Ligne de temps
Aziz El Amraoui
Responsable de Structure
18 janv. 2024
publication
Clarin-2 gene supplementation durably preserves hearing in a model of progressive hearing loss.
Lire plus20 nov. 2023
event
PhD thesis defense: Characterization of Disease Mechanisms Underlying Clarin-Mediated Progressive Hearing Loss | Maureen Wentling
Lire plus19 mai 2023
19 mai 2023
19 mai 2023
publication
Frankenbacteriosis targeting interactions between pathogen and symbiont to control infection in the tick vector.
Lire plus05 août 2022
28 juin 2022
publication
Déficience auditive induite par le bruit : comment peut-elle être prévenue et traitée ?
Lire plus04 avr. 2022
publication
Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions
Lire plus01 avr. 2022
publication
The genetic and phenotypic landscapes of Usher syndrome: from disease mechanisms to a new classification
Lire plus01 sept. 2021
publication
Vieillissement de l’oreille interne : bases génétiques et conséquences à l’échelle cellulaire et moléculaire.
Lire plus01 juin 2021
25 mai 2021
01 avr. 2021
01 janv. 2021
publication
Progress in Gene Editing Tools and Their Potential for Correcting Mutations Underlying Hearing and Vision Loss
Lire plus01 sept. 2020
21 juil. 2020
01 avr. 2020
publication
Identification of a CDH12 potential candidate genetic variant for an autosomal dominant form of transgrediens and progrediens palmoplantar keratoderma in a Tunisian family
Lire plus01 sept. 2019
publication
Clarin‐2 is essential for hearing by maintaining stereocilia integrity and function
Lire plus01 août 2018
publication
Clarin-1 gene transfer rescues auditory synaptopathy in model of Usher syndrome.
Lire plus12 févr. 2018
31 janv. 2018
publication
Cone degeneration is triggered by the absence of USH1 proteins but prevented by antioxidant treatments
Lire plus01 déc. 2017
publication
CIB2, defective in isolated deafness, is key for auditory hair cell mechanotransduction and survival
Lire plus19 oct. 2017
18 oct. 2017
01 août 2017
publication
Conformational switch of harmonin, a submembrane scaffold protein of the hair cell mechanoelectrical transduction machinery
Lire plus05 juin 2017
publication
Usher syndrome type 1–associated cadherins shape the photoreceptor outer segment
Lire plus20 avr. 2017
event
“A Spectrin-bound phosphatase bridges Crumbs-mediated polarity and Hippo-mediated growth” – Dr Graham Thomas
Lire plus21 févr. 2017
publication
Spectrin βV adaptive mutations and changes in subcellular location correlate with emergence of hair cell electromotility in mammalians.
Lire plus01 déc. 2016
publication
An innovative strategy for the molecular diagnosis of Usher syndrome identifies causal biallelic mutations in 93% of European patients
Lire plus20 oct. 2016
18 août 2016
publication
Novel gene function revealed by mouse mutagenesis screens for models of age-related disease
Lire plus03 juin 2016
18 janv. 2016
publication
Class III myosins shape the auditory hair bundles by limiting microvilli and stereocilia growth.
Lire plus19 nov. 2015
19 nov. 2015
16 juil. 2015
24 juin 2014
publication
An unusually powerful mode of low-frequency sound interference due to defective hair bundles of the auditory outer hair cells
Lire plus01 mars 2014
publication
The retinal phenotype of Usher syndrome: Pathophysiological insights from animal models Atteinte rétinienne dans le syndrome de Usher : contribution des modèles animaux à la physiopathologie
Lire plus01 févr. 2014
publication
Successful Gene Therapy in the RPGRIP1-deficient Dog: a Large Model of Cone-Rod Dystrophy.
Lire plus15 sept. 2013
publication
The giant spectrin βV couples the molecular motors to phototransduction and Usher syndrome type I proteins along their trafficking route
Lire plus01 janv. 2013
20 oct. 2012
publication
Gene and cell therapies to listen to the inner ear | Les thérapies génique et cellulaire à l’écoute de l’oreille interne
Lire plus20 oct. 2012
15 oct. 2012
publication
Localization of Usher 1 proteins to the photoreceptor calyceal processes, which are absent from mice.
Lire plus13 sept. 2012
publication
Pivotal role of M-DC8+ monocytes from viremic HIV-infected patients in TNF overproduction in response to microbial products.
Lire plus01 févr. 2012
publication
Usher syndrome (sensorineural deafness and retinitis pigmentosa): pathogenesis, molecular diagnosis and therapeutic approaches.
Lire plus01 janv. 2012
11 mai 2011
publication
Complete exon sequencing of all known Usher syndrome genes greatly improves molecular diagnosis.
