Timeline

Aziz El Amraoui
Head of Structure
04 Jul 2021
publication

[Usher syndrome type I and the differentiation of inner ear sensory cells’ hair bundles].

Read more
01 Jun 2021
publication

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

Read more
25 May 2021
publication

Review of Genotype-Phenotype Correlations in Usher Syndrome.

Read more
01 Apr 2021
publication

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

Read more
01 Sep 2020
publication

Disease mechanisms and gene therapy for Usher syndrome.

Read more
21 Jul 2020
publication

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

Read more
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.

Read more
01 Sep 2019
publication

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

Read more
01 Aug 2018
publication

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

Read more
12 Feb 2018
publication

Variants in CIB2 cause DFNB48 and not USH1J

Read more
31 Jan 2018
publication

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

Read more
01 Dec 2017
publication

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

Read more
19 Oct 2017
project

UsherModels

Read more
18 Oct 2017
project

HearInNoise

Read more
10 Jul 2017
publication

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

Read more
05 Jun 2017
publication

Usher syndrome type 1-associated cadherins shape the photoreceptor outer segment.

Read more
20 Apr 2017
event

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

Read more
21 Feb 2017
publication

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

Read more
01 Dec 2016
publication

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

Read more
20 Oct 2016
publication

Cadherins in the auditory sensory organ

Read more
18 Aug 2016
publication

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

Read more
18 Jan 2016
publication

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

Read more
19 Nov 2015
project

TherapUsher

Read more
19 Nov 2015
project

RHU LIGHT4DEAF

Read more
16 Jul 2015
team

Progressive Sensory Disorders, PathoPhysiology and Therapy

Read more
24 Jun 2014
publication

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

Read more
01 Mar 2014
publication

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

Read more
01 Feb 2014
publication

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

Read more
23 May 2013
publication

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

Read more
01 Jan 2013
publication

Cadherin defects in inherited human diseases.

Read more
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

Read more
20 Oct 2012
publication

Hereditary hearing loss | Atteintes héréditaires de l’audition

Read more
15 Oct 2012
publication

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

Read more
01 Feb 2012
publication

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

Read more
01 Jan 2012
publication

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

Read more
11 May 2011
publication

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

Read more
01 Nov 2010
publication

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

Read more
01 Jan 2010
publication

Cadherins as targets for genetic diseases.

Read more
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

Read more
01 Feb 2009
publication

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

Read more
20 Oct 2008
publication

Myosin VII

Read more
16 Sep 2008
publication

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

Read more
31 Jul 2007
publication

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

Read more
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.

Read more
01 Nov 2005
publication

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

Read more
15 Oct 2005
publication

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

Read more
01 Jul 2005
publication

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

Read more
08 Dec 2004
publication

Interactions in the network of Usher syndrome type 1 proteins.

Read more
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.

Read more
15 Apr 2004
publication

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

Read more
10 Nov 2003
publication

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

Read more
01 Nov 2003
publication

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

Read more
01 Oct 2003
publication

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

Read more
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.

Read more
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.

Read more
16 Dec 2002
publication

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

Read more
18 Apr 2002
publication

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

Read more
01 Aug 2001
publication

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

Read more
15 Nov 2000
publication

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

Read more
22 Sep 2000
publication

Unconventional myosin VIIA is a novel A-kinase-anchoring protein

Read more
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

Read more
01 Feb 2000
publication

Targeted disruption of otog results in deafness and severe imbalance

Read more
01 Apr 1999
publication

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

Read more
05 Feb 1999
publication

KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafness

Read more
01 Dec 1998
publication

Eya1 expression in the developing ear and kidney: towards the understanding of the pathogenesis of Branchio-Oto-Renal (BOR) syndrome

Read more
23 Dec 1997
publication

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

Read more
20 Oct 1997
publication

Towards a molecular understanding of the pathophysiology of Usher syndrome

Read more
15 Oct 1997
publication

Development and differentiation of pituitary cells

Read more
01 Aug 1997
publication

Expression of myosin VIIA during mouse embryogenesis

Read more
01 Mar 1997
publication

Cloning of the genes encoding two murine and human cochlear unconventional type I myosins

Read more
23 Dec 1996
publication

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

Read more
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

Read more
16 Apr 1996
publication

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

Read more
01 May 1995
publication

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

Read more
01 Dec 1993
publication

Experimental evidence for the early commitment of the presumptive adenohypophysis

Read more
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

Read more