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Recessive gene mutations underlie many developmental disorders and often lead to disabling neurological problems. Here, we report identification of a homozygous c.170G>A (p.Cys57Tyr or C57Y) mutation in the gene coding for protein disulfide isomerase A3 (PDIA3, also known as ERp57), an enzyme that catalyzes formation of disulfide bonds in the endoplasmic reticulum, to be associated with syndromic intellectual disability. Experiments in zebrafish embryos show that PDIA3C57Y expression is pathogenic and causes developmental defects such as axonal disorganization as well as skeletal abnormalities. Expression of PDIA3C57Y in the mouse hippocampus results in impaired synaptic plasticity and memory consolidation. Proteomic and functional analyses reveal that PDIA3C57Y expression leads to dysregulation of cell adhesion and actin cytoskeleton dynamics, associated with altered integrin biogenesis and reduced neuritogenesis. Biochemical studies show that PDIA3C57Y has decreased catalytic activity and forms disulfide-crosslinked aggregates that abnormally interact with chaperones in the endoplasmic reticulum. Thus, rare disease gene variant can provide insight into how perturbations of neuronal proteostasis can affect the function of the nervous system.

Information from former ice sheets may provide important context for understanding the response of today’s ice sheets to forcing mechanisms. Here we present a reconstruction of the last deglaciation of marine sectors of the Eurasian Ice Sheet, emphasising how the retreat of the Norwegian Channel and the Barents Sea ice streams led to separation of the British-Irish and Fennoscandian ice sheets at c. 18.700 and of the Kara-Barents Sea-Svalbard and Fennoscandian ice sheets between 16.000 and 15.000 years ago. Combined with ice sheet modelling and palaeoceanographic data, our reconstruction shows that the deglaciation, from a peak volume of 20 m of sea-level rise equivalent, was mainly driven by temperature forced surface mass balance in the south, and by Nordic Seas oceanic conditions in the north. Our results highlight the nonlinearity in the response of an ice sheet to forcing and the significance of ocean-ice-atmosphere dynamics in assessing the fate of contemporary ice sheets.

An Integral Field Unit (IFU) based on image-slicers has been added to the GREGOR Infrared Spectrograph (GRIS). This upgrade to the instrument makes possible 2D spectropolarimetry in the near-infrared by simultaneously recording the full Stokes profiles of spectral lines (in a given spectral interval) at all the points in the field of view. It provides high-cadence spectropolarimetric observations at the instrument's high spatial resolution and high polarization sensitivity at the GREGOR solar telescope. The IFU is ideal for observing the polarized spectrum of fast-evolving solar features at high spatial and spectral resolutions. The high observing cadence opens the possibility of time-series observations. The analysis of observations to this level of accuracy is essential for understanding the complex dynamics and interactions of solar plasma and magnetic fields. The image slicer of the IFU has eight slices of width 100 micron, covering a total field of view of 6" x 3". It was designed and built within the framework of the European projects SOLARNET and GREST, as a prototype for future instruments of the European Solar Telescope (EST) and was integrated into GRIS. After two commissioning campaigns in 2017 and 2018, the IFU was finally installed at the end of September 2018 and offered to all observers who use the telescope.

Florid cutaneous papillomatosis is a rare paraneoplastic dermatosis, most commonly associated with gastric adenocarcinoma. It is characterized by a sudden onset of hyperkeratotic papules, clinically indistinguishable from viral warts. We report an 80-year-old man who presented to our department with a two-month history of multiple verrucous lesions affecting the face, perioral region, and hands. Two years before, he was treated for a gastric adenocarcinoma with a subtotal gastrectomy, but showed no evidence of residual disease or recurrence. Given the clinical background, a diagnosis of florid cutaneous papillomatosis was considered. Skin biopsy excluded a viral origin, and tumour recurrence was later identified through an abdominal ultrasound. The onset of this entity is typically prior or concurrent with the diagnosis of the internal malignancy, but it may also represent the first sign of recurrence of a previously treated neoplasm. Its early recognition is essential to ensure a thorough investigation and prompt treatment.

