2,760 research outputs found

    Update on the fluorometric measurement of enzymatic activities for Lysosomal Storage Disorder detection: The example of MPS VI

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    Lysosomal Storage Disorders (LSD) are rare diseases that as a whole havea combined incidence ranging from 1:1500 to 1:7000 live births. One of suchdiseases is Mucopolysaccharidosis VI (MPS VI), or Maroteaux Lamy Syndrome.MPS VI patients undergo devastating and irreversible skeletal alterations andmultisystemic failure as from early childhood due to reduced Arylsulfatse B(ARSB) enzyme activity.Reaching a final diagnosis is not always a short cut path, but rather a yearslongbattle against uncertainty and unnecessary medical interventions. Ouraim is to contribute from the bench table with different approaches that couldserve as alternatives to pre-existing assays for screening and diagnosing MPSVI and other LSD.The present work is based on our research article authored by Franco etal.1 where we studied the effect of blood-derived hemoglobin, and other bloodcomponents, on the fluorescence of 4-Methylumbelliferone when measuringARSB enzyme activity from dried blood spot (DBS) samples.Our experience indicates that to date there are plenty of differentapproaches for measuring ARSB enzyme activity, although the sample typerequired or the assay in itself often make them more adaptable for either highthroughput screening or small scale diagnostics.As a whole, the fluorometric determinations seem to be the mostaccessible to low budget laboratories with equally valuable performancesas a sophisticated mass spectrometry analysis for this disease. Furthermore,the DBS serves as an attractive sample type for screening the disease in largepopulations.Fil: Franco, Paula Gabriela. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Química y Físico-Química Biológicas ; Argentina. Universidad de Buenos Aires. Facultad de Farmacia y Bioquimica. Departamento de Quimica Biologica. Cátedra de Química Biológica Patológica; ArgentinaFil: Adamo, Ana María. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Química y Físico-Química Biológicas ; Argentina. Universidad de Buenos Aires. Facultad de Farmacia y Bioquimica. Departamento de Quimica Biologica. Cátedra de Química Biológica Patológica; ArgentinaFil: Mathieu, Patricia Andrea. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Química y Físico-Química Biológicas ; Argentina. Universidad de Buenos Aires. Facultad de Farmacia y Bioquimica. Departamento de Quimica Biologica. Cátedra de Química Biológica Patológica; ArgentinaFil: Perez, Maria Julia. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Química y Físico-Química Biológicas ; Argentina. Universidad de Buenos Aires. Facultad de Farmacia y Bioquimica. Departamento de Quimica Biologica. Cátedra de Química Biológica Patológica; ArgentinaFil: Setton, Clara Patricia. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Química y Físico-Química Biológicas ; Argentina. Universidad de Buenos Aires. Facultad de Farmacia y Bioquimica. Departamento de Quimica Biologica. Cátedra de Química Biológica Patológica; ArgentinaFil: Silvestroff, Lucas. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Química y Físico-Química Biológicas ; Argentina. Universidad de Buenos Aires. Facultad de Farmacia y Bioquimica. Departamento de Quimica Biologica. Cátedra de Química Biológica Patológica; Argentin

    Língu maquista: um dialeto quase extinto, unindo Portugal, Macau e Brasil

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    A língu maquista é um dialeto antigo que traduz uma das mais relevantes tradições de Macau. Este dialeto, também conhecido por patois, era comummente falado nos círculos familiares macaenses ainda no início do século XX. Porém, começa a cair em desuso a partir dos anos 30 do referido século e, actualmente, encontra-se em extinção. Citando José dos Santos Ferreira «dentro de escassos anos, dele nada mais restará senão uma pálida lembrança, como o tanger nostálgico duma suave melodia cheia de reminiscências». O presente trabalho pretende fazer a apologia da vida de um dialeto que adaptou, entre várias prosas e poesias, a lírica camoniana, deixando visíveis as reminiscências da Língua Portuguesa, como melodia nostálgica, unindo três continentes e três regiões geográficas: Portugal, Macau e Brasil

    Effect of vacuum packaging on the biochemical, viscoelastic, and sensory properties of a Spanish cheese during chilled storage

