Friday, August 23, 2024

A multiple animal and cellular models approach to study frataxin deficiency in Friedreich Ataxia

Valentine Mosbach, Hélène Puccio, A multiple animal and cellular models approach to study frataxin deficiency in Friedreich Ataxia, Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, Volume 1871, Issue 7, 2024, 119809, ISSN 0167-4889, doi:10.1016/j.bbamcr.2024.119809. 

 Over the years, several cellular and animal models for FA have been developed. These models are all complementary and possess their own strengths to investigate different aspects of the disease, such as the epigenetics of the locus or the pathophysiology of the disease, as well as being used to developed novel therapeutic approaches. This review will explore the recent advancements in the different mammalian models developed for FA.