Mitochondrial fission process 1 protein (MTFP1) (MTFP1)

Mitochondrial fission process 1 protein (MTFP1) is an integral inner mitochondrial membrane protein that regulates mitochondrial fusion, primarily by facilitating outer membrane fusion through GTP hydrolysis and dimerization with MTFP2. It features an N-terminal GTP-binding domain, central transmembrane domains for anchoring, and a C-terminal dimerization domain, enabling interactions that maintain mitochondrial network integrity, membrane potential, and respiratory function. MTFP1 also modulates the mitochondrial permeability transition pore (mPTP) complex by binding components like ANT and CypD, preventing aberrant pore opening that could lead to depolarization, ATP loss, and apoptosis. Expressed widely in tissues such as heart, liver, skeletal muscle, and colon, it balances fusion against fission mediated by Drp1, supporting homeostasis in energy-demanding cells. Dysregulation of MTFP1 contributes to pathologies including neurodegeneration, cardiomyopathy, and cancer via fragmented mitochondria, elevated ROS, and disrupted bioenergetics, positioning it as an emerging therapeutic target despite limited drug data.

Other names

Mitochondrial fission process 1Mitochondrial outer membrane fusion protein 1