معرفی
Aziz El Amraoui is a leading researcher and Head of the Progressive Sensory Disorders (PSD) research unit at the Institut de l’Audition, part of the Institut Pasteur in Paris, France. His work focuses on understanding and treating progressive sensory impairments, particularly those affecting hearing, balance, and vision, with a special emphasis on Usher syndrome.
His research spans molecular genetics, advanced imaging, electrophysiology, and gene therapy. He investigates disease mechanisms, environmental modulators (e.g., aging, noise), and develops innovative treatments using AAV vectors, mini-genes, and CRISPR-based technologies. His team utilizes murine and porcine models to study disease progression and therapeutic efficacy across species.
Recent key discoveries include identifying CLRN2 as a novel deafness gene (DFNB117), developing effective gene therapy for progressive hearing loss with a narrow therapeutic window, and resolving the Usher vision loss paradox by identifying the role of calyceal processes in human photoreceptors—structures absent in mice.
His publication record shows consistent output in high-impact journals such as Molecular Therapy, EMBO Molecular Medicine, and PNAS, with work spanning genetics, neurobiology, and translational medicine. His research has direct implications for early diagnosis, personalized treatment, and the development of scalable gene therapies for sensory disorders.
- Progressive Hearing Loss and CLRN2 gene therapy
- Usher Syndrome pathogenesis and treatment
- Evolution of sensory systems (e.g., spectrin βV)
- Noise- and age-induced hearing loss
- Vestibular deficits in deafness
- Gene editing and delivery systems
He leads multiple funded projects including FFB USH1B, UsherModels, HearInNoise, and TherapUsher, and actively mentors PhD and Master’s students. His collaborative network includes institutions in the UK, Germany, and beyond.
Aziz El Amraoui’s work is central to advancing precision medicine for sensory disorders, bridging fundamental science with therapeutic innovation to improve the lives of millions affected by hearing and vision loss.
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