Robust behavioral assessment of the inducible Friedreich's ataxia mouse does not show improvement with NRF2 induction.

Friedreich's ataxia, a recessive disorder caused by a mutation in the frataxin (Fxn) gene, has few mouse models that demonstrate a progressive behavioral decline paralleling patients. A mouse model of systemic frataxin deficiency, the FXNKD, was recently developed using a doxycycline inducible method; it is thought to mimic the patient phenotype seen where frataxin levels are decreased, but it is not determined whether it is reliable for assessment of therapeutics. FXNKD mice underwent testing for twelve weeks alongside littermates, undergoing tests of motor function, gait, and sensation.