Figure 11.

Mutual relationships among mtDNA, M-LPH, TFAM, and BER enzymes. TFAM fully coats mtDNA and protects it from oxidative damage. Phosphorylation of TFAM blocks its binding to mtDNA, and the DNA-free TFAM is rapidly and selectively degraded by LONP1. When the protective effect of TFAM is lost, mtDNA becomes vulnerable to oxidative stress, resulting in mitochondrial dysfunction. LIG3 and OGG1 are involved in mitochondrial BER. It is likely that M-LPH mitigates the phosphorylation of TFAM, thus protecting it from degradation.