and the glucagon-inhibited glucose synthesis from pyruvate alone or from a low lactate/pyruvate ratio. These findings demonstrate that the effect of both EGF and glucagon on glucose production by isolated hepatocytes depends on the red-ox state of the substrate.



Early alterations in energy metabolism in the hippocampus of APPswe/PS1dE9 mouse model of Alzheimer's disease.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Pedrós I, Petrov D, Allgaier M, Sureda F, Barroso E, Beas-Zarate C, Auladell C, Pallàs M, Vazquez M, Casadesús G, Folch J, Camins A

The present study had focused on the behavioral phenotype and gene expression profile of molecules related to insulin receptor signaling in the hippocampus of 3 and 6 month-old APPswe/PS1dE9 (APP/PS1) transgenic mouse model of Alzheimer's disease (AD). Elevated levels of the insoluble Aß (1-42) were detected in the brain extracts of the transgenic animals as early as 3 months of age, prior to the Aß plaque formation (pre-plaque stage). By the early plaque stage (6 months) both the soluble…



Tau hyperphosphorylation and increased BACE1 and RAGE levels in the cortex of PPARß/d-null mice.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Barroso E, del Valle J, Porquet D, Vieira Santos AM, Salvadó L, Rodríguez-Rodríguez R, Gutiérrez P, Anglada-Huguet M, Alberch J, Camins A, Palomer FX, Pallàs M, Michalik L, Wahli W, Vazquez M

The role of peroxisome proliferator activator receptor (PPAR)ß/d in the pathogenesis of Alzheimer's disease has only recently been explored through the use of PPARß/d agonists. Here we evaluated the effects of PPARß/d deficiency on the amyloidogenic pathway and tau hyperphosphorylation. PPARß/d-null mice showed cognitive impairment in the object recognition task, accompanied by enhanced DNA-binding activity of NF-?B in the cortex and increased expression of IL-6. In addition, two NF-?B-…



TNF-a inhibits PPARß/d activity and SIRT1 expression through NF-?B in human adipocytes.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Serrano-Marco L, Chacón MR, Maymó-Masip E, Barroso E, Salvadó L, Wabitsch M, Garrido-Sánchez L, Tinahones FJ, Palomer FX, Vendrell J, Vazquez M

The mechanisms linking low-grade chronic inflammation with obesity-induced insulin resistance have only been partially elucidated. PPARß/d and SIRT1 might play a role in this association. In visceral adipose tissue (VAT) from obese insulin-resistant patients we observed enhanced p65 nuclear translocation and elevated expression of the pro-inflammatory cytokines TNF-a and IL-6 compared to control subjects. Inflammation was accompanied by a reduction in the levels of SIRT1 protein and an increase…



PPARß/d activation blocks lipid-induced inflammatory pathways in mouse heart and human cardiac cells.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Alvarez-Guardia D, Palomer FX, Coll T, Serrano L, Rodríguez-Calvo R, Davidson MM, Merlos M, El Kochairi I, Michalik L, Wahli W, Vazquez M

Owing to its high fat content, the classical Western diet has a range of adverse effects on the heart, including enhanced inflammation, hypertrophy, and contractile dysfunction. Proinflammatory factors secreted by cardiac cells, which are under the transcriptional control of nuclear factor-?B (NF-?B), may contribute to heart failure and dilated cardiomyopathy. The underlying mechanisms are complex, since they are linked to systemic metabolic abnormalities and changes in cardiomyocyte phenotype…



Atorvastatin prevents peroxisome proliferator-activated receptor gamma coactivator-1 (PGC-1) downregulation in lipopolysaccharide-stimulated H9c2 cells.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Planavila A, Sánchez RM, Merlos M, Laguna JC, Vazquez M

Although abnormalities in cardiac fatty acid metabolism are involved in the development of several cardiac pathologies, the mechanisms underlying these changes are not well understood. Given the prominent role played by peroxisome proliferator-activated receptor beta/delta (PPARbeta/delta in cardiac fatty acid metabolism, the aim of this study was to examine the effects of nuclear factor (NF)-kappaB activation on the activity of this nuclear receptor. Embryonic rat heart-derived H9c2 cells…



Agonist-induced activation releases peroxisome proliferator-activated receptor beta/delta from its inhibition by palmitate-induced nuclear factor-kappaB in skeletal muscle cells.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Jové M, Laguna JC, Vazquez M

