Trehalose alleviates the phenotype of Machado–Joseph disease mouse models

Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3, is the most common of the dominantly inherited ataxias worldwide and is characterized by mutant ataxin-3 aggregation and neuronal degeneration. There is no treatment available to block or delay disease progression. In this work we investigated whether trehalose, a natural occurring disaccharide widely used in food and cosmetic industry, would rescue biochemical, behavioral and neuropathological features of an in vitro and of a severe MJD transgenic mouse model.This work was funded by BioBlast Pharma, the ERDF through the Regional Operational Program Center 2020, Competitiveness Factors Operational Program (COMPETE 2020) and National Funds through FCT (Foundation for Science and Technology) - SFRH/BD/87404/2012, BrainHealth2020 projects (CENTRO-01-0145-FEDER-000008), ViraVector (CENTRO-01-0145FEDER-022095), CortaCAGs (POCI-01-0145-FEDER-016719), SpreadSilenc‑ing POCI-01-0145-FEDER-029716 and POCI-01-0145-FEDER-007440, as well as the SynSpread, ESMI and ModelPolyQ under the EU Joint ProgramNeurodegenerative Disease Research (JPND), the last two co-funded bythe European Union H2020 program, GA No. 643417; by National Ataxia Foundation (USA), the American Portuguese Biomedical Research Fund (APBRF) and the Richard Chin and Lily Lock Machado–Joseph Disease Research Fund.info:eu-repo/semantics/publishedVersio

Objeto de Aprendizagem Gamificado 2D na Modalidade EAD como Forma de Inclusão Social para o Desenvolvimento de Competências Profissionais

This study is part of a larger research, carried out in a profestional master's degree in education and new technologies. From a focus of social and profestional inclusion of young people from 14 to 24 years old, it involved the construction of a 2D Gamified Learning Object (LO), as a complementary part of a profestional training program in the totally distance modality. The purpose of this object was to create more involvement, a sense of reality and engagement of these students in the profestional world. The planning and construction of this LO included the creation of learning situations that sought more real experiences, with content related to the profestional daily life, encompassing gamified mechanisms and immersive structures to promote the acquisition of competences, skills and attitudes necestary for these young people to work in the world. of work. At the same time that the apprentices carried out this profestional program at a distance, they already applied it immediately to their reality and were also evaluated, so that this OA could also be validated by 66 students. The theoretical support for this study was from the authors: Zichermann and Cunningham (2011); Wiley (2011); Behar (2013); Bates (2016), among others. The methodology of this research is based on a case study, which emphasized four important events: 1) the 2D learning design of an OA; 2) its integration with the educational program in the distance modality already established in SENAC-PR, 3) its evaluation by the apprentices; 4) and its validation. The results obtained after the completion of this program and the application of a questionnaire to the participants are adequate for the development of skills aimed at profestional work, as well as the elements of gamification involved in the activity, understood as an appropriate language, although with its limitations, to the public target group in question. The relevant aspect to highlight is inclusive education, with improved self-seem and engagement, reaching a good level of satisfaction in a context of great lack in the offer of face-to-face educational products, making Distance Education viable and necestary. Keywords: Development of competences. Immersive environments. On-line education. Profestional education. Learning object.  Este estudo é parte da uma pesquisa maior, realizada num mestrado profissional em educação e novas tecnologias. A partir de um foco de inclusão social e profissional de jovens de 14 a 24 anos, envolveu a construção de um Objeto de Aprendizagem (OA) gamificado 2D, como parte complementar de um programa de formação profissional na modalidade educacional totalmente a distância. A finalidade deste objeto visou criar mais envolvimento, senso de realidade e engajamento destes alunos no mundo profissional. O planejamento e a construção deste OA incluíram a criação de situações de aprendizagem que buscassem vivências mais reais, com conteúdos ligados ao cotidiano profissional, abrangendo mecanismos gamificados e estruturas imersivas para promover a aquisição de competências, habilidades e atitudes necestárias para atuação destes jovens no mundo do trabalho. Ao mesmo tempo em que os aprendizes realizavam este programa profissional a distância, eles já aplicavam imediatamente em sua realidade e eram também avaliados, de forma que este OA também pôde ser validado por 66 alunos. O suporte teórico deste estudo foi dos autores Zichermann e Cunningham (2011); Wiley (2011); Behar (2013); Bates (2016), entre outros. A metodologia desta pesquisa pauta-se em um estudo de caso, o qual enfatizou quatro importantes eventos: 1) o design de aprendizagem em 2D de um OA; 2) sua integração com o programa educacional na modalidade a distância já estabelecido no SENAC- PR, 3) sua avaliação pelos aprendizes; 4) sua validação. Os resultados obtidos, após a realização deste programa e aplicação de questionário aos participantes, mostram-se adequados ao desenvolvimento de competências voltadas ao trabalho profissional, assim como os elementos da gamificação envolvidos na atividade, entendidos como uma linguagem apropriada, embora com suas limitações, ao público-alvo em questão. O aspecto relevante a destacar é a educação inclusiva, com melhoria de autoestima e engajamento, alcançando bom nível de satisfação em  contexto de grande carência na oferta de produtos educacionais presenciais, tornando-se viável e necestária a EAD. Palavras-chave: Desenvolvimento de competências. Ambientes imersivos. Educação a Distância. Educação profissional. Objeto de aprendizagem.  

