Epilepsy Caused by an Abnormal Alternative Splicing with Dosage Effect of the SV2A Gene in a Chicken Model

Photosensitive reflex epilepsy is caused by the combination of an individual's enhanced sensitivity with relevant light stimuli, such as stroboscopic lights or video games. This is the most common reflex epilepsy in humans; it is characterized by the photoparoxysmal response, which is an abnormal electroencephalographic reaction, and seizures triggered by intermittent light stimulation. Here, by using genetic mapping, sequencing and functional analyses, we report that a mutation in the acceptor site of the second intron of SV2A (the gene encoding synaptic vesicle glycoprotein 2A) is causing photosensitive reflex epilepsy in a unique vertebrate model, the Fepi chicken strain, a spontaneous model where the neurological disorder is inherited as an autosomal recessive mutation. This mutation causes an aberrant splicing event and significantly reduces the level of SV2A mRNA in homozygous carriers. Levetiracetam, a second generation antiepileptic drug, is known to bind SV2A, and SV2A knock-out mice develop seizures soon after birth and usually die within three weeks. The Fepi chicken survives to adulthood and responds to levetiracetam, suggesting that the low-level expression of SV2A in these animals is sufficient to allow survival, but does not protect against seizures. Thus, the Fepi chicken model shows that the role of the SV2A pathway in the brain is conserved between birds and mammals, in spite of a large phylogenetic distance. The Fepi model appears particularly useful for further studies of physiopathology of reflex epilepsy, in comparison with induced models of epilepsy in rodents. Consequently, SV2A is a very attractive candidate gene for analysis in the context of both mono- and polygenic generalized epilepsies in humans

Prefsort: an external preference mapping on weighted sorting data

Prefsort: an external preference mapping on weighted sorting data. 9. Pangborn sensory science symposiu

Le goût de l'eau et les préférences des consommateurs en matière d'eau de boisson

National audienc

Preference for meat substitute with plant-based proteins (PBP): An experiment with real product consumption

International audiencePeople are interested by meat reduction or meat substitution for many arguments: health, environment, animal welfare. Firms explore new models and new supply (better meat or meat substitutes) to take, to keep or to increase market shares. What consumers really prefer and choose? In choice experiments

Natural meat vs. Plant-Based Protein : What drives consumer choice ?

International audiencePeople are interested by meat reduction or meat substitution for many arguments: health, environment, animal welfare. Firms explore new models and new supply (better meat or meat substitutes) to take, to keep or to increase market shares. What consumers really prefer and choose? In choice experiments

Preference for meat substitute with plant-based proteins (PBP): An experiment with real product consumption

International audiencePeople are interested by meat reduction or meat substitution for many arguments: health, environment, animal welfare. Firms explore new models and new supply (better meat or meat substitutes) to take, to keep or to increase market shares. What consumers really prefer and choose? In choice experiments

Natural meat vs. Plant-Based Protein : What drives consumer choice ?

International audiencePeople are interested by meat reduction or meat substitution for many arguments: health, environment, animal welfare. Firms explore new models and new supply (better meat or meat substitutes) to take, to keep or to increase market shares. What consumers really prefer and choose? In choice experiments

Preferences & choices experiments with real products consumption: application with plant-based proteins

International audienceIn a context of vegetablization of meals, a range of plant-based substitutes is developing that imitate the taste and nutritional properties of meat products. Moreover, the motivations to reduce the consumption of meat products or to replace them by plant-based substitutes are based on several arguments such as health, environment or animal welfare. Through two studies, we explore the preferences for plant-based meat substitutes by combining the tools of sensory evaluation with those of experimental economics. Thus, subjects taste real products and express themselves about them, then they are exposed to choices related to these products (maintaining consumption or returning to their usual product). Our results show that a third of the participants appreciate the substitutes and are ready to renew their consumption.Dans un contexte de végétalisation des repas se développe une offre de substituts végétaux imitant les propriétés gustatives et nutritionnelles des produits carnés. Par ailleurs, les motivations à réduire la consommation de produits carnés ou à les remplacer par des substituts végétaux repose sur plusieurs arguments tels que la santé, l’environnement ou le bien-être animal. A travers deux études, nous explorons les préférences pour des substituts végétaux à la viande en associant les outils de l’évaluation sensorielles à ceux de l’économie expérimentale. Ainsi les sujets dégustent des produits réels et s’expriment à leur sujet, puis ils sont soumis à des choix relatifs à ces produits (maintient de la consommation ou retour à leur produit habituel). Nos résultats montre qu’un tiers des participants apprécient les substituts et qu’ils sont prêt à en renouveler la consommation

