FoxO3a overexpression prevents both glycogen overload and autophagic buildup in skeletal muscle of Pompe disease

FoxO3a overexpression prevents both glycogen overload and autophagic buildup in skeletal muscle of Pompe disease. 6eme congrès international de Myologi

Satellite cells fail to contribute to muscle repair but are functional in Pompe disease (glycogenosis type II)

Satellite cells fail to contribute to muscle repair but are functional in Pompe disease (glycogenosis type II). 6eme congrés international de Myologi

Cadeia produtiva do gengibre (Zingiber officinale ROSCOE) no estado do Paraná : análise e recomendações para melhoria da qualidade

Orientador : Raquel R.B.NegrelleCo-orientadores: Luiz Doni Filho, Neusa G.A. RuckerTese (doutorado) - Universidade Federal do Paraná. Setor de Ciências Agrárias. Curso de Pós-Graduação em Agronomia - área de concentração Produção Vegetal, Departamento de Fitotecnia e FitossanitarismoInclui bibliografia e glossárioResumo: O presente trabalho teve como objetivo o estudo prospectivo da cadeia produtiva do Zingiber officinale Roscoe no Estado do Paraná, identificando os principais agentes envolvidos e paralelamente proceder análise dos pontos de estrangulamentos, especificamente no que concerne à qualidade microbiológica deste produto em todos os níveis, desde a produção até a fase final da comercialização, visando identificar causas e propor soluções no sentido da melhoria do sistema como um todo. Este estudo foi desenvolvido no período de 2000 a 2002 englobando pesquisa de campo, entrevistas abertas com representantes de estabelecimentos de comercialização, produtores e demais atores da cadeia produtiva, além de análises laboratoriais do produto "in natura" disponível no mercado consumidor da Região Metropolitana de Curitiba - PR. Este documento está organizado em 9 capítulos, que apresentam vastas informações englobando aspectos botânicos, ecológicos, fisico-químicos e farmacológicos do gengibre, e que estão incluídas nos capítulos 1 e 2. O capítulo 3 apresenta características gerais da principal região produtora brasileira, Morretes - PR, englobando localização, aspectos sócio-econômicos, geológicos e geomorfológicos e caracterização climática. O capítulo 4 apresenta o estudo prospectivo da cadeia produtiva do gengibre no Estado do Paraná, que engloba panorama mundial, brasileiro e paranaense do volume de produção agrícola de gengibre. Inclui-se também caracterização da comunidade produtora agrícola do litoral paranaense, além da identificação e caracterização dos outros diferentes níveis da cadeia produtiva do gengibre e a detecção dos principais pontos de estrangulamento nos diferentes níveis desta cadeia produtiva. A caracterização do cultivo e beneficiamento do gengibre no litoral paranaense é apresentada no capítulo 5, evidenciando suas particularidades frente ao descrito na literatura especializada. No capítulo 6 avaliou-se as condições higiênico-sanitárias dos estabelecimentos produtores, do processo de beneficiamento pós-colheita (lavagem, limpeza, secagem e embalagem) e das condições de manipulação do gengibre "in natura" no litoral paranaense. De maneira geral, as condições de higiene e limpeza observadas foram consideradas precárias, determinando alta potencialidade de contaminação do gengibre com agentes que poderiam colocar em risco a saúde do consumidor. No capítulo 7 são apresentados os resultados dos estabelecimentos de comercialização da Região Metropolitana de Curitiba - PR, no que tange à qualidade e adequação às normas vigentes. Dos aspectos observados, as condições dos vestuários e dos equipamentos de proteção individual (manipuladores) foram considerados precários, o que pode