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Jean-Louis Mandel is one of the 4 laureates in neuroscience of the Kavli 2022 Prize

Prix et distinctions Actualités |

Jean-Louis Mandel, professor emeritus at the University of Strasbourg and honorary professor at the Collège de France, is one of the four winners of the Kavli Prize in the field of neuroscience, the Norwegian Academy of Science and Letters announced on Wednesday 1 June 2022. This prize is awarded to scientists who have made fundamental discoveries in their field.

4 scientists awarded in the field of neuroscience

In 2022, the Norwegian Academy of Science and Letters is awarding the Kavli Prize to four scientists in the field of neuroscience:

  • Harry T. Orr (USA), University of Minnesota Medical School
  • Christopher A. Walsh (USA), Harvard Medical School
  • Huda Y. Zoghbi (Lebanon / USA), Baylor College of Medicine
  • Jean-Louis Mandel (France), University of Strasbourg

This prize is accompanied by a one million dollar award, divided between the winners, to reward their research. This year, the Kavli Prize is also awarded to seven other scientists in the fields of nanoscience and astrophysics.

 

Jean-Louis Mandel, awarded for his work on a mechanism common to more than 50 genetic disorders

Former University Professor - hospital practitioner (PU-PH) and former director of the IGBMC, Jean-Louis Mandel discovered the mechanism of unstable repeat expansions. Working on an unusual mutation in an X chromosome gene causing Fragile X syndrome, he showed that the mutation was in fact an unstable expansion of a chain of triple-letter repeats in the FMR1 gene that disrupted the transcription step of the gene and the production of a protein essential for proper brain function, the FMRP protein.

This still unknown mechanism was later shown to be responsible for Huntington's disease and about fifty other genetic neurological diseases. This discovery has also led to the development of therapeutic treatments, such as the one developed by Hervé Moine and Nicolas Charlet-Berguerand for the neurodegenerative syndrome FXTAS.

Jean-Louis Mandel continues to work with the team led by Amélie Piton on the identification of genes involved in other genetic forms of intellectual disability and autism. In 2016, the scientist also launched, in collaboration with USIAS, an international participatory research platform to study the clinical manifestations of these genetic diseases: the GenIDA project