Florid cutaneous papillomatosis is a rare paraneoplastic dermatosis, most commonly associated with gastric adenocarcinoma. It is characterized by a sudden onset of hyperkeratotic papules, clinically indistinguishable from viral warts. We report an 80-year-old man who presented to our department with a two-month history of multiple verrucous lesions affecting the face, perioral region, and hands. Two years before, he was treated for a gastric adenocarcinoma with a subtotal gastrectomy, but showed no evidence of residual disease or recurrence. Given the clinical background, a diagnosis of florid cutaneous papillomatosis was considered. Skin biopsy excluded a viral origin, and tumour recurrence was later identified through an abdominal ultrasound. The onset of this entity is typically prior or concurrent with the diagnosis of the internal malignancy, but it may also represent the first sign of recurrence of a previously treated neoplasm. Its early recognition is essential to ensure a thorough investigation and prompt treatment.

One of the most remarkable natural events on Earth are the large lateral flank collapses of oceanic volcanoes, involving volumes of rock exceeding tens ofkm3• These collapses are relatively frequent in recent geological times as supported by evidence found in the geomorphology of volcanic island edifices and associated debris flows deposited on the proximal ocean floor. The Island of Fogo in the Cape Verde archipelago is one of the most active and prominent oceanic volcanoes on Earth. The island has an average diameter of 25 km and reaches a maximum elevation of 2829 m above sea level (m a.s.l.) at Pico do Fogo, a young stratovolcano located within a summit depression open eastward due to a large lateral flank collapse. The sudden collapse of the eastern flank of Fogo Island produced a megatsunami -73 ky ago. The limits of the flank collapse were deduced as well from geomorphologic markers within the island. The headwall of the collapse scar is interpreted as either being located beneath the post-collapse volcanic infill of the summit depression or located further west, corresponding to the Bordeira wall that partially surrounds it. The magnetotelluric (MT) method provides a depth distribution of the ground resistivity obtained by the simultaneous measurement of the natural variations of the electric and magnetic field of the Earth. Two N-S magnetotelluric profiles were acquired across the collapsed area to determine its geometry and boundaries. The acquired MT data allowed the determination of the limits of the collapsed area more accurately as well as its morphology at depth and thickness of the post-collapse infill. According to the newly obtained MT data and the bathymetry of the eastern submarine flank of Fogo, the volume involved in the flank collapse is estimated in -110 km3 • This volume -the first calculated onshore- stands between the previously published more conservative and excessive calculations -offshore- that were exclusively based in geomorphic evid

One of the most remarkable natural events on Earth are the large lateral flank collapses of oceanic volcanoes, involving volumes of rock exceeding tens ofkm3• These collapses are relatively frequent in recent geological times as supported by evidence found in the geomorphology of volcanic island edifices and associated debris flows deposited on the proximal ocean floor. The Island of Fogo in the Cape Verde archipelago is one of the most active and prominent oceanic volcanoes on Earth. The island has an average diameter of 25 km and reaches a maximum elevation of 2829 m above sea level (m a.s.l.) at Pico do Fogo, a young stratovolcano located within a summit depression open eastward due to a large lateral flank collapse. The sudden collapse of the eastern flank of Fogo Island produced a megatsunami -73 ky ago. The limits of the flank collapse were deduced as well from geomorphologic markers within the island. The headwall of the collapse scar is interpreted as either being located beneath the post-collapse volcanic infill of the summit depression or located further west, corresponding to the Bordeira wall that partially surrounds it. The magnetotelluric (MT) method provides a depth distribution of the ground resistivity obtained by the simultaneous measurement of the natural variations of the electric and magnetic field of the Earth. Two N-S magnetotelluric profiles were acquired across the collapsed area to determine its geometry and boundaries. The acquired MT data allowed the determination of the limits of the collapsed area more accurately as well as its morphology at depth and thickness of the post-collapse infill. According to the newly obtained MT data and the bathymetry of the eastern submarine flank of Fogo, the volume involved in the flank collapse is estimated in -110 km3 • This volume -the first calculated onshore- stands between the previously published more conservative and excessive calculations -offshore- that were exclusively based in geomorphic evid

