Transcription Factor A, Mitochondrial (TFAM)

TFAM encodes a mitochondrial transcription factor that is a key activator of mitochondrial transcription as well as a participant in mitochondrial genome replication. Studies in mice have demonstrated that this gene product is required to regulate the mitochondrial genome copy number and is essential for embryonic development. A mouse model for Kearns-Sayre syndrome was produced when expression of this gene was eliminated by targeted disruption in heart and muscle cells.The mature TCF6 gene product, mitochondrial transcription factor A (TFAM; also known as mtTF1 or mtTFA), is a 162-amino acid protein that activates transcription of each mitochondrial DNA (mtDNA) strand by binding to an element of approximately 30 nucleotides present in both the light-strand and the heavy-strand promoters.