Timeline

Aziz El Amraoui
Head of Structure
18 Jan 2024
publication

Clarin-2 gene supplementation durably preserves hearing in a model of progressive hearing loss.

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20 Nov 2023
event

PhD thesis defense: Characterization of Disease Mechanisms Underlying Clarin-Mediated Progressive Hearing Loss | Maureen Wentling

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19 May 2023
project

FFB USH1B project

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19 May 2023
project

NanoEar

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19 May 2023
publication

Frankenbacteriosis targeting interactions between pathogen and symbiont to control infection in the tick vector.

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05 Aug 2022
publication

Usher syndrome Recent advances in our understanding of genes and therapeutics

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28 Jun 2022
publication

Déficience auditive induite par le bruit : comment peut-elle être prévenue et traitée ?

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04 Apr 2022
publication

Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions

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01 Apr 2022
publication

The genetic and phenotypic landscapes of Usher syndrome: from disease mechanisms to a new classification

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01 Sep 2021
publication

Vieillissement de l’oreille interne : bases génétiques et conséquences à l’échelle cellulaire et moléculaire.

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01 Jun 2021
publication

A biallelic variant in CLRN2 causes non-syndromic hearing loss in humans

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25 May 2021
publication

Review of Genotype-Phenotype Correlations in Usher Syndrome

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01 Apr 2021
publication

Stem Cells and Gene Therapy in Progressive Hearing Loss: the State of the Art

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01 Jan 2021
publication

Progress in Gene Editing Tools and Their Potential for Correcting Mutations Underlying Hearing and Vision Loss

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01 Sep 2020
publication

Disease mechanisms and gene therapy for Usher syndrome

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21 Jul 2020
publication

Inner Ear Gene Therapies Take Off: Current Promises and Future Challenges

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01 Apr 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

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01 Sep 2019
publication

Clarin‐2 is essential for hearing by maintaining stereocilia integrity and function

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01 Aug 2018
publication

Clarin-1 gene transfer rescues auditory synaptopathy in model of Usher syndrome.

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12 Feb 2018
publication

Variants in CIB2 cause DFNB48 and not USH1J

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31 Jan 2018
publication

Cone degeneration is triggered by the absence of USH1 proteins but prevented by antioxidant treatments

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01 Dec 2017
publication

CIB2, defective in isolated deafness, is key for auditory hair cell mechanotransduction and survival

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19 Oct 2017
project

UsherModels

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18 Oct 2017
project

HearInNoise

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01 Aug 2017
publication

Conformational switch of harmonin, a submembrane scaffold protein of the hair cell mechanoelectrical transduction machinery

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05 Jun 2017
publication

Usher syndrome type 1–associated cadherins shape the photoreceptor outer segment

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20 Apr 2017
event

“A Spectrin-bound phosphatase bridges Crumbs-mediated polarity and Hippo-mediated growth” – Dr Graham Thomas

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21 Feb 2017
publication

Spectrin βV adaptive mutations and changes in subcellular location correlate with emergence of hair cell electromotility in mammalians.

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01 Dec 2016
publication

An innovative strategy for the molecular diagnosis of Usher syndrome identifies causal biallelic mutations in 93% of European patients

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20 Oct 2016
publication

Cadherins in the auditory sensory organ

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18 Aug 2016
publication

Novel gene function revealed by mouse mutagenesis screens for models of age-related disease

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03 Jun 2016
publication

Cadherins in the Auditory Sensory Organ

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18 Jan 2016
publication

Class III myosins shape the auditory hair bundles by limiting microvilli and stereocilia growth.

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19 Nov 2015
project

TherapUsher

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19 Nov 2015
project

RHU LIGHT4DEAF

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16 Jul 2015
team

Progressive Sensory Disorders, PathoPhysiology and Therapy

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24 Jun 2014
publication

An unusually powerful mode of low-frequency sound interference due to defective hair bundles of the auditory outer hair cells

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01 Mar 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

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01 Feb 2014
publication

Successful Gene Therapy in the RPGRIP1-deficient Dog: a Large Model of Cone-Rod Dystrophy.

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15 Sep 2013
publication

The giant spectrin βV couples the molecular motors to phototransduction and Usher syndrome type I proteins along their trafficking route

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01 Jan 2013
publication

Cadherin defects in inherited human diseases.

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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

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20 Oct 2012
publication

Hereditary hearing loss | Atteintes héréditaires de l’audition

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15 Oct 2012
publication

Localization of Usher 1 proteins to the photoreceptor calyceal processes, which are absent from mice.

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13 Sep 2012
publication

Pivotal role of M-DC8+ monocytes from viremic HIV-infected patients in TNF overproduction in response to microbial products.

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01 Feb 2012
publication

Usher syndrome (sensorineural deafness and retinitis pigmentosa): pathogenesis, molecular diagnosis and therapeutic approaches.

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01 Jan 2012
publication

The auditory hair cell ribbon synapse: from assembly to function.