The mechanisms by which elevated levels of free fatty acids cause insulin resistance are not well understood, but there is a strong correlation between insulin resistance and intramyocellular lipid accumulation in skeletal muscle. In addition, accumulating evidence suggests a link between inflammation and type 2 diabetes. The aim of this work was to study whether the exposure of skeletal muscle cells to palmitate affected peroxisome proliferator-activated receptor (PPAR) beta/delta activity.…



Atorvastatin improves peroxisome proliferator-activated receptor signaling in cardiac hypertrophy by preventing nuclear factor-kappa B activation.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Planavila A, Laguna JC, Vazquez M

Nuclear factor (NF)-kappa B signaling pathway plays a pivotal role in cardiac hypertrophy. Although it has been reported that statins inhibit cardiac hypertrophy by reducing generation of reactive oxygen species, it is not yet known whether statins prevent NF-kappa B activation and whether this effect can be related to the reduction in the peroxisome proliferator-activated receptor (PPAR) pathway. In this study, we examined the role of atorvastatin on NF-kappa B activity and PPAR signaling in…



Increased production of the ether-lipid platelet-activating factor in intestinal epithelial cells infected by Salmonella enteritidis.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Egea L, Giménez R, Lúcia D, Modolell I, Badia J, Baldoma L, Aguilar J

When exposed to enteric pathogens intestinal epithelial cells produce several cytokines and other proinflammatory mediators. To date there is no evidence that the ether-lipid platelet-activating factor (PAF) is one of these mediators. Our results revealed a significant increase in PAF production by human colonic tissue 4 h after infection by enterohemorrhagic Escherichia coli (EHEC) or Salmonella enteritidis. PAF is produced in the gut by cells of the immune system in response to bacterial…



Inactivation of propanediol oxidoreductase of Escherichia coli by metal-catalyzed oxidation.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Cabiscol E, Badia J, Baldoma L, Hidalgo E, Aguilar J, Ros J

1,2-Propanediol oxidoreductase, which reduces the L-lactaldehyde formed in the fermentation of L-fucose or L-rhamnose to L-1,2-propanediol in E. coli, was inactivated by a component of E. coli cell extracts in the presence of oxygen. Pure propanediol oxidoreductase preparations were shown to be inactivated in vitro by aerobic incubations in the presence of Fe3+ and ascorbate. The Fe3+ ascorbate-mediated inactivation reaction was inhibited by catalase, although not by superoxide dismutase…



Reduced novelty-P3 associated with increased behavioral distractibility in schizophrenia.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Cortiñas M, Corral MJ, Garrido G, Garolera M, Pajares M, Escera C

Behavioral and electrophysiological brain responses were used to examine the relationship between the vulnerability to distraction and the orienting response in schizophrenia. Nineteen schizophrenics and nineteen matched healthy controls were instructed to ignore task-irrelevant auditory stimuli while they classified capital letters and digits. The auditory sequences contained repetitive standard tones occasionally replaced by complex novel sounds. Relative to controls, patients showed an…



Impaired preparatory re-mapping of stimulus-response associations and rule-implementation in schizophrenic patients--the role for differences in early processing.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Finke M, Barceló F, Garolera M, Cortiñas M, Garrido G, Pajares M, Escera C

An accurate representation of task-set information is needed for successful goal directed behavior. Recent studies point to disturbances in the early processing stages as plausible causes for task-switching deficits in schizophrenia. A task-cueing protocol was administered to a group of schizophrenic patients and compared with a sample of age-matched healthy controls. Patients responded slower and less accurate compared with controls in all conditions. The concurrent recording of event-related…



Electrophysiological and behavioral evidence of gender differences in the modulation of distraction by the emotional context.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Garcia-Garcia M, Domínguez-Borràs J, San-Miguel I, Escera C

Gender differences in brain activity while processing emotional stimuli have been demonstrated by neuroimaging and electrophysiological studies. However, the possible differential effects of emotion on attentional mechanisms between women and men are less understood. The present study aims to elucidate any gender differences in the modulation of unexpected auditory stimulus processing using an emotional context elicited by aversive images. Fourteen men and fourteen women performed a well-…



On the functional significance of Novelty-P3: facilitation by unexpected novel sounds.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
San-Miguel I, Morgan HM, Klein C, Linden D, Escera C

The unexpected occurrence of a change in the auditory context has been shown to result in distraction due to a momentary attention shift. These unexpected sounds elicit the Novelty-P3 (NP3) response which has been proposed as an electrophysiological index of distractibility, and used as such in the evaluation of several clinical populations. However, unexpected sounds also result in facilitation under certain conditions. Here, we investigate the electrophysiological concomitants of novel sounds…