potencializar a contaminação do produto e colocar em risco a saúde do consumidor. O capítulo 8 teve como objetivo caracterizar o perfil microbiológico do gengibre "in natura" comercializado na Região Metropolitana de Curitiba - PR, tendo como base a Resolução - CNPPA n. 12 - Brasil, 1978 e a Resolução - RDC n. 12 - Brasil, 2001. Para tanto, foram realizadas a determinação do número mais provável (NMP) de coliformes totais, coliformes a 45° C/g e Escherichia coli, e a presença de Salmonella sp em 25 gramas. Sintetizando, um conjunto de propostas e recomendações aos agentes econômicos que atuam e processam a cadeia produtiva do gengibre no Estado do Paraná, em especial o município de Morretes - PR, é apresentado no capítulo 9Abstract: Prospective studies of the productive chain of the Zingiber officinale Roscoe on Parana State identifying the main agents envolved and parallel procedure analysis of the strangulation points, in order to microbiology qualify this product in whole levels from the production until commercialization last phase, aiming identify causes and propose solutions to the improvement of the system in general. This survey developed from 2000 to 2002 involved field research, interviews with representative of the commercialization establishments, producers and others actors of the productive chain, besides made in laboratories of the quality of crude product available to the consumer market in Curitiba. This paper is organized in nine chapters that present information involving botanical, ecological, physique-chemical and pharmacological aspects of ginger that are included in chapters 1 and 2. The chapter 3 presents the characteristics of the main brazilian productive area, embody localization, social and economic aspects, geologycal, geomorphologycal and climate characterization. The chapter 4 the study of the productive chain of this product in Parana State, which includes the paranaense, the brazilian and the worldwide panoramic view of the production volume and commercialization of the ginger; characterization of the agricultural community; identification and characterization of others different levels of the productive chain and also, the main strangulation points on these different levels of the productive chain. In the chapter 5 the agricultural process of the ginger benefit and cultivation in Morretes Town, Paraná, Brazil was caracterized. In the chapter 6 the hygienic-sanitary conditions of the producers establishments, postharvest benefit system and manipulation conditions of crude ginger in the paranaense coastland, Brazil were evaluated with an aim to subsidy the ofert of the best quality product to consumer market. In general, the cleaning and hygienic conditions were precarious, determining a high potential of ginger contamination with agents that can put in risk the consumers' health. The chapter 7 presents the results of the commercialization establishments in the metropolitan area of Curitiba considering adequation to the current norm. Of the observed items, the manipulation conditions was considered precarious due to the inadequate conditions that contributes to contamination, bringing about serious problems to consumers' health. The chapter 8 aimed the microbiological quality of the ginger market in the metropolitan area of Curitiba based on the Brazilian legislation. The microbiological analysis found total coliforms, 45° C/g coliforms, Escherichia coli most probable number (MPN) and Salmonella sp presence. Synthetizing, a group of considerations and recommendations to economic agents that actuate and carry on the productive chain of the ginger in the Parana State, specially in Morretes Town, is presented in the chapter

