WOLFF Nicolas

Study of protein complexs associated with Usher 2 Syndrome.

Project status: active

In humans, hearing is based on the transformation of sound vibrations into electrical signals. This mechanism takes place in the cells located in the cochlea, which is the inner ear’s sensory organ. These cells are covered in small hairs, hence the name “hair cells”. They are essential and are organized in tufts in which the transformation of the signal involves a network of proteins whose organization is still ill-defined today.

➜ Learn more about the cochlea

The Usher Syndrome Type II is the leading genetic cause of hearing loss associated with vision loss in patients. Four proteins expressed in a complex are associated with this disease : ADGRV1, usherin, whirlin and PDZD7. As the molecular organization of this complex is unknown, Prof. Nicolas Wolff intends to elucidate its composition, structure and function in collaboration with Prof. Dorit Hanein, Jean-Philippe Pin and Niels Volkmann.

The results will allow for a better understanding of hearing loss associated with Usher Type II mutations and will be essential in the development of new specific therapeutic approaches.

Professor Nicolas Wolff
Investigator-Team leader
Institut Pasteur, France

Related scientific publication(s):

Baptiste Colcombet-Cazenave, Florence Cordier, Yanlei Zhu, Guillaume Bouvier, Eleni Litsardaki, Louise Laserre , Marie S Prevost, Bertrand Raynal, Célia Caillet-Saguy , Nicolas Wolff. Deciphering the Molecular Interaction Between the Adhesion G Protein-Coupled Receptor ADGRV1 and its PDZ-Containing Regulator PDZD7.Front Mol Biosci. 2022 Jun 28;9:923740. doi: 10.3389/fmolb.2022.923740. eCollection 2022.