The Penfield Lecture was inaugurated in 1985 to honour Wilder Penfield, pioneering neurosurgeon and founder of The Neuro.
Speaker: Christine Petit, MD, PhD
Professor, College de France, Chair of Genetics and Cellular Physiology
Professor “Classe exceptionnelle”, Institut Pasteur, laboratory of Genetics and Physiology of Hearing
Head of Inserm UMRS1120, Pierre et Marie Curie Université (UPMC), Paris, FRANCE
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The molecular mechanisms underlying the development and functioning of the auditory system remained virtually unknown until genetic dissection emerged as a way of getting to them. In this lecture, I will show why and how the genes responsible for deafness in humans made it possible to bring the auditory system into the molecular era. I will focus, in particular, on the biology of the auditory sensory cells. I will show how the molecular mechanisms underlying the essential functions and properties of these cells —auditory sensory transduction, generation of sound distortions and the temporal precision of their activity — were elucidated and how the role of previously neglected structures was discovered. Our recent results, notably, demonstrate that some of these deafness forms are not exclusively peripheral in origin, indicating that the proteins encoded by some deafness genes are also involved in auditory cortex development. In parallel, this work also shed light on the pathogenesis of a very large number of forms of deafness, most of which were hereditary. Based on these data, active research with the aim of curing hearing impairment is currently underway. We will see here the preliminary results obtained in animals and the challenges that lie ahead.
Christine PETIT is a geneticist and neuroscientist. She studied medicine simultaneously with genetics and biochemistry at the Faculty of Sciences, Paris XI University. She became a research scientist at Institut Pasteur in the mid-1970s, Professor at Collège de France, where she holds the Genetics and Cell Physiology chair, in 2002, and then founding Director of the Hearing Institute, an Institut Pasteur center affiliated to INSERM, in Paris, in 2019.
Having realized that the auditory system had escaped molecular characterization due to the paucity of cells in the cochlea, she suggested a genetic dissection of sound processing in this sensory organ, based on the genes responsible for sensorineural deafness in humans. She identified a number of these genes by studying geographically isolated deaf populations. She then deciphered molecular mechanisms involved in cochlear ion and redox homeostasis, and neurotransmitter exocytosis at the hair-cell synapse. In parallel, she elucidated the pathogenic processes underlying numerous human hereditary forms of deafness. In recent years, she and her colleagues have been exploring gene therapy for the treatment of hereditary deafness, providing proof of concept for several deafness forms.
Christine PETIT is a member of the French Academy of Sciences and a foreign member of the National Academy of Sciences (USA). She has received many awards, including the L’Oréal-UNESCO "For Women in Science" Award in 2004, the Louis-Jeantet Prize for Medicine in 2006, the INSERM Grand Prix de la Recherche Médicale in 2007, The Brain Prize from the Grete Lundbeck Foundation in 2012, and the Kavli Prize, which she shared with Jim Hudspeth and Robert Fettiplace, in 2018.