Table 1. Novel mitochondrially targeted agents for FRDA.

Agent	Mechanism	Status	References
Oleic acid derivative	Ferroptosis inhibitor	Preclinical	[,35]
Novel lipophilic phenothiazines	Ferroptosis inhibitor	Preclinical	[,36]
RT-001	Ferroptosis inhibitor	Pivotal trial	[,37,38]
PTC 743	Ferroptosis inhibitor	Pivotal trial	[,39]
Omaveloxolone	Nrf2 activator	Pivotal trial	[50,52,53]
Letiriglitozone	PPARgamma activator	Preclinical	[68]
Elamipretide	Cardioplipin facilitator	Early trials	[69–73]