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Understanding the RNA gain of function diseases: Nicolas Charlet-Berguerand’s project supported by the ERC

Sept. 10, 2012

The European research council (ERC) has just awarded Nicolas Charlet-Berguerand with a Starting grant to support his research on RNA gain of function diseases.


The DNA of our genes is first transcribed in RNA before it is itself translated into proteins. Yet part of transcribed RNA do not encode any protein. In some cases, these “non-coding” RNA may be stored and with varying degrees of consequence for the organism, affect cell functioning. “RNA gain of function” diseases is a class of genetic human diseases that includes myotonic dystrophies of type 1 and 2 (congenital or adult form), fragile X-associated tremor/ataxia syndrome, amyotrophic lateral sclerosis associated with a frontotemporal dementia and spinocerebellar ataxias 10, 31 and 36.

Nicolas Charlet-Berguerand and his team are focusing on the identification of the molecular mechanisms of these rare diseases. At the head of his own group since 2006, this young team leader is now a laureate of the prestigious ERC starting grant, a highly competitive award offered by the European research council to talented young, European researchers. The budget of 1.5 million Euros will enable Nicolas to conduct particularly innovative experiments over the 5 years of the project.

At the IGBMC, 5 junior team leaders are already laureates of this grant (Hélène Puccio, Robert Schneider, Bruno Klaholz, Maria-Elena Torres-Padillla and Romeo Ricci).

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