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    The unique qualities of Spanish cheeses, such as the San Simón da Costa (SSC) cheese, are protected by the Protected Designation of Origin (PDO) status. The technological importance of chilled storage at 4 °C of vacuum-packaged (V) and natural (N) (unpackaged) cheeses was examined. For this purpose, the physico-chemical, biochemical, mechanical (puncture tests), viscoelastic (oscillatory and transient tests) and sensory properties of V and N cheeses were compared and analysed. During chilled storage, the caseins in V cheeses did not undergo proteolytic reactions. Low temperature maintained a low intensity of proteolytic phenomena for up to 6 months. Lipolysis was more intense in the N than in the V samples. The moisture content decreased in the N cheeses during chilled storage, and thus, the casein matrix concentration and ionic strength increased, resulting in an increase in the gel strength (S) parameter and complex modulus (G*), and the conformational stability−high stress amplitude (σmax). The low and similar values of the n’ and n’’ exponents (mechanical spectra) and the n parameter (transient tests) indicated the high degree of the temporal stability of the cheese network in both the N and V samples, irrespective of storage time. Likewise, the similar values of the phase angle (δ) for the N and V cheeses during storage indicate energy-stable bonds in the SSC cheese matrix. The attributes of the oral tactile phase (firmness, friability, gumminess, and microstructure perception), mechanical parameters and viscoelastic moduli enabled the discrimination of the packaged and unpackaged cheeses. Cheeses chilled and stored without packaging were awarded the highest scores for sensory attributes (preference) by trained panellists.Xunta de Galicia | Ref. ED431E 2018/0

    Tristão e Isolda: mito e magia

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    Este artigo pretende explorar os elementos mágicos como desencadeadores e agregadores do mito de amor essenciais ao romance Tristão e Isolda. Pretende igualmente demonstrar que na fatalidade da paixão está a originalidade da obra e que as causas misteriosas dessa paixão estão simbolizadas pela ação do filtro mágico, admitindo dois grandes momentos míticos do romance: os amantes sob o efeito do filtro e os amantes na separação e na morte, que sugerem o impasse e as incertezas do amor humano

    Eficiencia de fructificación en madurez en trigo pan

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    Su uso en un programa de mejoramiento como carácter de selección para incrementar el número de granos/m2EEA BalcarceFil: Alonso, María Pía. Instituto Nacional de Tecnología Agropecuaria (INTA). Estación Experimental Agropecuaria Balcarce; Argentina.Fil: Alonso, María Pía. Universidad Nacional de Mar del Plata. Facultad de Ciencias Agrarias; Argentina.Fil: Franco, María Fiorella. Instituto Nacional de Tecnología Agropecuaria (INTA). Estación Experimental Agropecuaria Balcarce; Argentina.Fil: Franco, María Fiorella. Universidad Nacional de Mar del Plata. Facultad de Ciencias Agrarias; Argentina.Fil: Pontaroli, Ana Clara. Instituto Nacional de Tecnología Agropecuaria (INTA). Estación Experimental Agropecuaria Balcarce; Argentina.Fil: Pontaroli, Ana Clara. Universidad Nacional de Mar del Plata. Facultad de Ciencias Agrarias; Argentina.Fil: Pontaroli, Ana Clara. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina.Fil: Alonso, Maria Pía. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina.Fil: Franco, Maria Fiorella. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina

    Students' engagement in distance learning: creating a scenario with LMS and social network aggregation

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    Already a part of our daily lives, Web 2.0 is becoming also a part of Education, as it evolves to accompany society, education is becoming more personal, and with a focus on knowledge, reflection, social connection and engagement, as to include both digital natives and immigrants [1]. According to Siemens [2] today's learning depends on connectivity among individuals and it tends to dissolve frontiers between formal and informal learning. This paper presents a study1, that aims to comprehend how an informal platform (such as Facebook) while complement of a formal platform (such as Moodle) can contribute to a greater engagement by the Higher Education level students', as well as to measure the impact these tools can have on the knowledge acquisition process. Embedded on the Higher Education context, the study is centred in the levels of the students' engagement and on the frequency and quality of their contributions in Forums, being its participants, lecturers and students of this level of education. Since it is objective of the study to understand how interaction and collaboration contribute to students' involvement in elearning hybrid contexts the adopted theoretical framework is the Activity Theory, and the methodological approach chosen is of a mixed nature, using Social Network Analysis tools (SNA).info:eu-repo/semantics/publishedVersio
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