Elamipretide 2TFA (RX-31 2TFA) is a novel mitochondrion-targeted antioxidant.

Elamipretide (MTP-131) is a mitochondria-targeted antioxidant tetrapeptide (D-Arg-dimethylTyr-Lys-Phe-NH2) that reduces toxic reactive oxygen species (ROS) production and stabilizes cardiolipin, with blood-brain-barrier permeability. Elamipretide is a potential therapeutic agent in the rare disease of Barnes syndrome.

MTP 131 is a mitochondria-targeted peptide antioxidant.1,2It localizes to the mitochondria and reducestert-butyl hydroperoxide-induced lipid peroxidation and apoptosis in SH-SY5Y cells when used at concentrations ranging from 0.001 to 1 nM.1MTP 131 (2 mg/kg) reduces infarct volume, hemispheric swelling, and glutathione (GSH) depletion in a mouse model of acute cerebral ischemia induced by middle cerebral artery occlusion (MCAO).2It increases survival, improves motor function, and decreases degeneration of the lumbar spinal cord in a superoxide dismutase 1 mutant (SOD1G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS) when administered at a dose of 5 mg/kg. MTP 131 reduces albuminuria, urinary hydrogen peroxide levels, and mesangial matrix accumulation, as well as preserves superoxide production, in adb/dbmouse model of diabetic nephropathy.3 1.Zhao, K., Luo, G., Giannelli, S., et al.Mitochondria-targeted peptide prevents mitochondrial depolarization and apoptosis induced by tert-butyl hydroperoxide in neuronal cell linesBiochem. Pharmacol.70(12)1796-1806(2005) 2.Szeto, H.H.Mitochondria-targeted peptide antioxidants: Novel neuroprotective agentsAAPS J.8(3)E521-E531(2006) 3.Miyamoto, S., Zhang, G., Hall, D., et al.Restoring mitochondrial superoxide levels with elamipretide (MTP-131) protects db/db mice against progression of diabetic kidney diseaseJ. Biol. Chem.295(21)7249-7260(2020)

Elamipretide acetate (MTP 131 acetate) is a derivative of Elamipretide, a small mitochondria-targeting tetrapeptide (D-Arg-dimethylTyr-Lys-Phe-NH2) that can be used in studies to reduce the production of toxic reactive oxygen species and stabilize cardiolipin.

Elamipretide TFA (RX-31 TFA) is a cardiolipin peroxidase inhibitor and mitochondria-targeting peptide[1].

Elamipretide Triacetate (Elamipretide TriTFA) is an aromatic-cationic tetrapeptide that readily penetrates cell membranes and transiently localizes to the inner mitochondrial membrane where it associates with cardiolipin.It has been used in trials studying the treatment of Leber's Hereditary Optic Neuropathy.