Lire plus01 nov. 2010
01 janv. 2010
01 juin 2009
publication
The outer hair cell of the mammalian cochlea: An outstanding amplifier | La cellule ciliée externe de la cochlée des mammifères: Un amplificateur aux propriétés exceptionnelles
Lire plus01 févr. 2009
20 oct. 2008
15 oct. 2008
publication
αII-βV spectrin bridges the plasma membrane and cortical lattice in the lateral wall of the auditory outer hair cells
Lire plus15 août 2007
publication
Shroom2, a myosin-VIIa- and actin-binding protein, directly interacts with ZO-1 at tight junctions.
Lire plus15 déc. 2005
publication
Usherin, the defective protein in Usher syndrome type IIA, is likely to be a component of interstereocilia ankle links in the inner ear sensory cells
Lire plus01 nov. 2005
publication
Vezatin, a protein associated to adherens junctions, is required for mouse blastocyst morphogenesis.
Lire plus15 oct. 2005
publication
Usher I syndrome: unravelling the mechanisms that underlie the cohesion of the growing hair bundle in inner ear sensory cells
Lire plus04 juil. 2005
publication
Syndrome de Usher de type 1 et développement de la touffe ciliaire des cellules sensorielles de l’oreille interne Usher syndrome type I and the differentiation of inner ear sensory cells’ hair bundles
Lire plus01 juil. 2005
publication
PHR1, an integral membrane protein of the inner ear sensory cells, directly interacts with myosin 1c and myosin VIIa.
Lire plus01 févr. 2005
01 févr. 2005
publication
Myosin XVa and whirlin, two deafness gene products required for hair bundle growth, are located at the stereocilia tips and interact directly
Lire plus15 avr. 2004
publication
Unconventional myosin VIIa and vezatin, two proteins crucial for Listeria entry into epithelial cells
Lire plus10 nov. 2003
publication
Rab27A and its effector MyRIP link secretory granules to F-actin and control their motion towards release sites
Lire plus01 nov. 2003
publication
Differential distribution of harmonin isoforms and their possible role in Usher-1 protein complexes in mammalian photoreceptor cells.
Lire plus01 oct. 2003
01 août 2003
publication
Defects in whirlin, a PDZ domain molecule involved in stereocilia elongation, cause deafness in the whirler mouse and families with DFNB31
Lire plus01 mars 2003
publication
Usher syndrome type I G (USH1G) is caused by mutations in the gene encoding SANS, a protein that associates with the USH1C protein, harmonin.
Lire plus16 déc. 2002
publication
Myosin VIIa, harmonin and cadherin 23, three Usher I gene products that cooperate to shape the sensory hair cell bundle.
Lire plus18 avr. 2002
publication
MyRIP, a novel Rab effector, enables myosin VIIa recruitment to retinal melanosomes.
Lire plus01 août 2001
publication
Spatiotemporal expression of otogelin in the developing and adult mouse inner ear
Lire plus15 nov. 2000
publication
Vezatin, a novel transmembrane protein, bridges myosin VIIA to the cadherin-catenins complex
Lire plus22 sept. 2000
11 avr. 2000
publication
KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway.
Lire plus01 févr. 2000
01 avr. 1999
publication
A mutation in OTOF, encoding otoferlin, a FER-1-like protein, causes DFNB9, a nonsyndromic form of deafness
Lire plus05 févr. 1999
publication
KCNQ4, a Novel Potassium Channel Expressed in Sensory Outer Hair Cells, Is Mutated in Dominant Deafness
Lire plus01 déc. 1998
publication
Eya1 expression in the developing ear and kidney: Towards the understanding of the pathogenesis of branchio-oto-renal (BOR) syndrome
Lire plus23 déc. 1997
20 oct. 1997
15 oct. 1997
01 août 1997
01 mars 1997
publication
Cloning of the Genes Encoding Two Murine and Human Cochlear Unconventional Type I Myosins
Lire plus23 déc. 1996
publication
Comigration of tyrosine hydroxylase- and gonadotropin-releasing hormone-immunoreactive neurons in the nasal area of human embryos
Lire plus01 août 1996
publication
Human Usher 1B/mouse shaker-1: the retinal phenotype discrepancy explained by the presence/absence of myosin VIIA in the photoreceptor cells
Lire plus16 avr. 1996
publication
Human myosin VIIA responsible for the Usher 1B syndrome: a predicted membrane-associated motor protein expressed in developing sensory epithelia.
Lire plus01 mai 1995
publication
Interactions between adenohypophyseal, hypothalamic and nasal presumptive territories during early neurulation process
Lire plus01 déc. 1993
publication
Experimental Evidence for the Early Commitment of the Presumptive Adenohypophysis
Lire plus01 juin 1993
publication