This gene 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. [provided by RefSeq
Other Designations:
OTTHUMP00000019633,Transcription factor 6-like 2 (mitochondrial transcription factor),mitochondrial transcription factor A