1,721,061 research outputs found
Monaco (Antonio). — L'alternance école-production
Gonnin-Bolo Annette. Monaco (Antonio). — L'alternance école-production. In: Revue française de pédagogie, volume 110, 1995. Filles et garçons devant l'école. pp. 129-132
Monaco (Antonio). — L'alternance école-production
Gonnin-Bolo Annette. Monaco (Antonio). — L'alternance école-production. In: Revue française de pédagogie, volume 110, 1995. Filles et garçons devant l'école. pp. 129-132
Protein aggregation and autophagy dysfunction: new lessons from mucopolysaccharidoses
Mucopolysaccharidoses (MPS) are inherited metabolic diseases with strong neurological involvement. MPSs are caused by defects in lysosomal enzymes involved in the degradation of glycosaminoglycans (GAGs), which consequently accumulate into the lysosomes as primary storage. Macroautophagy/autophagy impairment is well known to drive neurodegeneration in MPSs, however, mechanisms underlying such dysfunction are still poorly understood. Recently, by studying a mouse model for MPS-III (Sanfilippo syndrome) we have shown that the progressive aggregation of amyloid proteins in neuronal cell bodies occurs downstream of the GAG storage and, in turn, impairs the autophagy pathway by affecting lysosomal-dependent autophagosome clearanc
Protein Aggregation and Dysfunction of Autophagy-Lysosomal Pathway: A Vicious Cycle in Lysosomal Storage Diseases
Many neurodegenerative conditions are characterized by the deposition of protein aggregates (mainly amyloid-like) in the central nervous system (CNS). In post-mitotic CNS cells protein aggregation causes cytotoxicity by interfering with various cellular functions. Mutations in different genes may directly cause protein aggregation. However, genetic factors together with aging may contribute to the onset of protein aggregation also by affecting cellular degradative functions, in particular the autophagy-lysosomal pathway (ALP). Increasing body of evidence show that ALP dysfunction and protein aggregation are functionally interconnected and induce each other during neurodegenerative processes. We will summarize the findings supporting these concepts by focusing on lysosomal storage diseases (LSDs), a class of metabolic inherited conditions characterized by global lysosomal dysfunction and often associated to a severe neurodegenerative course. We propose a model by which the inherited lysosomal defects initiate aggregate-prone protein deposition, which, in turns, worsen ALP degradation function, thus generating a vicious cycle, which boost neurodegenerative cascades
Linac Based STereotactic Arrhythmia Radioablation (STAR) of Atrial fibrillation
Background and Aims: Stereotactic arrhythmia radioablation (STAR) is a novel therapeutic
approach for cardiac arrhythmias. The aim of this trial is to investigate the feasibility of STAR for the
treatment of paroxysmal atrial fibrillation (AF) in elderly patients.
Method: inclusion criteria were: age > 70 years, symptomatic AF, antiarrhythmic drugs failure or
intolerance. All patients underwent to 4D cardiac Computed Tomography simulation. The clinical
target volume was identified in the area around pulmonary veins (PV). STAR was performed with a
total dose of 25Gy (single fraction) delivered in 3 minutes.
Results: Twenty patients were enrolled and 18 underwent STAR. One patient withdrew informed
consent before treatment and 1 patient was excluded due to unfavorable esophagus position. With a
median follow-up (FU) of 16 months (range 12-23), no acute toxicity more than grade 3 was reported.
Five patients had a grade 1 esophagitis 24 hours after STAR; eight patients had an asymptomatic
grade 1 pericardial effusion, one patient had a torsade de pointes treated effectively by electrical
cardioversion and subsequent cardiac ICD implantation. Most patients had a significant reduction in
AF episodes. Five patients, due to arrhythmias recurrences after STAR, performed
electrophysiological study documenting successful PV isolation. Finally, a significant improvement
of quality of life was documented (48±15 at enrollment vs 75±15 at 12 months FU; p<0.001).
Conclusion: The present phase II trial demonstrated the feasibility of STAR in paroxysmal AF elderly
patients and its potential role in increasing the quality of life. Surely, more robust data are needed
about safety and efficacy
Effects of leptin on FSH cells in the pituitary gland of Podarcis siculus
Leptin is the hormone synthesised by adipocytes, which plays an important role in regulating appetite and metabolism. In mammals, this pleiotropic hormone also plays a key role in controlling gonadotropin secretion by stimulatory hypothalamic and pituitary actions. However, little is known about leptin in lower vertebrates and particularly few studies are available on reptiles. In the present work, we analysed the action of recombinant human leptin on FSH cells in the pituitary gland of Podarcis siculus female lizards exposed to four different concentrations of the hormone. FSH cells showed a dose-dependent reaction. The data are indicative of the role played by leptin in modulating the cellular activity of such cells in the pituitary gland of P. siculus, similar to what was already reported in mammals. A functional receptor is evidently able to respond to leptin in this lizard, but further comparative studies are needed to understand the role of this hormone in ectothermic vertebrates
Neuroglial alterations in the zebrafish brain exposed to cadmium chloride
Cadmium is an extremely toxic heavy metal that widely occurs in industrial workplaces with various hazardous effects on brain functions. The cytotoxic effects of cadmium chloride (CdCl2 ) on the neuroglial components of the zebrafish brain were analysed by detecting the glial fibrillary acidic protein (GFAP) expression and the mRNA levels of myelin genes mbp, mpz and plp1 in adult specimens exposed to cadmium for 2, 7 and 16 days. A significant decrease in the GFAP protein by Western blotting experiments was observed after 2 days of treatment, reaching 55% after 16 days. No change was observed in the mRNA levels. Using immunohistochemistry, a reduction in GFAP-positive structures was revealed with a progressive trend in all the brains at 2, 7 and 16 days of treatment. In particular, a considerable reduction in GFAP-positive fibres, with a different course, was observed in the ventricle areas and at the pial surface and in blood vessels after 16 days. Our experiments also showed a structural and chemical alteration of myelin and upregulation of mpz mRNA levels, the oligodendrocyte gene that is upregulated in experiments of neuronal injury, but not of plp1 and mbp mRNA levels, other myelin structural genes. These data confirm the toxic action of cadmium on the zebrafish brain. This action is time-dependent and involves the glial cells, key components of the protection and function of nerve cells, hence the basis for many neurological diseases
Going Beyond Counting First Authors in Author Co-citation Analysis
The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
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