Cardiac and placental mitochondrial characterization in a rabbit model of intrauterine growth restriction.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Guitart-Mampel M, Gonzalez-Tendero A, Niñerola S, Morén C, Catalán-Garcia M, González-Casacuberta I, Juárez-Flores DL, Ugarteburu O, Matalonga L, Cascajo MV, Tort F, Cortés A, Tobias E, Milisenda JC, Grau JM, Crispi F, Gratacós E, Garrabou G, Cardellach F

Intrauterine growth restriction (IUGR) is associated with cardiovascular remodeling persisting into adulthood. Mitochondrial bioenergetics, essential for embryonic development and cardiovascular function, are regulated by nuclear effectors as sirtuins. A rabbit model of IUGR and cardiovascular remodeling was generated, in which heart mitochondrial alterations were observed by microscopic and transcriptomic analysis. We aimed to evaluate if such alterations are translated at a functional…



Deficiency of perforin and hCNT1, a novel inborn error of pyrimidine metabolism, associated with a rapidly developing lethal phenotype due to multi-organ failure.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Perez-Torras S, Mata-Ventosa A, Drögemöller B, Tarailo-Graovac M, Meijer J, Meinsma R, van Cruchten AG, Kulik W, Viel-Oliva A, Bidon-Chanal A, Ross CJ, Wassermann WW, van Karnebeek CDM, Pastor-Anglada M, van Kuilenburg ABP

Pyrimidine nucleotides are essential for a vast number of cellular processes and dysregulation of pyrimidine metabolism has been associated with a variety of clinical abnormalities. Inborn errors of pyrimidine metabolism affecting enzymes in the pyrimidine de novo and degradation pathway have been identified but no patients have been described with a deficiency in proteins affecting the cellular import of ribonucleosides. In this manuscript, we report the elucidation of the genetic basis…



Increased subcortical neural responses to repeating auditory stimulation in children with autism spectrum disorder

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Font-Alaminos M, Cornella M, Costa-Faidella J, Hervás A, Leung S, Rueda I, Escera C

Recent research has highlighted atypical reactivity to sensory stimulation as a core symptom in children with autism spectrum disorder (ASD). However, little is known about the dysfunctional neurological mechanisms underlying these aberrant sensitivities. Here we tested the hypothesis that the ability to filter out auditory repeated information is deficient in children with ASD already from subcortical levels, yielding to auditory sensitivities. We recorded the frequency-following response (FFR…



Spatial processing in a mental rotation task: Differences between high and low math-anxiety individuals.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Nunez M, González-Gómez B, Colomé À

Previous studies suggested that highly math-anxious (HMA) individuals invest more attentional resources than their low math-anxious (LMA) peers in numerical tasks, and have worse spatial skills. We aimed to explore whether they also need to apply more resources in spatial tasks. In this study, HMA and LMA individuals saw normal or mirror-reversed letters in six orientations and made mirror-normal decisions. In both groups, response times and errors increased with angular deviation from…



New and potential strategies for the treatment of PMM2-CDG.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Gámez A, Serrano M, Gallego D, Vilas A, Pérez-Dueñas B

BACKGROUND: Mutations in the PMM2 gene cause phosphomannomutase 2 deficiency (PMM2; MIM# 212065), which manifests as a congenital disorder of glycosylation (PMM2-CDG). Mutant PMM2 leads to the reduced conversion of Man-6-P to Man-1-P, which results in low concentrations of guanosine 5'-diphospho-D-mannose, a nucleotide-activated sugar essential for the construction of protein oligosaccharide chains. To date the only therapeutic options are preventive and symptomatic. SCOPE OF REVIEW:…



The Spatial-Numerical Association of Response Codes (SNARC) effect in highly math-anxious individuals: An ERP study.

