Mitochondrial Trafficking and Processing of Telomerase RNA TERC
Ying Cheng,
Peipei Liu,
Qian Zheng,
Ge Gao,
Jiapei Yuan,
Pengfeng Wang,
Jinliang Huang,
Leiming Xie,
Xinping Lu,
Tanjun Tong,
Jun Chen,
Zhi Lu,
Jisong Guan,
Geng Wang
Affiliations
Ying Cheng
MOE Key Laboratory of Bioinformatics, Cell Biology and Development Center, School of Life Sciences, Tsinghua University, Beijing 100084, China
Peipei Liu
MOE Key Laboratory of Bioinformatics, Cell Biology and Development Center, School of Life Sciences, Tsinghua University, Beijing 100084, China
Qian Zheng
MOE Key Laboratory of Bioinformatics, Cell Biology and Development Center, School of Life Sciences, Tsinghua University, Beijing 100084, China
Ge Gao
MOE Key Laboratory of Bioinformatics, Cell Biology and Development Center, School of Life Sciences, Tsinghua University, Beijing 100084, China
Jiapei Yuan
MOE Key Laboratory of Bioinformatics, Cell Biology and Development Center, School of Life Sciences, Tsinghua University, Beijing 100084, China
Pengfeng Wang
Peking University Research Center on Aging, Beijing 100191, China
Jinliang Huang
MOE Key Laboratory of Bioinformatics, Cell Biology and Development Center, School of Life Sciences, Tsinghua University, Beijing 100084, China
Leiming Xie
MOE Key Laboratory of Bioinformatics, Cell Biology and Development Center, School of Life Sciences, Tsinghua University, Beijing 100084, China
Xinping Lu
MOE Key Laboratory of Bioinformatics, Cell Biology and Development Center, School of Life Sciences, Tsinghua University, Beijing 100084, China
Tanjun Tong
Peking University Research Center on Aging, Beijing 100191, China; Department of Biochemistry and Molecular Biology, Peking University Health Science Center, Beijing 100191, China
Jun Chen
Peking University Research Center on Aging, Beijing 100191, China; Department of Biochemistry and Molecular Biology, Peking University Health Science Center, Beijing 100191, China
Zhi Lu
MOE Key Laboratory of Bioinformatics, Cell Biology and Development Center, School of Life Sciences, Tsinghua University, Beijing 100084, China
Jisong Guan
MOE Key Laboratory of Bioinformatics, Cell Biology and Development Center, School of Life Sciences, Tsinghua University, Beijing 100084, China
Geng Wang
MOE Key Laboratory of Bioinformatics, Cell Biology and Development Center, School of Life Sciences, Tsinghua University, Beijing 100084, China; Corresponding author
Summary: Mitochondrial dysfunctions play major roles in many diseases. However, how mitochondrial stresses are relayed to downstream responses remains unclear. Here we show that the RNA component of mammalian telomerase TERC is imported into mitochondria, processed to a shorter form TERC-53, and then exported back to the cytosol. We found that the import is regulated by PNPASE, and the processing is controlled by mitochondrion-localized RNASET2. Cytosolic TERC-53 levels respond to changes in mitochondrial functions but have no direct effect on these functions. These findings uncover a mitochondrial RNA trafficking pathway and provide a potential mechanism for mitochondria to relay their functional states to other cellular compartments. : The functions of most mitochondrial RNAs imported from the cytosol are poorly understood. Cheng et al. provide evidence that the RNA component of mammalian telomerase TERC is imported into mitochondria, processed to a shorter form TERC-53, and then exported back to the cytosol. The cytosolic TERC-53 level serves as a potential indicator of mitochondrial functions. Keywords: mitochondria, RNA, import, export, processing, mitochondrial dysfunction, retrograde signaling, TERC, telomerase, RNASET2