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<dc:title xml:lang="fr">Etude des dysmorphoses et de la croissance de la mandibule chez un modèle murin de la dysplasie ectodermique hypohidrotique</dc:title>
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<dc:subject xml:lang="fr">Dysplasie ectodermique hypohidrotique</dc:subject>
<dc:subject xml:lang="fr">Mandibule</dc:subject>
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<dc:subject xml:lang="fr">Analyse de Fourier elliptique</dc:subject>
<dc:subject xml:lang="fr">Croissance</dc:subject>
<dc:subject xml:lang="en">Hypohidrotic ectodermal dysplasia</dc:subject>
<dc:subject xml:lang="en">Mandible</dc:subject>
<dc:subject xml:lang="en">Images Analysis</dc:subject>
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<dcterms:abstract xml:lang="fr">La dysplasie ectodermique hypohidrotique liée à l’X (DEX) est consécutive à la mutation du gène Eda. Ce projet de recherche avait pour but d’étudier les dysmorphoses cranio-faciales chez le mutant murin Tabby représentant l’équivalent phénotypique de la DEX. La forme des mandibules en vue latérale a été étudiée à partir d’approches quantitatives associant μ-CT, traitement d’images, analyses de Fourier elliptique et analyses métriques. Une première étude ex vivo menée sur 39 spécimens Tabby et 35 souris wild-type adultes a permis de mettre en évidence un hypo-développement mandibulaire chez Tabby. Deuxièmement, une étude longitudinale in vivo de la croissance mandibulaire mise en place sur une cohorte de 23 individus (12 WT et 11 Tabby) a montré que les individus Tabby présentaient cet hypodéveloppement dès le 1er mois et qu’il se maintenait à la fin de la première année de vie. Les défauts du gène Eda affectent ainsi le développement de la mandibule en plus des dérivés ectodermiques.</dcterms:abstract>
<dcterms:abstract xml:lang="en">The X-linked hypohidrotic ectodermal dysplasia (XLHED) is the result of Eda gene defect. This research project studied the cranio-facial dysmorphoses in Tabby murin mutant which having a similar phenotype to the XLHED. A association of mutiple quantitive approachs (μ-CT, images processing, elliptical Fourier analyse and metric analyses) permitted to study the mandible’s shape in a lateral view. A first ex vivo study led on 39 specimens Tabby and 35 WT mice allowed to highlight a mandibular hypodevelopment in Tabby. Secondly, a longitudinal in vivo study of mandibular growth, based on 23 specimen (12WT and 11 Tabby), showed that Tabby presented this hypodevelopment from the 1st month and that it remained at the end of the first year of life. The Eda gene affects the development of mandible and ectodermal structures.</dcterms:abstract>
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