Repositorio: r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Nunez M, Colomé À, González-Gómez B

The Spatial-Numerical Association of Response Codes (SNARC) effect was examined in highly (HMA) and low math-anxious (LMA) individuals performing a number comparison in an ERP study. The SNARC effect consists of faster latencies when the response side is congruent with number location in the mental number line (MNL). Despite the stronger SNARC effect in the HMA group, their responses in incongruent trials were slower than in congruent trials only for the largest numerical magnitudes.…



Chronic hyperammonemia alters in opposite ways membrane expression of GluA1 and GluA2 AMPA receptor subunits in cerebellum. Molecular mechanisms involved

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
A. CABRERA-PASTOR, L. TAORO-GONZALEZ, E. LOPEZ-MERINO, F. LLANSOLA, V. FELIPO

Hyperammonemia contributes to altered neurotransmission and cognition in patients with hepatic encephalopathy. Hyperammonemia in rats affects differently high- and low-affinity AMPA receptors (AMPARs) in cerebellum. We hypothesized that hyperammonemia would alter differently membrane expression of AMPARs GluA1 and GluA2 subunits by altering its phosphorylation. This work aims were: 1) assess if hyperammonemia alters GluA1 and GluA2 subunits membrane expression in cerebellum and 2) analyze the…



A Drosophila model of GDAP1 function reveals the involvement of insulin signalling in the mitochondria-dependent neuromuscular degeneration

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
V. DEL AMO, M. PALOMINO-SCHATZLEIN, M. SECO-CERVERA, J. GARCIA-GIMENEZ, F. PALLARDO, A. PINEDA-LUCENA, M. GALINDO

Charcot-Marie-Tooth disease is a rare peripheral neuropathy for which there is no specific treatment. Some forms of Charcot-Marie-Tooth are due to mutations in the GDAP1 gene. A striking feature of mutations in GDAP1 is that they have a variable clinical manifestation, according to disease onset and progression, histology and mode of inheritance. Studies in cellular and animal models have revealed a role of GDAP1 in mitochondrial morphology and distribution, calcium homeostasis and oxidative…



Ink4/Arf locus restores glucose tolerance and insulin sensitivity by reducing hepatic steatosis and inflammation in mice with impaired IRS2-dependent signalling

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
A. VINUE, I. ANDRES-BLASCO, A. HERRERO-CERVERA, L. PIQUERAS, V. ANDRES, D. BURKS, M. SANZ, H. GONZALEZ-NAVARRO

Single nucleotide polymorphisms near the Ink4/Arf locus have been associated with type-2 diabetes mellitus. Previous studies indicate a protective role of the locus in the carbohydrate metabolism derangement associated with ageing in wild-type mice. The present study demonstrates that the increased Ink4/Arf locus expression in 1-year-old mice, partially-deficient for the insulin receptor substrate (IRS)2 (Irs2 +/-SuperInk4/Arf mice) ameliorates hepatic steatosis, inflammation and insulin…



mRNA export and gene expression: The SAGA-TREX-2 connection

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
E. GARCIA-OLIVER, V. GARCIA-MOLINERO, S. RODRIGUEZ-NAVARRO

In the gene expression field, different steps have been traditionally viewed as discrete and unconnected events. Nowadays, genetic and functional studies support the model of a coupled network of physical and functional connections to carry out mRNA biogenesis. Gene expression is a coordinated process that comprises different linked steps like transcription, RNA processing, export to the cytoplasm, translation and degradation of mRNAs. Its regulation is essential for cellular survival and can…



Mining of miRNAs and potential targets from gene oriented clusters of transcripts sequences of the anti-malarial plant, Artemisia annua

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
A. PEREZ-QUINTERO, G. SABLOK, T. TATARINOVA, A. CONESA, J. KUO, C. LOPEZ

miRNAs involved in the biosynthesis of artemisinin, an anti-malarial compound form the plant Artemisia annua, have been identified using computational approaches to find conserved pre-miRNAs in available A. annua UniGene collections. Eleven pre-miRNAs were found from nine families. Targets predicted for these miRNAs were mainly transcription factors for conserved miRNAs. No target genes involved in artemisinin biosynthesis were found. However, miR390 was predicted to target a gene involved in…



An exon three-way junction structure modulates splicing and degradation of the SUS1 yeast pre-mRNA

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
A. ABUQATTAM, J. SERRANO-QUILEZ, S. RODRIGUEZ-NAVARRO, J. GALLEGO

The SUS1 gene of Saccharomyces cerevisiae is unusual as it contains two introns and undergoes alternative splicing, retaining one or both introns depending on growth conditions. The exon located between the two introns can be skipped during splicing and has been detected in circular form. This exon (E2) has also been found to influence the splicing of the flanking introns, an unusual situation in budding yeast where splicing mainly relies on intron recognition. Using SHAPE (selective 2'-…



Extracellular histones activate autophagy and apoptosis via mTOR signaling in human endothelial cells

