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Advances in Mitochondrial Nucleoid Structure and Related Diseases


Xiang-Yu He1, Li-Ping Jin2, Zhong Liu3, Qing-Feng Yan1*
1Institute of Genetics, College of Life Science, Zhejiang University, Hangzhou 310058, China;2Anxi Health Center, Liangzhu, Hangzhou 311113, China; 3The First Affiliated Hospital of College of Medicine, Zhejiang University
Abstract: Mitochondrial DNA (mtDNA) is organized in solid particles with a series of proteins, and these nucleoprotein complexes are named as mitochondrial nucleoids because of the prokaryotic origin of mitochondria. Proteins in mitochondrial nucleoids consist of mitochondrial transcription factors, mitochondrial single stranded- DNA binding proteins and multiple functional proteins which catalyze the metabolic reactions in mitochondria. The elucidation of mitochondrial nucleoid structure is meaningful to interpret the mechanisms of the maintenance and inheritance of mitochondrial genome, and in revealing the potential regulation of mitochondrial gene expression. This review focuses on the current opinions in the components, structure and functions of mitochondrial nucleoid, especially how these proteins coordinate the mtDNA and mitochondrial metabolisms like TCA cycle and amino acid metabolism. Meanwhile, abnormality in nucleoid-associated proteins leads to many human diseases. The research in mitochondrial nucleoids is helpful to develop new therapeutics.


CSTR: 32200.14.cjcb.2011.02.0011