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11 May 2011
publication

Complete exon sequencing of all known Usher syndrome genes greatly improves molecular diagnosis.

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01 Nov 2010
publication

[Stem cell therapy in the inner ear: recent achievements and prospects].

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01 Jan 2010
publication

Cadherins as targets for genetic diseases.

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01 Jun 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

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01 Feb 2009
publication

[The outer hair cell of the mammalian cochlea: an outstanding amplifier].

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20 Oct 2008
publication

Myosin VII

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15 Oct 2008
publication

αII-βV spectrin bridges the plasma membrane and cortical lattice in the lateral wall of the auditory outer hair cells

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15 Aug 2007
publication

Shroom2, a myosin-VIIa- and actin-binding protein, directly interacts with ZO-1 at tight junctions.

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15 Dec 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

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01 Nov 2005
publication

Vezatin, a protein associated to adherens junctions, is required for mouse blastocyst morphogenesis.

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15 Oct 2005
publication

Usher I syndrome: unravelling the mechanisms that underlie the cohesion of the growing hair bundle in inner ear sensory cells

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04 Jul 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

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01 Jul 2005
publication

PHR1, an integral membrane protein of the inner ear sensory cells, directly interacts with myosin 1c and myosin VIIa.

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01 Feb 2005
publication

Interactions in the network of Usher syndrome type 1 proteins

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01 Feb 2005
publication

Myosin XVa and whirlin, two deafness gene products required for hair bundle growth, are located at the stereocilia tips and interact directly

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15 Apr 2004
publication

Unconventional myosin VIIa and vezatin, two proteins crucial for Listeria entry into epithelial cells

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10 Nov 2003
publication

Rab27A and its effector MyRIP link secretory granules to F-actin and control their motion towards release sites

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01 Nov 2003
publication

Differential distribution of harmonin isoforms and their possible role in Usher-1 protein complexes in mammalian photoreceptor cells.

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01 Oct 2003
publication

Involvement of the Rab27 binding protein Slac2c/MyRIP in insulin exocytosis.

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01 Aug 2003
publication

Defects in whirlin, a PDZ domain molecule involved in stereocilia elongation, cause deafness in the whirler mouse and families with DFNB31

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01 Mar 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.

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16 Dec 2002
publication

Myosin VIIa, harmonin and cadherin 23, three Usher I gene products that cooperate to shape the sensory hair cell bundle.

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18 Apr 2002
publication

MyRIP, a novel Rab effector, enables myosin VIIa recruitment to retinal melanosomes.

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01 Aug 2001
publication

Spatiotemporal expression of otogelin in the developing and adult mouse inner ear

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15 Nov 2000
publication

Vezatin, a novel transmembrane protein, bridges myosin VIIA to the cadherin-catenins complex

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22 Sep 2000
publication

Unconventional Myosin VIIA Is a Novel A-kinase-anchoring Protein

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11 Apr 2000
publication

KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway.

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01 Feb 2000
publication

Targeted disruption of otog results in deafness and severe imbalance

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01 Apr 1999
publication

A mutation in OTOF, encoding otoferlin, a FER-1-like protein, causes DFNB9, a nonsyndromic form of deafness

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05 Feb 1999
publication

KCNQ4, a Novel Potassium Channel Expressed in Sensory Outer Hair Cells, Is Mutated in Dominant Deafness

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01 Dec 1998
publication

Eya1 expression in the developing ear and kidney: Towards the understanding of the pathogenesis of branchio-oto-renal (BOR) syndrome

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23 Dec 1997
publication

Otogelin: a glycoprotein specific to the acellular membranes of the inner ear

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20 Oct 1997
publication

Towards a molecular understanding of the pathophysiology of Usher syndrome

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15 Oct 1997
publication

Development and differentiation of pituitary cells

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01 Aug 1997
publication

Expression of myosin VIIA during mouse embryogenesis

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01 Mar 1997
publication

Cloning of the Genes Encoding Two Murine and Human Cochlear Unconventional Type I Myosins

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23 Dec 1996
publication

Comigration of tyrosine hydroxylase- and gonadotropin-releasing hormone-immunoreactive neurons in the nasal area of human embryos

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01 Aug 1996
publication

Human Usher 1B/mouse shaker-1: the retinal phenotype discrepancy explained by the presence/absence of myosin VIIA in the photoreceptor cells

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16 Apr 1996
publication

Human myosin VIIA responsible for the Usher 1B syndrome: a predicted membrane-associated motor protein expressed in developing sensory epithelia.

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01 May 1995
publication

Interactions between adenohypophyseal, hypothalamic and nasal presumptive territories during early neurulation process

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01 Dec 1993
publication

Experimental Evidence for the Early Commitment of the Presumptive Adenohypophysis

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01 Jun 1993
publication

Experimental evidence for an early commitment of gonadotropin-releasing hormone neurons, with special regard to their origin from the ectoderm of nasal cavity presumptive territory

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