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
J. IBANEZ-CABELLOS, C. AGUADO, D. PEREZ-CREMADES, J. GARCIA-GIMENEZ, C. BUENO-BETI, E. GARCIA-LOPEZ, C. ROMA-MATEO, S. NOVELLA, C. HERMENEGILDO, F. PALLARDO

Circulating histones have been proposed as targets for therapy in sepsis and hyperinflammatory symptoms. However, the proposed strategies have failed in clinical trials. Although different mechanisms for histone-related cytotoxicity are being explored, those mediated by circulating histones are not fully understood. Extracellular histones induce endothelial cell death, thereby contributing to the pathogenesis of complex diseases such as sepsis and septic shock. Therefore, the comprehension of…



The MELAS mutation m.3243A > G promotes reactivation of fetal cardiac genes and an epithelial-mesenchymal transition-like program via dysregulation of miRNAs

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
S. MESEGUER, J. PANADERO, C. NAVARRO-GONZALEZ, M. VILLARROYA, R. BOUTOUAL, G. COMI, M. ARMENGOD

The pathomechanisms underlying oxidative phosphorylation (OXPHOS) diseases are not well-understood, but they involve maladaptive changes in mitochondria-nucleus communication. Many studies on the mitochondria nucleus cross-talk triggered by mitochondrial dysfunction have focused on the role played by regulatory proteins, while the participation of miRNAs remains poorly explored. MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes) is mostly caused by mutation m.…



Whirlin increases TRPV1 channel expression and cellular stability

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
M. CIARDO, A. ANDRES-BORDERIA, N. CUESTA, P. VALENTE, M. CAMPRUBI-ROBLES, J. YANG, R. PLANELLS-CASES, A. FERRER-MONTIEL

The expression and function of TRPV1 are influenced by its interaction with cellular proteins. Here, we identify Whirlin, a cytoskeletal PDZ-scaffold protein implicated in hearing, vision and mechanosensory transduction, as an interacting partner of TRPV1. Whirlin associates with TRPV1 in cell lines and in primary cultures of rat nociceptors. Whirlin is expressed in 55% of mouse sensory C-fibers, including peptidergic and non-peptidergic nociceptors, and co-localizes with TRPV1 in 70% of them.…



Structural and functional changes induced by tyrosine nitration in cytochrome c, a bi-functional protein

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
M. DE LA ROSA, J. GARCIA-HEREDIA, J. MARTINEZ-FABREGAS, P. NIETO, S. RUBIO-NOVELLA, M. ORZAEZ, S. KOCANIS, M. TEIXEIRA, E. PEREZ-PAYA, M. HERVAS, J. NAVARRO, A. DIAZ-QUINTANA, I. DIAZ-MORENO




Nitration of tyrosine 74 prevents human cytochrome c to play a key role in apoptosis signaling by blocking caspase-9 activation

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
J. GARCIA-HEREDIA, I. DIAZ-MORENO, P. NIETO, M. ORZAEZ, S. KOCANIS, M. TEIXEIRA, E. PEREZ-PAYA, A. DIAZ-QUINTANA, M. DE LA ROSA

Tyrosine nitration is one of the most common post-transcriptional modifications of proteins, so affecting their structure and function. Human cytochrome c, with five tyrosine residues, is an excellent case study as it is a well-known protein playing a double physiological role in different cell compartments. On one hand, it acts as electron carrier within the mitochondrial respiratory electron transport chain, and on the other hand, it serves as a cytoplasmic apoptosis-triggering agent. In a…



Multimerisation of receptor-type protein tyrosine phosphatases PTPBR7 and PTP-SL attenuates enzymatic activity

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
Y. NOORDMAN, E. AUGUSTUS, J. SCHEPENS, R. CHIRIVI, P. RIOS, R. PULIDO, W. HENDRIKS

Dimerisation of receptor-type protein tyrosine phosphatases (RPTPs) represents an appealing mechanism to regulate their enzymatic activity. Studies thus far mostly concern the dimerisation behaviour of RPTPs possessing two tandemly oriented catalytic PTP domains. Mouse gene Ptprr encodes four different protein isoforms (i.e. PTPBR7, PTP-SL and PTPPBS gamma-42/37) that contain a single PTP domain. Using selective membrane permeabilisation we here demonstrate that PTP-SL, like PTPBR7, is a single…



The MELAS mutation m.3243A > G alters the expression of mitochondrial tRNA fragments

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
Meseguer S, Navarro-González C, Panadero J, Villarroya M, Boutoual R, Sánchez-Alcázar JA, Armengod ME

