Thursday, September 15, 2022

A promissing mouse model for Friedreich Ataxia progressing like human patients

Catherine GĂ©rard, Annabelle Fortin Archambault, Camille Bouchard, Jacques P. Tremblay; Behavioural Brain Research, 2022, 114107, doi:10.1016/j.bbr.2022.1141

Jackson Laboratories Inc. developed a new mouse model that has 800 GAA repeats. We demonstrate here that these mice accurately reflect the human disease with a progressive neuromuscular degeneration highlighted by the two beam tests and the beginning of heart hypertrophy at 26 weeks. YG8-800 mice are thus currently a promising mouse model for FRDA.