C17orf80 binds the mitochondrial genome to promote its replication

Author:

Wu Hao12ORCID,Zhang Wenshuo3ORCID,Xu Fengli1ORCID,Peng Kun1ORCID,Liu Xiaoyu1ORCID,Ding Wanqiu1ORCID,Ma Qi1ORCID,Cheng Heping14ORCID,Wang Xianhua14ORCID

Affiliation:

1. State Key Laboratory of Membrane Biology, Beijing Key Laboratory of Cardiometabolic Molecular Medicine, Peking-Tsinghua Center for Life Sciences, Institute of Molecular Medicine, College of Future Technology, Peking University 1 , Beijing, China

2. Academy of Advanced Interdisciplinary Study, Peking University 2 , Beijing, China

3. Peking-Tsinghua Center for Life Sciences, College of Life Sciences, Peking University 4 , Beijing, China

4. Research Unit of Mitochondria in Brain Diseases, Chinese Academy of Medical Sciences, PKU-Nanjing Institute of Translational Medicine 3 , Nanjing, China

Abstract

Serving as the power plant and signaling hub of a cell, mitochondria contain their own genome which encodes proteins essential for energy metabolism and forms DNA–protein assemblies called nucleoids. Mitochondrial DNA (mtDNA) exists in multiple copies within each cell ranging from hundreds to tens of thousands. Maintaining mtDNA homeostasis is vital for healthy cells, and its dysregulation causes multiple human diseases. However, the players involved in regulating mtDNA maintenance are largely unknown though the core components of its replication machinery have been characterized. Here, we identify C17orf80, a functionally uncharacterized protein, as a critical player in maintaining mtDNA homeostasis. C17orf80 primarily localizes to mitochondrial nucleoid foci and exhibits robust double-stranded DNA binding activity throughout the mitochondrial genome, thus constituting a bona fide new mitochondrial nucleoid protein. It controls mtDNA levels by promoting mtDNA replication and plays important roles in mitochondrial metabolism and cell proliferation. Our findings provide a potential target for therapeutics of human diseases associated with defective mtDNA control.

Funder

National Natural Science Foundation of China

National Key Basic Research Program of China

CAMS Innovation Fund for Medical Sciences

Publisher

Rockefeller University Press

Subject

Cell Biology

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