Recent evidences highlight the importance of mitochondria-nucleus communication for the clinical phenotype of oxidative phosphorylation (OXPHOS) diseases. However, the participation of small non-coding RNAs (sncRNAs) in this communication has been poorly explored. We asked whether OXPHOS dysfunction alters the production of a new class of sncRNAs, mitochondria] tRNA fragments (mt tRFs), and, if so, whether mt tRFs play a physiological role and their accumulation is controlled by the action of…



Dynamics of the intrinsically disordered protein NUPR1 in isolation and in its fuzzy complexes with DNA and prothymosin alpha

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
J. NEIRA, M. PALOMINO-SCHATZLEIN, C. RICCI, M. ORTORE, B. RIZZUTI, J. IOVANNA

Intrinsically disordered proteins (IDPs) explore diverse conformations in their free states and, a few of them, also in their molecular complexes. This functional plasticity is essential for the function of IDPs, although their dynamics in both free and bound states is poorly understood. NUPR1 is a protumoral multifunctional IDP, activated during the acute phases of pancreatitis. It interacts with DNA and other IDPs, such as prothymosin alpha (ProT alpha), with dissociation constants of similar…



Regulation of the autophagic PI3KC3 complex by laforin/malin E3-ubiquitin ligase, two proteins involved in Lafora disease

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
P. SANCHEZ-MARTIN, M. LAHUERTA, R. VIANA, E. KNECHT, P. SANZ

Lafora progressive myoclonus epilepsy is a fatal rare neurodegenerative disorder characterized by the accumulation of insoluble abnormal glycogen deposits in the brain and peripheral tissues. Mutations in at least two genes are responsible for the disease: EPM2A, encoding the glucan phosphatase laforin, and EPM2B, encoding the RING-type E3-ubiquitin ligase malin. Both laforin and malin form a functional complex in which laforin recruits the substrates to be ubiquitinated by malin. We and others…



Acute depletion of telomerase components DKC1 and NOP10 induces oxidative stress and disrupts ribosomal biogenesis via NPM1 and activation of the P53 pathway.

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
Ibáñez-Cabellos JS, Seco-Cervera M, Picher-Latorre C, Pérez-Machado G, García-Giménez JL, Pallardó FV

Mutations in DKC1, NOP10, and TINF2 genes, coding for proteins in telomerase and shelterin complexes, are responsible for diverse diseases known as telomeropathies and ribosomopathies, including dyskeratosis congenita (DC, ORPHA 1775). These genes contribute to the DC phenotype through mechanisms that are not completely understood. We previously demonstrated in models of DC that oxidative stress is an early and independent event that occurs prior to telomere shortening. To clarify the…



An N-terminal half fragment of the histidine phosphocarrier protein, HPr, is disordered but binds to HPr partners and shows antibacterial properties

Repositorio: r-CIPF. Repositorio Institucional Producción Científica del Centro de Investigación Principe Felipe (CIPF)
J. NEIRA, M. PALOMINO-SCHATZLEIN, E. HURTADO-GOMEZ, M. ORTORE, A. FALCO

Background: The phosphotransferase system (PTS) modulates the preferential use of sugars in bacteria. It is formed by a protein cascade in which the first two proteins are general (namely enzyme I, EI, and the histidine phosphocarrier protein, HPr) and the others are sugar-specific permeases; the active site of HPr is His15. The HPr kinase/phosphorylase (HPrK/P), involved in the use of carbon sources in Gram-positive, phopshorylates HPr at a serine. The regulator of sigma D protein (Rsd) also…