Preferences & choices experiments with real products consumption: application with plant-based proteins

International audienceIn a context of vegetablization of meals, a range of plant-based substitutes is developing that imitate the taste and nutritional properties of meat products. Moreover, the motivations to reduce the consumption of meat products or to replace them by plant-based substitutes are based on several arguments such as health, environment or animal welfare. Through two studies, we explore the preferences for plant-based meat substitutes by combining the tools of sensory evaluation with those of experimental economics. Thus, subjects taste real products and express themselves about them, then they are exposed to choices related to these products (maintaining consumption or returning to their usual product). Our results show that a third of the participants appreciate the substitutes and are ready to renew their consumption.Dans un contexte de végétalisation des repas se développe une offre de substituts végétaux imitant les propriétés gustatives et nutritionnelles des produits carnés. Par ailleurs, les motivations à réduire la consommation de produits carnés ou à les remplacer par des substituts végétaux repose sur plusieurs arguments tels que la santé, l’environnement ou le bien-être animal. A travers deux études, nous explorons les préférences pour des substituts végétaux à la viande en associant les outils de l’évaluation sensorielles à ceux de l’économie expérimentale. Ainsi les sujets dégustent des produits réels et s’expriment à leur sujet, puis ils sont soumis à des choix relatifs à ces produits (maintient de la consommation ou retour à leur produit habituel). Nos résultats montre qu’un tiers des participants apprécient les substituts et qu’ils sont prêt à en renouveler la consommation

Perception, préférence et comportement des consommateurs vis-à-vis d eaux embouteillées et d eaux du robinet

Pour des raisons économiques et environnementales, boire de l eau du robinet est préférable à boire de l eau en bouteille. Mais l eau du robinet a-t-elle le même goût que l eau en bouteille ? Existe-t-il différents types de goûts d eau ? Dans l affirmative, en quoi ces différences de goût impactent-elles les préférences des consommateurs et leur choix en matière d eau de boisson ? Finalement, comment identifier des leviers de la consommation d eau du robinet ? L étude de la perception du goût de l eau a permis de mettre en évidence trois principaux liés à la minéralité totale des eaux dégustées. Les eaux du robinet, une fois déchlorées, s inscrivent dans le même espace sensoriel que les eaux embouteillées. Ces résultats ont conduit à l élaboration d une nouvelle méthode d analyse sensorielle (Positionnement Sensoriel Polarisé). Trois principaux profils de préférences ont été identifiés : la majorité des consommateurs préfère les eaux de minéralité moyenne au goût neutre, un autre segment aime les eaux de faible minéralité au goût amer et métallique et enfin un petit segment semble préférer les eaux au goût prononcé qu il soit amer ou salé. Ces profils de préférences ont pu en partie être expliqués par la région d habitation et les habitudes de consommation en matière d eau de boisson. Toutefois, davantage que les préférences, c est l image de l eau du robinet qui s est avérée être le facteur déterminant du comportement des consommateurs en matière d eau à boire. Ainsi, les meilleurs leviers de la consommation d eau du robinet relèveraient d actions de communication et d éducation du consommateur.For environmental and economic considerations, drinking tap water rather than bottled water is strongly advisable. But are the taste of tap waters and the taste of bottled waters the same? What are the tastes of water? What are the preferences of consumers among these tastes? And finally what are the keys of tap water consumption? The study of the perception of water highlighted three main tastes bitter, tasteless, salty linked to the total amount of minerals. Presented with no chlorine, the sensory space of tap waters is included in the larger space of bottled waters. These results enabled the development of a new sensory methodology (Polarized Sensory Positioning) which consists in comparing a sample of water to three references chosen as prototypes of these three main tastes. Three main preference profiles have been determined: most consumers prefer waters with medium mineralization with no particular taste, another cluster likes low mineralized waters, bitter and metallic and finally a small percentage of the population seems to like waters with a pronounced taste, whatever bitter or salty. These preference profiles have been partly explained by the living place and consumer behaviors toward drinking water. Nevertheless, more than the preferences, the image of tap water seems to be the most important factor of consumer s behavior. The keys of tap water consumption would be communication and education of consumers.DIJON-BU Sciences Economie (212312102) / SudocSudocFranceF