Electrochemical and bioelectrocatalytical properties of novel block-copolymers containing interacting ferrocenyl units

The electrochemical characterization of three different polystyrene-b-polybutadiene block copolymers functionalized with ferrocenyl units electronically communicated, PSm-PBn(HSiMeFc2)p where m=615, n=53, p=39 (1), m=375, n=92, p=76 (2) and m=455, n=204, p=170 (3), has been carried out both in solution and electrochemically deposited onto platinum electrodes. The bioelectrocatalytical properties of electrodes modified with the polymers in the nicotinamide dinucleotide (NADH) and glucose oxidase (GOx) oxidations have been investigated as a function of the constitution and structure of the polymers. The analytical properties of electrodes modified with these polymers as sensors of NADH and GOx are described. In addition, an amperometric biosensor for glucose, prepared by electrostatic immobilization of glucose oxidase onto a platinum electrode modified with one of the ferrocenyl block copolymers as an example, has been developed. The results confirm that electrodes modified with the examined copolymers act as efficient redox mediators for the electrocatalytic oxidation of both reduced nicotinamide dinucleotide cofactor and glucose oxidase. The reaction with NADH proceeds via formation of a charge-transfer intermediate before yielding the reaction products. This is a novel example of electrodes modified with ferrocene derivatives that can be applied to the determination of NADH without the use of diaphorase. The redox copolymers co-immobilized with glucose-oxidase have been successfully used as amperometric biosensors for glucose determinations. As expected these compounds allow using lower working potentials. The sensitivities and detection limits obtained are comparable or even better than those of other ferrocene-modified polymers mediator electrodes

MPK6, sphinganine and the \u3ci\u3eLCB2a\u3c/i\u3e gene from serine palmitoyltransferase are required in the signaling pathway that mediates cell death induced by long chain bases in \u3ci\u3eArabidopsis\u3c/i\u3e

• Long chain bases (LCBs) are sphingolipid intermediates acting as second messengers in programmed cell death (PCD) in plants. Most of the molecular and cellular features of this signaling function remain unknown. • We induced PCD conditions in Arabidopsis thaliana seedlings and analyzed LCB accumulation kinetics, cell ultrastructure and phenotypes in serine palmitoyltransferase (spt), mitogen-activated protein kinase (mpk), mitogenactivated protein phosphatase (mkp1) and lcb-hydroxylase (sbh) mutants. • The lcb2a-1 mutant was unable to mount an effective PCD in response to fumonisin B1 (FB1), revealing that the LCB2a gene is essential for the induction of PCD. The accumulation kinetics of LCBs in wild-type (WT) and lcb2a-1 plants and reconstitution experiments with sphinganine indicated that this LCB was primarily responsible for PCD elicitation. The resistance of the null mpk6 mutant to manifest PCD on FB1 and sphinganine addition and the failure to show resistance on pathogen infection and MPK6 activation by FB1 and LCBs indicated that MPK6 mediates PCD downstream of LCBs. • This work describes MPK6 as a novel transducer in the pathway leading to LCBinduced PCD in Arabidopsis, and reveals that sphinganine and the LCB2a gene are required in a PCD process that operates as one of the more effective strategies used as defense against pathogens in plants

Caracterização de sistemas de produção por zonas agroecológicas: a experiência do Centro Nacional de Pesquisa de Milho e Sorgo.

E recomendavel que as analises do setor agricola considerem a interacao entre aspectos ambientais e socio-economicos. Este tipo de abordagem implica a necessidade de uma grande quantidade de recursos fisicos, humanos e financeiros, o que tem limitado a amplitude geografica dos estudos com este tipo de preocupacao. Propoe-se uma metodologia que estabelece criterios de separacao no espaco agricola, considerando recursos naturais e aspectos socio-economicos. Por delimitar as fronteiras geograficas a partir de criterios agroecologicos, e nao politicos, ela permite a extrpolacao dos resultados para regioes com caracteristicas semelhantes. Esta metodologia fornece melhor balanco entre amplitude geografica de extrpolacao, identificacao dos aspectos realmente importantes para definicao politicas de intervencoes no setor agricola e baixo custo de execucao. E apresentado um exemplo de aplicacao da metodologia, com adescricao dos procedimentos empregados na sua aplicacao em relacao a producao de milho em uma regiao especifica

Avaliação dos impactos econômicos, sociais e ambientais da cultivar de sorgo granífero BR 304 na safra 2005/2006.

bitstream/CNPMS/20496/1/Doc_67.pd

Caracterização de sistemas de produção por zonas agroecológicas: a experiência do Centro Nacional de Pesquisa de Milho e Sorgo.

E recomendavel que as analises do setor agricola considerem a interacao entre aspectos ambientais e socio-economicos. Este tipo de abordagem implica a necessidade de uma grande quantidade de recursos fisicos, humanos e financeiro, o que tem limitado a amplitude geografica dos estudos com este tipo de preocupacao. Propoe-se uma metodologia que estabelece criterios de separacao no espaco agricola, considerando recursos naturais e aspectos socio-economico. Por delimitar as fronteiras geograficas a partir de criterios agroecologicos, e nao politicos, ela permite a estrapolacao dos resultados para regioes com caracteristicas semelhantes. Esta metodologia fornece melhor balanco entre amplitude importantes para definicao politicas de intervencoes no setor agricola e baixo custo de execucao. E apresentado um exemplo de aplicacao da metodologia, com a descricao dos procedimentos empregados na sua aplicacao em relacao a producao de milho em uma regiao especifica

Avaliação dos impactos econômicos, sociais e ambientais da cultivar de milho BR 201 na safra 2005/2006.

bitstream/CNPMS/20495/1/Doc_66.pd