Prevenir y combatir los diferentes tipos de violencia contra la infancia y adolescencia LGTBI+: guía para profesionales, How to Prevent and Combat Violence Against LGBTI+ and Gender Non Conforming Children and Youth: A Handbook for Professionals working in: Education, Health, Family Associations and Child Protection Agencies, Media and Public Spaces, Kaip užkirsti kelią įvairių formų smurtui prieš LGBTI+ vaikus?: Vadovas profesionalams, dirbantiems švietimo, sveikatos apsaugos, paramos šeimoms, žiniasklaidos ir viešojo sektoriaus srityse, Jak zapobiegać i przeciwstawiać się dyskryminacji oraz przemocy wobec dzieci i młodzieży LGBTI+?: Podręcznik dla specjalistów i specjalistek pracujących w dziedzinie edukacji, ochrony zdrowia, mediów, administracji, w stowarzyszeniach rodzinnych i organizacjach zajmujących się ochroną praw dziecka, Kako spriječiti i suzbiti različite vrste nasilja nad LGBTI+ i rodno nenormativnom djecom: Priručnik za stručnjake_inje koji_e rade u područjima obrazovanja i zdravstva, u udrugama koje se bave obiteljskom tematikom i službama za zaštitu djece, u medijima i javnim prostorima, Hoe Verschillende Soorten Geweld Tegen LGBTI+- en Gendervariante Kinderen Voorkomen en Bestreden Kunnen Worden: Een Handboek voor Professionelen tewerkgesteld in het Onderwijs, de Gezondheidssector, Gezinsverenigingen en Kinderbeschermingsorganisaties, de Media en Publieke Ruimtes, Kako preprečevati različne oblike nasilja nad LGBTI+ otroki in mladimi, ki svojo spolno identiteto in spolni izraz izražajo na načine, ki so v družbi prepoznani kot spolno nenormativni?: Priročnik za strokovnjake, ki delujejo na področju izobraževanja, zdravstva, družinskih združenj in organizacij za zaščito otrok, medijev in javnih prostorov, Πώς να αποτρέψετε και να καταπολεμήσετε διάφορες μορφές βίας κατά των ΛΟΑΤΙ+ και των μη συμμορφούμενων με το φύλο τους παιδιών: Εγχειρίδιο για Επαγγελματίες που εργάζονται στους Τομείς: της Εκπαίδευσης, της Υγείας, της Οικογενειακής Μέριμνας και της Παιδικής Προστασίας, των Μέσων Μαζικής Ενημέρωσης και σε Δημόσιους Χώρους, Hogyan léphetünk fel az LMBTQI és nemileg nonkonform gyermekekkel és fiatalokkal szembeni erőszak ellen?: Kézikönyv az alábbi területeken dolgozó szakembereknek: oktatás, egészségügy, családszervezetek, családsegítő szolgálatok és gyermekvédelmi intézmények, média, közösségi terek, Como Prevenir e Combater a Violência contra Crianças e Jovens LGBTI+ e de Género Diverso: Áreas : Educação, Saúde, Trabalho com Famílias, Média, Espaços Públicos e Intervenção Comunitária

Repositorio: DUGiDocs – Universitat de Girona
Diversity and Childhood (Projecte), Sándor, Bea

Coordinador del projecte: Universitat de Girona; Autoria: Bea Sándor; Adaptació a l'espanyol: Núria Sadurní, Beatriz San Román, Jose Antonio Langarita, Marisela Montenegro, Pilar Albertín i Joan Pujol; Disseny gràfic: Zsolt S. Szabó; Adaptació gràfica de la guia: Cristina de Dios, This handbook was created within the framework of the Diversity and Childhood: Changing social attitudes towards gender diversity in children across Europe (DaC) project. As its main expected results, the project seeks to raise awareness and reduce prejudice, as well as to improve protection against violence and support for LGBTI+ and gender non-conforming children and youth.1 DaC also aims to provide effective tools to stakeholders and envisages strengthening the well-being and the…, Este manual ha sido elaborado en el marco del proyecto de Diversity and Childhood: Cambiando las actitudes sociales hacia la diversidad de género en la infancia en toda Europa (DaC). Dicho proyecto trata de sensibilizar y reducir los prejuicios, así como de mejorar los mecanismos de protección contra la violencia hacia la infancia y la adolescencia LGTBI+. El proyecto DaC también tiene como objetivo proporcionar instrumentos eficaces a las profesionales, 1 fortalecer el bienestar y los…, Projecte "Diversity and childhood: changing social attitudes towards gender diversity in children across Europe", finançat pel Programa de Drets, Igualtat i Ciutadania (2014-2020) de la Unió Europea (REC-RDAP-GBV-AG-2018-856680)



Interactive App: Frequently Asked Questions about violence against LGBTI+ children

Repositorio: DUGiDocs – Universitat de Girona
Diversity and Childhood (Projecte)

Frequently Asked Questions about violence against LGBTI+ children for Interactive App in English, Hungarian, Dutch, Croatian, Polish, Greek, Lithuanian, Portuguese, Slovenian, and Spanish, Projecte "Diversity and childhood: changing social attitudes towards gender diversity in children across Europe", finançat pel Programa de Drets, Igualtat i Ciutadania (2014-2020) de la Unió Europea (REC-RDAP-GBV-AG-2018-856680)



