Team 4 – Gourdon group

Several years ago, we created a transgenic mouse model (DMSXL) carrying very large human genomic sequences containing the DMPK gene and the largest CTG repeat introduced in mice so far (>1500CTG). These mice show a very high level of CTG repeat instability and reproduce molecular and physiological feature of DM1.

Our research follows 3 axes:

  • Mechanisms involved in trinucleotide repeat instability
    • From mouse & cell models to DM1 families
  • Molecular and physio-pathological consequences of CTG repeat expansions
    • behind the neuropsychological impairment and brain abnormalities
    • involved in the congenital form of the disease
  • Preclinical testing in DM1 mice
    • Muscle & brain molecular and physiological phenotypes

 

Group members

Geneviève GOURDON, PhD, DR-Inserm, Group leader
Mario GOMES-PEREIRA, PhD, CR-Inserm
Stéphanie TOME, PhD, CR-Inserm
Aline HUGUET-LACHON IE, University
Hélène BENYAMINE, IE
Anchel GONZALEZ, Post-Doc
Mouli CHAKRABORTY, Post-Doc
Antoine MANGIN, AI
Aurélien CORDIER, AI
Sandra BRAZ, Doctorate
Elodie DANDELOT, Doctorate

Contact  : genevieve.gourdon@inserm.fr

 

Relevant publications

Other publications

https://www.ncbi.nlm.nih.gov/pubmed/?term=Gourdon+g