Polyester (PES) fabrics were treated with plasma to enhance the adhesion of reduced graphene oxide (RGO) and produce conductive fabrics. The surface energy of the plasma treated fabrics was measured using contact angle measurements and showed a stabilization of this parameter with plasma dosages of 3000 W min m(-2). The surface roughness measured by atomic force microscopy (AFM) also showed a stabilization with the same plasma dosage value. The plasma treatment induced negative charges on the surface of the fibers and graphene oxide (GO) also presented negative charges - and so deposition of GO on the surface of the PES fibers was not possible. For this reason, bovine serum albumin (BSA) was employed as an intermediate coating that acquired a positive charge and enabled the self-assembly of GO on plasma treated PES fibers. Electrochemical impedance spectroscopy (EIS) was employed to measure the resistance of the conductive fabrics. The plasma treatment and BSA coating improved the coating level of the samples and hence the conductivity of the fabrics was improved with the application of fewer RGO layers. RGO adhesion on fabrics was also improved as shown in rubbing fastness tests. (C) 2015 Elsevier B.V. All rights reserved.

Polyester (PES) fabrics were treated with plasma to enhance the adhesion of reduced graphene oxide (RGO) and produce conductive fabrics. The surface energy of the plasma treated fabrics was measured using contact angle measurements and showed a stabilization of this parameter with plasma dosages of 3000 W min m(-2). The surface roughness measured by atomic force microscopy (AFM) also showed a stabilization with the same plasma dosage value. The plasma treatment induced negative charges on the surface of the fibers and graphene oxide (GO) also presented negative charges - and so deposition of GO on the surface of the PES fibers was not possible. For this reason, bovine serum albumin (BSA) was employed as an intermediate coating that acquired a positive charge and enabled the self-assembly of GO on plasma treated PES fibers. Electrochemical impedance spectroscopy (EIS) was employed to measure the resistance of the conductive fabrics. The plasma treatment and BSA coating improved the coating level of the samples and hence the conductivity of the fabrics was improved with the application of fewer RGO layers. RGO adhesion on fabrics was also improved as shown in rubbing fastness tests. (C) 2015 Elsevier B.V. All rights reserved.

[EN] Polyester fabrics have been treated with plasma to increase polypyrrole/PW12O403-(hybrid material) adhesion to its surface. With the plasma treatment, the roughness of the fibers increases as it has been observed by means of atomic force microscopy (AFM). Polar functional groups are also created on the surface of polyester fabrics as Xray photoelectron spectroscopy (XPS) measurements have shown. These polar groups contribute to the adhesion of polypyrrole to the fibers. Coatings obtained on plasma treated fabrics were more resistant to washing and rubbing fastness tests. The use of an inorganic counter ion (PW12O40 3-) that contains an element with a high atomic number (W) helps to locate zones where the coating is missed; this is achieved by means of micrographs obtained by backscattered electrons (BSE). The electrical resistance of the fabrics was also measured by electrochemical impedance spectroscopy (EIS), obtaining also better results with the plasma treated fabrics.

[EN] Polyester fabrics have been treated with plasma to increase polypyrrole/PW12O403-(hybrid material) adhesion to its surface. With the plasma treatment, the roughness of the fibers increases as it has been observed by means of atomic force microscopy (AFM). Polar functional groups are also created on the surface of polyester fabrics as Xray photoelectron spectroscopy (XPS) measurements have shown. These polar groups contribute to the adhesion of polypyrrole to the fibers. Coatings obtained on plasma treated fabrics were more resistant to washing and rubbing fastness tests. The use of an inorganic counter ion (PW12O40 3-) that contains an element with a high atomic number (W) helps to locate zones where the coating is missed; this is achieved by means of micrographs obtained by backscattered electrons (BSE). The electrical resistance of the fabrics was also measured by electrochemical impedance spectroscopy (EIS), obtaining also better results with the plasma treated fabrics.

