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AAT Bioquest

What are the processes of mitochondrial DNA (mtDNA) replication?

Posted June 6, 2024


Answer

Mitochondrial DNA replication follows a unidirectional process from two distinct origins: the origin of H-strand replication (OH) in the D-loop and the origin of L-strand replication (OL) downstream of OH. OH initiates synthesis of a daughter H-strand, displacing the parental H-strand. When the leading daughter H-strand passes OL, synthesis of the daughter L-strand begins. RNA primer synthesis is facilitated by transcription factors and proteins including TFAM,POLRMT, and TFB2M. DNA polymerase γ catalyzes strand synthesis, while Twinkle helicase unwinds DNA and TOP1MT relaxes supercoiled DNA. SSBP1 stabilizes single-stranded regions. RNA primers are removed by RNase H1, and MGME1 processes the 5′-end. After gap filling and ligation by DNA ligase III, daughter duplexes are linked at OH as hemicatenanes. TOP3A catalyzes decatenation, allowing daughter molecules to separate.

Additional resources

Mitochondrial DNA replication in mammalian cells: overview of the pathway

Mitochondria

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