The Role of DNA Repair in Maintaining Mitochondrial DNA Stability

Mitochondria are vital double-membrane organelles that act as a "powerhouse" inside the cell and have essential roles to maintain cellular functions, e.g., ATP production, iron-sulfur synthesis metabolism, and steroid synthesis. An important difference with other organelles is that they contain their own mitochondrial DNA (mtDNA). Such powerful organelles are also sensitive to both endogenous and exogenous factors that can cause lesions to their structural components and their mtDNA, resulting in gene mutations and eventually leading to diseases. In this review, we will mainly focus on mammalian mitochondrial DNA repair pathways that safeguard mitochondrial DNA integrity and several important factors involved in the repair process, especially on an essential pathway, base excision repair. We eagerly anticipate to explore more methods to treat related diseases by constantly groping for these complexes and precise repair mechanisms.