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Mitochondrial Transcription Factor A and Related Diseases

Zhang Jiliang^1,2,3#, Zhang Yijia^4#, Wang Jie^1,3#, Wu Zhi^1,2,3, Zhang Ya^1,2,3, Zeng Lin^1,3, Ma Luoyan^1,3,Chen Tengfei^1,3, Wang Lu^1,2,3, Liu Yongzhang

¹Institute of Biophysics, Wenzhou Medical College, Wenzhou 325035, China; ²Attardi Institute of Mitochondrial Biomedicine, Wenzhou Medical College, Wenzhou 325035, China; ³Department of Biology, School of Laboratory Medici

Abstract: A high-mobility group (HMG) protein， the human mitochondrial transcription factor A (TFAM) is encoded in the nucleus and imported into mitochondria， where it plays an important role in regulating mtDNA copy number， transcription and maintenance. Numerous studies indicated that depletion of TFAM protein leads to mtDNA mutation and decreased mtDNA copy number， which can cause the mitochondrial dysfunction and diseases. Many published papers have shown the effect of TFAM gene polymorphism and the change of its protein expression level on related diseases， but the mechanism needed to be further clarified. In this review， we provide a brief summary of current insights of mitochondrial transcription factor A and its related diseases.

CSTR: 32200.14.cjcb.2013.05.0023