Single-stranded DNA binding protein 1, mitochondrial (SSBP1)

Single-stranded DNA binding protein 1, mitochondrial (SSBP1) is a highly conserved protein that binds to single-stranded DNA in the mitochondrial matrix, stabilizing unwound DNA and preventing secondary structure formation during replication and repair[2][3][5][7]. SSBP1 ensures proper replication and maintenance of the mitochondrial genome by interacting with key replisome components such as DNA polymerase γ and the mitochondrial helicase Twinkle, thereby promoting genome stability, mtDNA synthesis, and protecting the genome from damage[2][3][5]. SSBP1 is critical for mitochondrial biogenesis and adapts under proteotoxic stress by translocating to the nucleus and regulating gene expression[3]. Mutations in SSBP1 are linked to several severe mitochondrial disorders, including mtDNA depletion syndrome, Leigh syndrome, MELAS, and inherited optic neuropathies[2][3]. Overexpression or altered regulation of SSBP1 is associated with enhanced proliferation and poor prognosis in multiple malignancies, underscoring its role in both mitochondrial disease and cancer biology[5]. No drugs are currently known to directly target SSBP1, but its central role in mitochondrial genome maintenance makes it a potential therapeutic target and disease biomarker in relevant conditions[3][5].

Other names

Mitochondrial single-stranded DNA-binding proteinSSBPmtSSBMt-SSBOPA13SOSS-B1PWP1-interacting protein 17