Beyond Genetic Factors in Familial Amyloidotic Polyneuropathy: Protein Glycation and the Loss of Fibrinogen's Chaperone Activity

Familial amyloidotic polyneuropathy (FAP) is a systemic conformational disease characterized by extracellular amyloid fibril formation from plasma transthyretin (TTR). This is a crippling, fatal disease for which liver transplantation is the only effective therapy. More than 80 TTR point mutations are associated with amyloidotic diseases and the most widely accepted disease model relates TTR tetramer instability with TTR point mutations. However, this model fails to explain two observations. First, native TTR also forms amyloid in systemic senile amyloidosis, a geriatric disease. Second, age at disease onset varies by decades for patients bearing the same mutation and some mutation carrier individuals are asymptomatic throughout their lives. Hence, mutations only accelerate the process and non-genetic factors must play a key role in the molecular mechanisms of disease. One of these factors is protein glycation, previously associated with conformational diseases like Alzheimer's and Parkinson's. The glycation hypothesis in FAP is supported by our previous discovery of methylglyoxal-derived glycation of amyloid fibrils in FAP patients. Here we show that plasma proteins are differentially glycated by methylglyoxal in FAP patients and that fibrinogen is the main glycation target. Moreover, we also found that fibrinogen interacts with TTR in plasma. Fibrinogen has chaperone activity which is compromised upon glycation by methylglyoxal. Hence, we propose that methylglyoxal glycation hampers the chaperone activity of fibrinogen, rendering TTR more prone to aggregation, amyloid formation and ultimately, disease

La renovación de la palabra en el bicentenario de la Argentina : los colores de la mirada lingüística

El libro reúne trabajos en los que se exponen resultados de investigaciones presentadas por investigadores de Argentina, Chile, Brasil, España, Italia y Alemania en el XII Congreso de la Sociedad Argentina de Lingüística (SAL), Bicentenario: la renovación de la palabra, realizado en Mendoza, Argentina, entre el 6 y el 9 de abril de 2010. Las temáticas abordadas en los 167 capítulos muestran las grandes líneas de investigación que se desarrollan fundamentalmente en nuestro país, pero también en los otros países mencionados arriba, y señalan además las áreas que recién se inician, con poca tradición en nuestro país y que deberían fomentarse. Los trabajos aquí publicados se enmarcan dentro de las siguientes disciplinas y/o campos de investigación: Fonología, Sintaxis, Semántica y Pragmática, Lingüística Cognitiva, Análisis del Discurso, Psicolingüística, Adquisición de la Lengua, Sociolingüística y Dialectología, Didáctica de la lengua, Lingüística Aplicada, Lingüística Computacional, Historia de la Lengua y la Lingüística, Lenguas Aborígenes, Filosofía del Lenguaje, Lexicología y Terminología

USO DAS TECNOLOGIAS DIGITAIS COMO SUPORTE PARA A APRENDIZAGEM NA ERA DA EDUCAÇÃO E INDÚSTRIA 4.0 - vol. 2

Chamamos aqui de principais tecnologias digitais de informação e comunicação, as comunidades virtuais de aprendizagem, a robótica, os sistemas tutoriais inteligentes, os sistemas hipermídia adaptativos, as simulações e laboratórios tradicionais e remotos e as tecnologias da realidade. Neste segundo volume do livro, discutimos o que chamamos acima de principais tecnologias digitais de informação, na forma de capítulos separados. Nele, procuramos integrar as informações teóricas trazidas no volume 1 com a prática no uso dessas tecnologias no ensino formal. Nossa esperança é que este material seja útil como bibliografia básica em cursos de pedagogia e licenciaturas, para disciplinas que tratem do uso de tecnologias digitais.&nbsp

Las plantas medicinales en el inventario español sobre los conocimientos Tradicionales relativos al patrimonio natural y a la biodiversidad

En las últimas décadas las organizaciones internacionales han puesto de manifiesto la importancia de los conocimientos ecológicos tradicionales para la conservación y el uso sostenible de la diversidad biológica, como se refleja en el Convenio sobre la Diversidad Biológica (CBD 1992). En España, el marco jurídico que recoge las normas y recomendaciones de esta estrategia es la Ley de Patrimonio Natural y Biodiveresidad 42/2007 que establece la creación del Inventario Español de Conocimientos Tradicionales (IECT) relevantes para la conservación y el uso sostenible de la biodiversidad y geodiversidad. Desde hace más de un año un equipo de más de 60 investigadores, incluyendo botánicos, zoólogos, antropólogos, ecólogos y geólogos de más de 20 centros de investigación y universidades del estado español, están trabajando en el IEDT, con el objetivo de recopilar y difundir la información publicada sobre los conocimientos tradicionales relativos a la flora, fauna, geodiversidad y el manejo de los ecosistemas