Objective. To detect the presence of antibodies to leptospira sp in pigs that didn´t show clinical symptoms, of swine production farms located throughout Argentina. Materials and methods. The location of farms and samples were chosen in proportion to those recorded in each province covering the whole country. The number of farms studied was 1,524, with 19,872 serum samples. This study takes into consideration areas identified by provinces, municipalities and properties. In the first phase 3,631 sera were processed and analyzed by MAT (microscopic agglutination test). Results. The 30% of the samples tested were positive for the screening test (MAT). Most of these samples presented coagglutinins for two or three serovars. In the titration of sera, the percentage of positives was the same, the most prevalent serovars were Icterohaemorrhagiae and Castellonis. The 90% of the samples had a final titer of 1:200. Conclusions. The existence of positive sera in all provinces indicates the contact of animals with leptospira throughout the country., Objetivo. Detectar la presencia de anticuerpos contra Leptospira sp en cerdos que no presentaban sintomatología clínica, pertenecientes a establecimientos de producción porcina ubicados en todo el territorio de la República Argentina. Materiales y métodos. La asignación de predios y muestras se realizó en forma proporcional a los registrados en cada provincia a lo largo de todo el país. En total se estudiaron 1.524 predios, de los cuales se tomaron 19.872 muestras de suero y en la primera etapa se procesaron 3.631 de los mismos. Este estudio toma en consideración zonas identificadas por provincia, municipio y predios. Los sueros fueron analizados por la técnica de MAT (prueba de microaglutinación). Resultados. El 30% de las muestras resultaron positivas a la prueba tamiz empleada (primera etapa del MAT). La mayoría presentó coaglutininas a dos o a tres serovariedades. Al realizar la titulación de los sueros (segunda etapa del MAT), el porcentaje de positividad se mantuvo, siendo los serovares de mayor prevalencia Castellonis e Icterohaemorrhagiae. El 90% de las muestras presentó un título final de 1:200. Conclusiones. La existencia de sueros positivos en todas las provincias indica el contacto de los animales con la leptospira a lo largo de todo el país.

Depression is the loss or lowering of pressure soul, resulting in diminishing the vital and psychic energy, libido, motivation and interest in the real. It is a disease that makes a person incapable, may have comorbidity with other diseases, has a risk of suicide, with numerous social and economic con- sequences. The memory deficits are reported as the major cognitive difficulties in symptomatic patients with depressive illness due to the role of the hippocampus in the formation and consolidation of new memories and their importance for the regulation of motivation and emotion. There is a distinction between depressive personality and depression itself, seen from the study of depressive states. Admittedly depressivity and depression, depressive personality and depressive access are combined situations, phenomena that ride: the feeling of failure is at a subjective level as a loss, a ‘loss of which has yet to gain’, and the other on the desire of loss - that the anger of depressivity imposes. Thus, health professionals have a role in recognition of the disturbance. Given the presence of symp- toms to neuropsychological assessment becomes a crucial tool in the diagnosis of these individuals., Depressão é a perda ou abaixamento da pressão anímica, traduzindo-se por diminuição da energia psíquica e vital, da libido, da motivação e do interesse pelo real. É uma doença que torna uma pessoa incapaz, pode apresentar comorbilidade com outras doenças, apresenta risco de suicídio, com numerosas consequências sociais e económicas. Os défices da memória são relatados como as maiores dificuldades cognitivas em pacientes sintomáticos e com doença depressiva devido ao papel fundamental do hipocampo na formação e consolidação de memórias novas assim como a sua importância para o regulamento da motivação e da emoção. Existe uma distinção entre a personalidade depressiva e a depressão propriamente dita, verificada a partir do estudo dos estados depressivos. É certo que depressividade e depressão, personalidade depressiva e acesso depressivo, são situações conjugadas, fenómenos que se cavalgam: o sentimento de falta é, no plano subjectivo já uma perda, uma «perda do que ainda não foi ganho»; e, por outro lado o desejo da perda – que a raiva da depressividade impõe. Assim, o profissional de saúde tem um papel preponderante no reconhecimento da perturbação. Perante a presença de sintomas a avaliação neuropsicológica torna-se a ferramenta decisiva no diagnóstico destes indivíduos.

