Timeline

Aziz El Amraoui

Head of Structure

09 Jul 2018

publication

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

12 Feb 2018

publication

Variants in CIB2 cause DFNB48 and not USH1J

31 Jan 2018

publication

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

01 Dec 2017

publication

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

19 Oct 2017

project

UsherModels

18 Oct 2017

project

HearInNoise

11 May 2017

publication

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

20 Apr 2017

event

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

08 Feb 2017

publication

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

20 Oct 2016

publication

Cadherins in the auditory sensory organ

18 Aug 2016

publication

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

27 Jul 2016

publication

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

11 Jan 2016

publication

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

19 Nov 2015

project

TherapUsher

19 Nov 2015

project

RHU LIGHT4DEAF

16 Jul 2015

team

Progressive Sensory Disorders, PathoPhysiology and Therapy

19 Oct 2014

publication

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

16 Sep 2014

team

Genetics & Physiology of Hearing

11 Jun 2014

publication

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

20 Mar 2014

publication

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

23 May 2013

publication

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

01 Jan 2013

publication

Cadherin defects in inherited human diseases

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

20 Oct 2012

publication

Hereditary hearing loss | Atteintes héréditaires de l’audition

01 Feb 2012

publication

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

01 Jan 2012

publication

The auditory hair cell ribbon synapse: from assembly to function

01 Nov 2010

publication

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

01 Jan 2010

publication

Cadherins as targets for genetic diseases

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

01 Feb 2009

publication

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

20 Oct 2008

publication

Myosin VII

16 Sep 2008

publication

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

31 Jul 2007

publication

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

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

15 Oct 2005

publication

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

01 Aug 2005

publication

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

01 Jul 2005

publication

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

08 Dec 2004

publication

Interactions in the network of Usher syndrome type 1 proteins

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

15 Apr 2004

publication

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

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

18 Apr 2002

publication

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

01 Aug 2001

publication

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

15 Nov 2000

publication

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

22 Sep 2000

publication

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

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

01 Feb 2000

publication

Targeted disruption of otog results in deafness and severe imbalance

01 Apr 1999

publication

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

05 Feb 1999

publication

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

01 Dec 1998

publication

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

23 Dec 1997

publication

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

20 Oct 1997

publication

Towards a molecular understanding of the pathophysiology of Usher syndrome

15 Oct 1997

publication

Development and differentiation of pituitary cells

01 Aug 1997

publication

Expression of myosin VIIA during mouse embryogenesis

01 Mar 1997

publication

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

23 Dec 1996

publication

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

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

16 Apr 1996

publication

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

01 May 1995

publication

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

01 Dec 1993

publication

Experimental evidence for the early commitment of the presumptive adenohypophysis

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

A little guide for advanced search:

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

Variants in CIB2 cause DFNB48 and not USH1J

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

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

UsherModels

HearInNoise

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

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

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

Cadherins in the auditory sensory organ

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

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

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

TherapUsher

RHU LIGHT4DEAF

Progressive Sensory Disorders, PathoPhysiology and Therapy

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

Genetics & Physiology of Hearing

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

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

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

Cadherin defects in inherited human diseases

Gene and cell therapies to listen to the inner ear | Les thérapies génique et cellulaire à l’écoute de l’oreille interne

Hereditary hearing loss | Atteintes héréditaires de l’audition

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

The auditory hair cell ribbon synapse: from assembly to function

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

Cadherins as targets for genetic diseases

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

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

Myosin VII

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

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

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

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

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

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

Interactions in the network of Usher syndrome type 1 proteins

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

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

Usher syndrome type I G (USH1G) is caused by mutations in the gene encoding SANS, a protein that associates with the USH1C protein, harmonin

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

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

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

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

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

Targeted disruption of otog results in deafness and severe imbalance

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

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

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

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

Towards a molecular understanding of the pathophysiology of Usher syndrome

Development and differentiation of pituitary cells

Expression of myosin VIIA during mouse embryogenesis

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

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

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

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

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

Experimental evidence for the early commitment of the presumptive adenohypophysis

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