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.

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

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.

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)