Conformational and thermal characterization of left ventricle remodeling post-myocardial infarction

Repositorio: r-IGTP. Repositorio Institucional de Producción Científica del Instituto de Investigación Germans Trias i Pujol
Samouillan, V, Revuelta-Lopez, E, Soler-Botija, C, Dandurand, J, Benitez-Amaro, A, Nasarre, L, de Gonzalo-Calvo, D, Bayes-Genis, A, Lacabanne, C, Llorente-Cortes, V

Adverse cardiac remodeling after myocardial infarction (MI) causes impaired ventricular function and heart failure. Histopathological characterization is commonly used to detect the location, size and shape of MI sites. However, the information about chemical composition, physical structure and molecular mobility of peri- and infarct zones post-MI is rather limited. The main objective of this work was to explore the spatiotemporal biochemical and biophysical alterations of key cardiac…



Bihotz Sakratuaren hilabethea / A.B. Joannategui Benediktanoak eskuaraz ezarria.

Repositorio: Memoria Digital Vasca = Euskal Memoria Digitala

Dialecto : texto en euskera navarro-labortano, S. XX -- Periodo : último euskera moderno, Euskalkia : nafar-lapurtera, XX. md. -- Aroa : azken euskara modernoa, Digitalización Vitoria-Gasteiz Archivos y Bibliotecas Julio 1995 19-41



Spanje en de Spanjaarden / door Azamat Batuk (N.G. Thieblin) ; naar het engelsch bewerkt door Jo. de Uries.

Repositorio: Memoria Digital Vasca = Euskal Memoria Digitala
Thieblin, Nicolás León.

Traducción holandesa del inglés, Retrato de Don Carlos, Digitalización Vitoria-Gasteiz Fundación Sancho el Sabio 2009, Cartoné



Euskal Herria : imagined territory / Jan Mansvelt Beck.

Repositorio: Memoria Digital Vasca = Euskal Memoria Digitala
Mansvelt Beck, Jan.

Es fotocopia, Versión en papel de la edición electrónica de: SGIR Fifth Pan International Relations Conference "Constructing World Orders". The Hague, 9-11 september 2004



Genetic analysis of IP3 and calcium signalling pathways in C. elegans.

Repositorio: r-IIS La Fe. Repositorio Institucional de Producción Científica del Instituto de Investigación Sanitaria La Fe
Baylis HA, Vázquez-Manrique RP

The nematode, Caenorhabditis elegans is an established model system that is particularly well suited to genetic analysis. C. elegans is easily manipulated and we have an in depth knowledge of many aspects of its biology. Thus, it is an attractive system in which to pursue integrated studies of signalling pathways. C. elegans has a complement of calcium signalling molecules similar to that of other animals.



The human liver fatty acid binding protein (FABP1) gene is activated by FOXA1 and PPARa; and repressed by C/EBPa: Implications in FABP1 down-regulation in nonalcoholic fatty liver disease.

Repositorio: r-IIS La Fe. Repositorio Institucional de Producción Científica del Instituto de Investigación Sanitaria La Fe
Guzmán C, Benet M, Pisonero-Vaquero S, Moya M, García-Mediavilla MV, Martínez-Chantar ML, González-Gallego J, Castell JV, Sánchez-Campos S, Jover R

Liver fatty acid binding protein (FABP1) prevents lipotoxicity of free fatty acids and regulates fatty acid trafficking and partition. Our objective is to investigate the transcription factors controlling the human FABP1 gene and their regulation in nonalcoholic fatty liver disease (NAFLD). Adenovirus-mediated expression of multiple transcription factors in HepG2 cells and cultured human hepatocytes demonstrated that FOXA1 and PPARa are among the most effective activators of human FABP1,…



Modulation of protein synthesis and degradation maintains proteostasis during yeast growth at different temperatures

Repositorio: r-IIS La Fe. Repositorio Institucional de Producción Científica del Instituto de Investigación Sanitaria La Fe
Benet, M, Miguel, A, Carrasco, F, Li, TL, Planells, J, Alepuz, P, Tordera, V, Perez-Ortin, JE

To understand how cells regulate each step in the flow of gene expression is one of the most fundamental goals in molecular biology. In this work, we have investigated several protein turnover-related steps in the context of gene expression regulation in response to changes in external temperature in model yeast Saccharomyces cerevisiae. We have found that the regulation of protein homeostasis is stricter than mRNA homeostasis. Although global translation and protein degradation rates are found…