SS-31
Also known as · Elamipretide · MTP-131 · Bendavia
Overview
A small cell-permeable peptide that selectively concentrates in the inner mitochondrial membrane via cardiolipin binding. Investigated for mitochondrial dysfunction in Barth syndrome, primary mitochondrial myopathy, and heart failure.
Research profile · for clinicians
Based on research: a mitochondria-targeted peptide (elamipretide) that concentrates in the inner mitochondrial membrane by binding cardiolipin. Studied in primary mitochondrial myopathy, Barth syndrome, and heart failure for stabilization of ATP output and reduced oxidative stress.
Mechanism of action
Stabilizes cardiolipin and the electron-transport-chain supercomplex assembly, reducing reactive oxygen species generation and preserving ATP synthesis under stress.
Research record
- [01]MMPOWER program — Phase II/III primary mitochondrial myopathy trials.
- [02]TAZPOWER trial in Barth syndrome.
- [03]Preclinical heart-failure and ischemia-reperfusion studies.
Selected references
Elamipretide in primary mitochondrial myopathy (MMPOWER-3)
Neurology, 2022