50 research outputs found

    From Individual to Collective Pinning: Effect of Long-range Elastic Interactions

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    We study the effect of long-range elastic interactions in the dynamical behavior of an elastic chain driven quasi-statically in a quenched random pinning potential and in the strong pinning limit. This is a generic situation occuring in solid friction, crack propagation, wetting front motion, ... Tuning the exponent of the algebraic decay of the elastic interaction with the distance is shown to give rise to three regimes: a Mean-Field (MF) regime, a Laplacian (L) regime and an intermediate regime where the critical exponents interpolate continuously between the MF and L limit cases. The effect of the driving mode on the avalanche statistics is also analyzed.Comment: 28 pages in RevTex, 17 figure

    A Novel Mutation Involving the Initiation Codon of FGF3 in a Family Described with Complete Inner Ear Agenesis, Microtia and Major Microdontia (LAMM Syndrome)

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    LAMM syndrome (OMIM #610706) is a rare autosomal recessive syndrome characterized by the association of Michel aplasia, microdontia and malformation of the external ear. Different mutations in FGF3 gene were reported in several families presenting with this syndrome. Clinical features and genetic results observed in a family with LAMM syndrome are reported. The diagnosis of isolated Michel aplasia was initially made in this family composed of two affected children. Microtia and microdontia was recently evidenced in both patients suggesting the diagnosis of LAMM syndrome. New auditory and orodental iconography was performed permitting to describe the patients’ phenotype in depth and to report rare findings of LAMM syndrome. The sequencing of FGF3 gene identified a novel missense mutation (c.2T>G), substituting the first initiator methionine in arginine, in the fibroblast growth factor 3 (FGF3) at the homozygous state in both patients. LAMM syndrome was confirmed and appropriate genetic counseling performed

    Nephrocalcinosis (enamel renal syndrome) caused by autosomal recessive FAM20A mutations

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    Calcium homeostasis requires regulated cellular and interstitial systems interacting to modulate the activity and movement of this ion. Disruption of these systems in the kidney results in nephrocalcinosis and nephrolithiasis, important medical problems whose pathogenesis is incompletely understood

    Variation de la morphologie faciale au sein de la superfamille Hominoidea (Mammalia, Primates)

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    STRASBOURG-Medecine (674822101) / SudocSudocFranceF

    Variabilité de la morphologie mandibulaire au sein des Hominoïdea actuels

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    LILLE2-UFR Odontologie (593502202) / SudocREIMS-BU Santé (514542104) / SudocSTRASBOURG-Medecine (674822101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

    Dositométrie en odontologie (l'expérience du service de consultations et de traitements dentaires des hôpitaux universitaires de Strasbourg)

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    STRASBOURG-Medecine (674822101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

    Etude des dysmorphoses et de la croissance de la mandibule chez un modèle murin de la dysplasie ectodermique hypohidrotique

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    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 -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.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 ( -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.STRASBOURG-Bib.electronique 063 (674829902) / SudocSudocFranceF

    Variations de taille et de forme du foramen magnum (étude qualitative et quantitative au sein d'une population humaine d'origine rhénane)

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    REIMS-BU Santé (514542104) / SudocSTRASBOURG-Medecine (674822101) / SudocLILLE2-UFR Odontologie (593502202) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

    Le dimorphisme sexuel dentaire (une revue de la littérature)

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    REIMS-BU Santé (514542104) / SudocSTRASBOURG-Medecine (674822101) / SudocPARIS-BIUM (751062103) / SudocLILLE2-UFR Odontologie (593502202) / SudocSudocFranceF
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