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Nicolas CHARLET-BERGUERAND.


 

Nicolas CHARLET-BERGUERAND.               

 

                                                           IGBMC,

French, Age 48 (Born 1973),                                                                            1 Rue Laurent Fries

Assistant Professor (INSERM DR2)                                                                  67400 ILLKIRCH, France.

ResearcherID: I-6727-2016                                                                              Phone: (+33) 3 88 65 33 09

ORCID ID: orcid.org/0000-0002-4423-4920                                                      Email: ncharlet@igbmc.fr

 

 

MAIN RESEARCH AREA

 

My researches are mainly focused on “non-coding” genome and human diseases, first in cancer (PhD, University of Paris), then in genetic diseases (post-doctoral training, Baylor college of Medecine). I started my independent group on RNA diseases at the IGBMC (Strasbourg, France) in 2007. Since, we are studying how expanded repeats located in the “non-coding” parts of the genome lead to human genetic diseases. We are notably interested in Myotonic Dystrophy (DM) caused by expanded CTG repeats located in the 3’UTR of the DMPK gene, Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS) due to expanded CGG repeats in the 5’UTR of the FMR1 gene and Amyotrophic Lateral Sclerosis and Frontotemporal Dementia (ALS-FTD) due to GGGGCC expansion in the first intron of the C9ORF72 gene. These expanded repeats are pathogenic by diverse mechanisms, such as non-canonical translation and/ or sequestration of specific RNA-binding proteins. Our goals are (i) to elucidate the molecular causes of these diseases, (ii) to establish relevant cellular and rodent (mouse and rat) models of these diseases and (iii) to identify drugs able to correct pathogenicity in these models.

 

 

EDUCATION & EXPERIENCE:

 

Since 2007      TEAM LEADER - IGBMC, STRASBOURG, FRANCE.

                        « Understanding the molecular causes of RNA diseases. »

INSTITUT DE GÉNÉTIQUE ET DE BIOLOGIE MOLÉCULAIRE ET CELLULAIRE,

CNRS UMR7104, INSERM U1258, Dept. of Translational Medicine, 67404 Illkirch, France.

 

2004 - 06          POST-DOCTORAL POSITION - IGBMC, STRASBOURG, FRANCE.

« Regulation of the transcriptionnal bypass of DNA oxidative lesions. »

INSTITUT DE GÉNÉTIQUE ET DE BIOLOGIE MOLÉCULAIRE ET CELLULAIRE,

CNRS UMR7104, INSERM U1258, Dept. of Functional Genomics, 67404 Illkirch, France.

                        Supervisor: Prof. Jean-Marc EGLY.

 

1999 - 03          SCIENTIFIC COLLABORATION (MILITARY DUTY) – BCM, HOUSTON, USA.

« Alternative splicing alteration in myotonic dystrophic patients.»

                        Baylor College OF Medicine, Dept. of Pathology, 77030 Houston, Texas, USA.

                        Supervisor: Prof. Thomas COOPER.

 

1996 - 03          PHD - UNIVERSITY OF PARIS VII, FRANCE.

                        Graduation: Excellent, with the felicitation of the Jury.

« Regulation of RET and GFRa1 alternative spling in pheochromocytomas tumors. »

                        Centre de Génétique Moléculaire, CNRS UPR 2167, 91190 Gif-sur-Yvette, France.

                        Supervisor: Dr. Claude THERMES.

 

1995 - 96          MASTER OF SCIENCE (BIOLOGY OF AGING) - UNIVERSITY OF PARIS VII, FRANCE.

Graduation: Very Good.

 

 

AWARDS, POSITIONS & FELLOWSHIPS:

 

2018                 Award of scientific excellence. INSERM prime d'excellence scientifique.

2012                 European Research Council Starting grant.

2012                 INSERM DR2. Group leader permanent position.

2011                 Award of scientific excellence. INSERM prime d'excellence scientifique.

2006                 INSERM CR1. Scientist permanent position.

2006                 Award Georges Deflandre. Foundation de France.

2004 06         ARC post-doctoral fellowship. Foundation against cancer.

1995 – 99         EMRT PhD fellowship. French Ministry of Research and Education.


Journal articles3 documents

  • Fernande Freyermuth, Frédérique Rau, Yosuke Kokunai, Thomas Linke, Chantal Sellier, et al.. Splicing misregulation of SCN5A contributes to cardiac-conduction delay and heart arrhythmia in myotonic dystrophy. Nature Communications, Nature Publishing Group, 2016, 7, pp.11067. ⟨10.1038/ncomms11067⟩. ⟨hal-01301863⟩
  • Chantal Sellier, Karen Usdin, Chiara Pastori, Veronica Peschansky, Flora Tassone, et al.. The multiple molecular facets of fragile X-associated tremor/ataxia syndrome.. Journal of Neurodevelopmental Disorders, BioMed Central, 2013, pp.23. ⟨inserm-01086046⟩
  • Charlotte Fugier, Arnaud F Klein, Caroline Hammer, Stéphane Vassilopoulos, Ylva Ivarsson, et al.. Misregulated alternative splicing of BIN1 is associated with T tubule alterations and muscle weakness in myotonic dystrophy.. Nature Medicine, Nature Publishing Group, 2011, 17 (6), pp.720-5. ⟨10.1038/nm.2374⟩. ⟨hal-00811986⟩