Mitochondrial import receptor subunit TOM70 (TOMM70)

Mitochondrial import receptor subunit TOM70 (TOMM70) is an integral component of the translocase of the outer mitochondrial membrane (TOM complex), which forms the main entry gate for the vast majority of nuclear-encoded mitochondrial proteins[1][3][4][6]. TOM70 acts as a receptor that recognizes preprotein–chaperone complexes and mediates the translocation of mitochondrial precursor proteins from the cytosol into the mitochondria, facilitating their passage through the TOMM40 channel in a chaperone-dependent manner[1][3][6]. Structurally, TOMM70 contains multiple tetratricopeptide repeat (TPR) motifs that aid protein–protein interactions[1]. In addition to supporting mitochondrial protein import, TOM70 is involved in cellular antiviral signaling, mitochondrial quality control, regulated apoptosis, and calcium transfer at ER-mitochondria contact sites[1][3]. Pathogenic variation in TOMM70 is associated with neurological disorders involving white matter disease, hypotonia, ataxia, and cognitive deficits, while broader roles are implicated in cancer, metabolic diseases, and infection[1][3]. TOMM70 is essential for viability and broadly expressed, especially in the central nervous system[1].

Other names

Translocase of outer mitochondrial membrane 70TOM70TOMM70ATranslocase of outer mitochondrial membrane 70 homolog AKIAA0719Translocase of outer membrane 70 kDa subunitTranslocase of outer mitochondrial membrane protein 70Mitochondrial precursor proteins import receptor

Modulation of mitochondrial protein import; Regulation of mitochondrial-mediated apoptosis; Regulation of antiviral signaling pathways