Over the last years, research on the inner ear and retinal sensory systems has become, in many cases, a blueprint for aspects pertinent to the nervous system and related neurodegenerative disorders. The senses of vision, balance, and hearing (Fig. 1) are essential for every significant activity of daily life, ranging from social interactions and mobility to an appreciation of music, art, and nature. Consequently, impairment of these sensory modalities has a profound negative impact in the quality of life of the affected individuals, which range from communication constraints and lower access to entertainment and working opportunities. In turn, this can lead to social isolation and depression 1-5. From comprehensive empirical data, the World Health Organization estimates that around 285 million people currently display severe vision impairments worldwide (see http://www.who.int/). Also, 460 million people — representing 5% of the world’s population — have a disabling hearing impairment (with or without balance deficit). This number is expected to increase to more than one billion people by 2050 (see http://www.who.int/). Altogether, these sensory deficits have a dramatic economic impact on healthcare systems and society as a whole.
Over the years, we showed that, even though processing different sensory signals — mechanical stimulation in hair cells and light inputs in photoreceptors—, the inner ear and retinal sensory cells both display membrane- and cytoskeleton-based structures that are key to hearing, balance and vision functioning (Fig. 1). The research interests of our lab span the inner ear and the retinal sensory systems, from animal model to human patients, from normal to abnormal function, from organ architecture to behavior, and from the molecular and genetic bases of hearing, balance, and/or vision deficits to innovative treatments solutions.
The Progressive Sensory Disorders (PSD) team is designed to bring together all necessary knowledge, ranging from tissue engineering & animal modeling, molecular & cellular biology, “Omics”, biochemistry and computational networks analyses, imaging, and physiology techniques. Our research goals are:
(1) understand and elucidate the way the sound-receptive and the light-sensitive sensory cells and neurons are tuned to the specific needs of corresponding sensory modalities, in normal and disease conditions.
(2) identify sensitive/specific disease markers and potential external & environmental risk factors (light- or noise-exposure, oxidative stress, age, …), and provide better predictions of disease progression & severity.
(3) develop and evaluate efficacy of innovative gene replacement therapies & gene editing mutation repair strategies to prevent, protect against, and/or correct Usher sense deficits, a prelude to future clinical trials.
Our projects call for multidisciplinary and integrative approaches, taking advantage of multiple internal, national and international collaborations. The lab is staffed by a dynamic team, with three permanent staff members, 2 Assistant Professors (Sedigheh Delmaghani and Sandrine Vitry) and a senior Technician (Sylvie Nouaille), working along with post-doctoral fellows, PhD, undergraduate and graduate students.
Recent team achievements, brief biographies of current lab members, list of former members, images of inner ear tissue, publications involving team members are indicated below.
If you want to join the team or have questions about our work, please feel free to contact us
(aziz.el-amraoui@pasteur.fr or PSD-PI@pasteur.fr).
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Figure 1: Similarities between the sensory organs of vision, hearing & balance. In absence of a cure, untreated decline of vision, balance and/or hearing have huge economic & societal impact worldwide. Whatever the cause – i.e., genetic, environment (noise, ototoxic drugs, light-phototoxicity, …), or aging – loss of hearing, balance, and/or vision is often linked to irreversible loss of the specialized sensory cells in the inner ear (hair cells) and the eye (photoreceptors), and associated neurons. We study disorders targeting these sensory cells as model systems to tackle neurodegenerative disorders from disease mechanisms to therapeutic options.
Summaries of some last achievements (2015-2020) : https://research.pasteur.fr/wp-content/uploads/2015/07/research_pasteur-enprogressive-sensory-disorders-pathophysiology-and-therapyfrdeficits-sensoriels-progressifs-pathophysiologie-et-therapie-psd-lab-achievements-2015-2020.pdf