This paper presents the role of Emotional Intelligence and the positive impact it can have on human life. In the early 1990s, Salovey and Mayer (1990) published an article that would result from several studies on the intelligence and the role of emotions in human development. Have suggested that there was a cognitive ability that until now had not been classified. Those powers it is known as Emotional Intelligence, from that moment raises a deep interest in many researchers around the world. Goleman (1996) draws attention to the world for a theme already in detail by other authors, the distinct conception of traditional intelligence. Making known the development that the role of emotional intelligence and their emotions could have on professional success, psychological and social development of each individual. The Emotional Intelligence enables man to become aware of their emotions, understand the feelings, as much as their others. It’s a skill that we possess in order to relate to others and communicate in an assertive manner, which will allow project an optimistic attitude towards life. Linked to a strong ally emotional intelligence and emotions, can provide levels of psychological well-being and physical not covered before. Joining and applying a positive psychology in your life, man can cover fields of stability never before achieved. Emotional intelligence is an art that requires a simple learning and conscious invaluable., O presente trabalho visa apresentar o papel da Inteligência Emocional e as repercussões positivas que pode ter na vida do ser Humano. No início da década de 1990, Salovey e Mayer (1990) publicaram um artigo que resultaria de vários estudos sobre a inteligência e o papel das emoções no desenvolvimento do Homem. Sugeriram assim, que existia uma competência cognitiva que até ao momento não tinha sido classificada. Tal competência recebe a designação de Inteligência Emocional, desde esse momento suscita um profundo interesse em vários pesquisadores por todo o mundo. Goleman (1996) chama a atenção ao mundo para um tema já aprofundado por vários autores, vincando a concepção distinta da Inteligência tradicional. Dando a conhecer o sucesso que o papel da Inteligência Emocional e Emoções poderiam ter no desenvolvimento profissional, psicológico e social de cada individuo. A Inteligência Emocional permite ao ser humano tomar consciência das suas emoções, compreender os sentimentos, tanto como as dos seus demais. É a uma aptidão que possuímos, de forma a relacionar com os outros e comunicar de forma assertiva, que vai permitir projectar uma atitude optimista perante a vida. A inteligência Emocional é uma arte que pressupõe uma aprendizagem simples e consciente de valor incalculável.

Strategies for non-invasive and quantitative imaging of gene expression in vivo have been developed over the past decade. Non-invasive assessment of the dynamics of gene regulation is of interest for the detection of endogenous disease-specific biological alterations (e.g., signal transduction) and for monitoring the induction and regulation of therapeutic genes (e.g., gene therapy). To demonstrate that non-invasive imaging of regulated expression of any type of gene after in vivo transduction by versatile vectors is feasible, we generated regulatable herpes simplex virus type 1 (HSV-1) amplicon vectors carrying hormone (mifepristone) or antibiotic (tetracycline) regulated promoters driving the proportional co-expression of two marker genes. Regulated gene expression was monitored by fluorescence microscopy in culture and by positron emission tomography (PET) or bioluminescence (BLI) in vivo. The induction levels evaluated in glioma models varied depending on the dose of inductor. With fluorescence microscopy and BLI being the tools for assessing gene expression in culture and animal models, and with PET being the technology for possible application in humans, the generated vectors may serve to non-invasively monitor the dynamics of any gene of interest which is proportionally co-expressed with the respective imaging marker gene in research applications aiming towards translation into clinical application.