Timeline

Aziz El Amraoui
Head of Structure
09 Jul 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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11 May 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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08 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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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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27 Jul 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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11 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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19 Oct 2014
publication

Successful gene therapy in the RPGRIP1-deficient dog: a large model of cone-rod dystrophy

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16 Sep 2014
team

Genetics & Physiology of Hearing

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11 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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20 Mar 2014
publication

The retinal phenotype of Usher syndrome: pathophysiological insights from animal models

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23 May 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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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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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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16 Sep 2008
publication

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

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31 Jul 2007
publication

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

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21 Nov 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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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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01 Aug 2005
publication

[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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08 Dec 2004
publication

Interactions in the network of Usher syndrome type 1 proteins

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08 Dec 2004
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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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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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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