Organ Clearing and Biphotonic Microscopy: an Innovative Complementary Technological Approach to Investigatethe Central Nervous System

3D imaging of the central nervous system at the microscopic level is essential to investigate morphological changes of various diseases or to assess the efficacy of a treatment (Denk et Horstman, 2004). However, this 3D exploration of the tissues is very limited because of the small volumes and the opacity of the tissues, which does not allow the passage of the light. Technological advances in biphotonic and light-sheet microscopy together with the development of numerous methods in tissue clearing represent innovative solutions for exploring the organs at the cellular level (Hama H. et al., 2011 ; Chung K. et al., 2013, Chenchen P. et al. 2016). These methods are very promising tools to assess new therapeutic strategies on neurodegenerative diseases developed by our research unit UMR703 using animal models and AAV vector encoding fluorescent proteins.For our study we used Scale, Clarity and uDISCO clearing methods and biphotonic imaging on thick brain sections. Harmonic and fluorescence signals conservation results will be discussed

Peptido-targeting of the mitochondrial transition pore complex for therapeutic apoptosis induction.

The permeability transition pore (PTPC), a polyprotein complex, participates in the mitochondrial homeostasis as well as in the mitochondrial phase of the intrinsic pathway of apoptosis. It integrates multiple death signals including alterations of the intracellular milieu, translocation of pro-apoptotic members of the Bax/Bcl-2 family, p53, and viral proteins. As a consequence, PTPC can act as a coordinator of the pro-apoptotic mitochondrial membrane permeabilization process and the release of pro-apoptotic intermembrane space proteins into the cytosol. Moreover, the deregulation of PTPC has been involved in several major human pathologies such as cancer, neurodegeneration, ischemia/reperfusion, aging, as well as hepatotoxicity. Therefore, PTPC has emerged as a promising potential therapeutic target. Here, we will review the current knowledge concerning the two opposite functions of the PTPC and its implication in various pathologies. We will discuss the possibility to target this complex with peptides to modulate apoptosis in an innovative therapeutic perspective

Organ clearing and biphotonic microscopy – an innovative complementary technological approach to investigate the central nervous system

Organ clearing and biphotonic microscopy – an innovative complementary technological approach to investigate the central nervous system. Sfµ 201

La Microspectroscopie Infra-Rouge : une méthode innovante pour suivre l’évolution de la maladie de Pompe dans le modèle murin GAA-KO 6neo/6neo

La Microspectroscopie Infra-Rouge : une méthode innovante pour suivre l’évolution de la maladie de Pompe dans le modèle murin GAA-KO 6neo/6neo. XXXème congrès de l'AFH: les nouveaux mondes de l'histologi

Diverse profiles of N‐acyl‐homoserine lactone molecules found in cnidarians

Many marine habitats, such as the surface and tissues of marine invertebrates, including corals, harbour diverse populations of microorganisms, which are thought to play a role in the health of their hosts and influence mutualistic and competitive interactions. Investigating the presence and stability of quorum sensing (QS) in these ecosystems may shed light on the roles and control of these bacterial communities. Samples of 13 cnidarian species were screened for the presence and diversity of N-acyl-homoserine lactones (AHLs; a prevalent type of QS molecule) using thin-layer chromatography and an Agrobacterium tumefaciens NTL4 biosensor. Ten of 13 were found to harbour species-specific, conserved AHL profiles. AHLs were confirmed in Anemonia viridis using liquid chromatography tandem mass spectrometry. To assess temporal role and stability, AHLs were investigated in A. viridis from intertidal pools over 16 h. Patterns of AHLs showed conserved profiles except for two mid-chain length AHLs, which increased significantly over the day, peaking at 20:00, but had no correlation with pool chemistry. Denaturing gel electrophoresis of RT-PCR-amplified bacterial 16S rRNA showed the presence of an active bacterial community that changed in composition alongside AHL profiles and contained a number of bands that affiliate with known AHL-producing bacteria. Investigations into the quorum sensing-controlled, species-specific roles of these bacterial communities and how these regulatory circuits are influenced by the coral host and members of the bacterial community are imperative to expand our knowledge of these interactions with respect to the maintenance of coral health

Efficient CNS targeting in adult mice by intrathecal infusion of single-stranded AAV9-GFP for gene therapy of neurological disorders

Adeno-associated virus (AAV) gene therapy constitutes a powerful tool for the treatment of neurodegenerative diseases. While AAVs are generally administered systemically to newborns in preclinical studies of neurological disorders, in adults the maturity of the blood-brain barrier (BBB) must be considered when selecting the route of administration. Delivery of AAVs into the cerebrospinal fluid (CSF) represents an attractive approach to target the central nervous system (CNS) and bypass the BBB. In this study, we investigated the efficacy of intra-CSF delivery of a single-stranded (ss) AAV9-CAG-GFP vector in adult mice via intracisternal (iCist) or intralumbar (it-Lumb) administration. It-Lumb ssAAV9 delivery resulted in greater diffusion throughout the entire spinal cord and green fluorescent protein (GFP) expression mainly in the cerebellum, cortex and olfactory bulb. By contrast, iCist delivery led to strong GFP expression throughout the entire brain. Comparison of the transduction efficiency of ssAAV9-CAG-GFP versus ssAAV9-SYN1-GFP following it-Lumb administration revealed widespread and specific GFP expression in neurons and motoneurons of the spinal cord and brain when the neuron-specific synapsin 1 (SYN1) promoter was used. Our findings demonstrate that it-Lumb ssAAV9 delivery is a safe and highly efficient means of targeting the CNS in adult mice