In May 2019, Sensorion signed a partnership framework agreement with Institut Pasteur in the field of genetic disorders of the inner ear. This agreement includes an option to obtain exclusive licenses at predefined terms to develop and market gene therapy drug candidates.
The Genetics and Physiology of the Hearing Unit of Institut Pasteur, led by Professor Christine Petit, has developed world-class expertise over the last 25 years in the molecular physiology and pathophysiology of the hearing system.


The first two programs initiated under the collaboration include deafness due to Otoferlin deficiency as well as Usher Syndrome Type 1.


Otoferlin deficiency (DFNB9 deafness)
• Otoferlin deficiency is a rare genetic disorder caused by mutations of the OTOF gene. It’s one of the more frequent forms of congenital deafness. Affected babies typically have a bilateral severe to profound hearing loss.
• The Otoferlin protein is the major calcium sensor for synaptic exocytosis in cochlear sensory cells (Inner Hair Cells, IHCs). Thus, it’s essential for transmitting sound information at the auditory sensory cell synapses.

 

Usher Syndrome Type 1
• Usher Syndrome Type 1 is the most severe expression of Usher Syndrome. It’s caused by various mutations on USH1C, MYO7A, CDH23, PCDH15, USH1G and CIB2 genes. It is characterized by congenital profound deafness and balance defects. Affected patients subsequently undergo sight loss during childhood, leading to blindness.
• Inner hair cells lacking the scaffold protein called Sans, encoded by the USH1G gene, cannot develop, maintain and have a functional hair bundle.

 

1. Akil et al. - Proceedings of the National Academy of Sciences Mar 2019, 116 (10) 4496-4501

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