Q00059

mtTFA(mitochondrial transcription factor A) also known as TFAM, TCF6 is a nuclear-encoded protein imported into mitochondria, where mtTFA plays a crucial role in mitochondrial biogenesis by regulating the replication and transcription of mitochondrial DNA (mtDNA). mtTFA function is essential for maintaining mtDNA copy number and integrity, which is vital for proper mitochondrial function and energy production in cells. Alterations in mtTFA expression have been linked to mitochondrial disorders characterized by symptoms like ocular myopathy, exercise intolerance, and muscle wasting, underscoring mtTFA importance in human health. During spermatogenesis in mice and humans, a reduction in mtTFA protein levels leads to decreased mtDNA copy number, which may help prevent paternal mtDNA transmission.