|Other Names||Transcription factor A, mitochondrial, mtTFA, Mitochondrial transcription factor 1, MtTF1, Transcription factor 6, TCF-6, Transcription factor 6-like 2, TFAM, TCF6, TCF6L2|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Binds to the mitochondrial light strand promoter and functions in mitochondrial transcription regulation. Required for accurate and efficient promoter recognition by the mitochondrial RNA polymerase. Promotes transcription initiation from the HSP1 and the light strand promoter by binding immediately upstream of transcriptional start sites. Is able to unwind DNA. Bends the mitochondrial light strand promoter DNA into a U-turn shape via its HMG boxes. Required for maintenance of normal levels of mitochondrial DNA. May play a role in organizing and compacting mitochondrial DNA.|
|Cellular Location||Mitochondrion. Mitochondrion matrix, mitochondrion nucleoid|
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This gene encodes a mitochondrial transcription factorthat is a key activator of mitochondrial transcription as well as aparticipant in mitochondrial genome replication. Studies in micehave demonstrated that this gene product is required to regulatethe mitochondrial genome copy number and is essential for embryonicdevelopment. A mouse model for Kearns-Sayre syndrome was producedwhen expression of this gene was eliminated by targeted disruptionin heart and muscle cells.
Wang, W., et al. Nucleic Acids Res. (2010) In press :Corneveaux, J.J., et al. Hum. Mol. Genet. 19(16):3295-3301(2010)Akhmetov, I.I., et al. Fiziol Cheloveka 36(2):121-125(2010)Shulman, J.M., et al. PLoS ONE 5 (6), E11244 (2010) :Laumet, G., et al. J. Alzheimers Dis. 20(4):1181-1188(2010)
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