Mulher, saúde e trabalho no Brasil: desafios para um novo agir Women, health, and labor in Brazil: challenges for new action

Apesar do marcante aumento da participação das mulheres no mercado de trabalho, no Brasil, persiste um desconhecimento generalizado sobre os efeitos do trabalho sobre sua saúde. Neste artigo, em revisão da literatura, pretendeu-se identificar questões teóricas e metodológicas na análise das relações entre trabalho e saúde de mulheres a partir de uma perspectiva de gênero. Assinala-se as características da inserção profissional feminina, decorrentes de seu papel na reprodução social, que resultam na necessidade de estratégias variadas de conciliação entre as atividades profissionais e as domésticas. Discute-se as implicações da inserção social das mulheres trabalhadoras para a sua saúde e a necessidade de repensar criticamente teorias e conceitos, estratégias metodológicas, fontes de informação, para dar conta da complexidade e das especificidades das condições de trabalho e de existência das mulheres. A recente atuação das mulheres sindicalistas na defesa do direito ao corpo, à saúde e à feminilidade no ambiente de trabalho não pode prescindir de um novo olhar, que se produz de modo ainda incipiente no espaço acadêmico, por inspiração do movimento social de mulheres. Nesse sentido, apontam-se os desafios para a produção do conhecimento sobre o tema, que desvendem as singularidades da realidade nacional, caracterizada pelo desemprego, o trabalho informal, os baixos salários, a fragilidade das organizações sindicais e sociais, além de relações tradicionais familiares e matrimoniais.<br>Despite the remarkable rise in women's participation in the labor market in Brazil, its consequences on health are still virtually unknown. This study aims to identify theoretical and methodological problems in the relationship between labor and women's health from a gender perspective. Characteristics of women's occupational placement are described and analyzed as resulting from their role in social reproduction. The study examines the development of several conciliatory strategies between paid work and housework which are discussed as potential determinants of health problems and support the need for a critical reappraisal of theoretical and methodological strategies to reach a better understanding of the complexity and specificities of women's living and working conditions. The author also stresses the role of women's recent participation in the trade union movements in defense of health, body rights, and women's issues in the workplace, as well as the need for a new framework embodied in the women's social movement. The study thus points to the challenge to produce knowledge on this subject in order to unveil the uniqueness of the national scenario marked by unemployment, informal jobs, low salaries, weak trade unions and other civil organizations, and traditional domestic and marriage relationships

Sustainability of traditional ecological knowledge: importance, distribution, endemicity and conservation of Spanish medicinal plants

Trabajo presentado en la 58th Annual Meeting of the Society for Economic Botany (Living in a global world: local knowledge ans sustainability), celebrada en Braganza (Portugal) del 4 al 9 de junio de 2017.-- IECTB authors: L Aceituno, R Acosta, A Alvarez, E Barroso, J Blanco, MA Bonet, L Calvet, E Carrio, R Cavero, U DAmbrosio , L Delgado, J Fajardo, I Fernandez-Ordonez, J Garcia, T Garnatje, JA Gonzalez, R Gonzalez-Tejero, A Gras, E Hernandez-Bermejo, E Laguna, JA Latorre, C. Lopez, MJ Macia, E Marcos, V Martinez, G Menendez, M Molina, R Morales, LM Munoz, C Obon, R Ontillera, M Parada, A Perdomo, I Perez, MP Puchades, V Reyes-Garcia, M Rigat, S Rios, D Rivera, R Rodriguez, O Rodriguez, R Roldan, L San Joaquin, FJ Tardio, JR Vallejo, J Valles, H Velasco and A Verde.More than 17,000 of the plant species of the world have been used as medicines. The Mediterranean basin, and specifically Spain, has a great floristic and ethnobotanical richness, comprising its useful flora around 3,000 plant species. This paper studies medicinal plants traditionally used in Spain in order to analyze the sustainability of their exploitation. Given that sustainability is related to the amount of the resource and its gathering pressure, its availability and cultural importance were analysed based on: the number of papers cited from a selection of over 180 papers, the number of 10x10 km UTM grid cells in which the plants were represented, the number of phytosociological inventories in which the presence of the plant has been registered, and searched on their current conservation status in European, national and regional legislations. The total number of wild or naturalized medicinal species in Spain reaches 1,393, 15% of them being endemic. A positive correlation was found among cultural importance and abundance (ρ=0.48) and among cultural importance and distribution (ρ=0.502), showing that abundant widely distributed species are those more commonly used. Most of the medicinal plants (72%) do not appear on the consulted regulations and do not have any legal protection or known threat and only 11 species are registered in any of the annexes of the European Habitats directive. While this study confirms that people tend to select as medicinal abundant and widely distributed species, many other criteria are used for selecting them.Peer reviewe