Tissue-Specif sc Loss of DARS2 Activates Stress Responses Independently of Respiratory Chain Deficiency in the Heart

Sukru Anil Dogan; Claire Pujol; Priyanka Maiti; Alexra Kukat; Shuaiyu Wang; Steffen Hermans; Katharina Senft; Rolf Wibom; Elena I. Rugarli; Aleksra Trifunovic

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Tissue-Specif sc Loss of DARS2 Activates Stress Responses Independently of Respiratory Chain Deficiency in the Heart

机译：DARS2的组织特异性sc丢失独立于心脏的呼吸链缺乏而激活应激反应。

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Adaptive stress responses activated upon mitochondria! dysfunction are assumed to arise in order to counteract respiratory chain deficiency. Here, we demonstrate that loss of DARS2 (mitochondrial aspartyi-tRNA synthetase) leads to the activation of various stress responses in a tissue-specific manner independently of respiratory chain deficiency. DARS2 depletion in heart and skeletal muscle leads to the severe deregulation of mitochondrial protein synthesis followed by a strong respiratory chain deficit in both tissues, yet the activation of adaptive responses is observed predominantly in cardiomyo-cytes. We show that the impairment of mitochondria! proteostasis in the heart activates the expression of mitokine FGF21, which acts as a signal for cell-autonomous and systemic metabolic changes. Conversely, skeletal muscle has an intrinsic mechanism relying on the slow turnover of mitochondrial transcripts and higher proteostatic buffering capacity. Our results show that mitochondrial dysfunction is sensed independently of respiratory chain deficiency, questioning the current view on the role of stress responses in mitochondrial diseases.

机译：线粒体激活适应性应激反应！假定出现功能障碍以抵消呼吸链缺乏症。在这里，我们证明了DARS2（线粒体天冬氨酰-tRNA合成酶）的丢失导致以组织特异性方式激活各种应激反应，而与呼吸链缺乏无关。心脏和骨骼肌中的DARS2耗竭会导致线粒体蛋白合成的严重失调，然后在两个组织中都出现强烈的呼吸链缺陷，但是在心肌细胞中主要观察到了适应性反应的激活。我们表明线粒体的损伤！心脏中的蛋白质稳态激活了有丝分裂因子FGF21的表达，这是细胞自主和全身代谢变化的信号。相反，骨骼肌具有内在机制，其依赖于线粒体转录物的缓慢转换和较高的蛋白静态缓冲能力。我们的结果表明，线粒体功能障碍的感觉独立于呼吸链缺乏，对当前应激反应在线粒体疾病中的作用提出了质疑。

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《Cell metabolism》 |2014年第3期|共12页
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Sukru Anil Dogan; Claire Pujol; Priyanka Maiti; Alexra Kukat; Shuaiyu Wang; Steffen Hermans; Katharina Senft; Rolf Wibom; Elena I. Rugarli; Aleksra Trifunovic;
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中图分类内分泌腺疾病及代谢病;
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1. Tissue-Specif sc Loss of DARS2 Activates Stress Responses Independently of Respiratory Chain Deficiency in the Heart [J] . Sukru Anil Dogan, Claire Pujol, Priyanka Maiti, Cell metabolism . 2014,第3期

机译：DARS2的组织特异性sc丢失独立于心脏的呼吸链缺乏而激活应激反应。
2. Human neuronal coenzyme Q10 deficiency results in global loss of mitochondrial respiratory chain activity, increased mitochondrial oxidative stress and reversal of ATP synthase activity: implications for pathogenesis and treatment [J] . Kate E. C. Duberley, Andrey Y. Abramov, Annapurna Chalasani, Journal of Inherited Metabolic Disease . 2013,第1期

机译：人类神经元辅酶Q10缺乏导致线粒体呼吸链活性全面丧失，线粒体氧化应激增加和ATP合酶活性逆转：对发病机理和治疗的影响
3. K deficiency caused defects in renal tubular cell proliferation, oxidative stress response, tissue repair and tight junction integrity, but enhanced energy production, proteasome function and cellular K+ uptakeproliferation, oxidative stress response, tissuerepair and tight junction integrity, but enhancedenergy production, proteasome function andcellular+ deficiency caused defects in renal tubular cellproliferation, oxidative stress response, tissuerepair and tight junction integrity, but enhancedenergy production, proteasome function andcellular K+ uptakeK deficiency caused defects in renal tubular cell proliferation, oxidative stress response, tissue repair and tight junction integrity, but enhanced energy production, proteasome function and cellular Ksup xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xmlns:oasis="http://docs.oasis-open.orgs/oasis-exchange/table"+/sup uptake [J] . Chompunoot Kapincharanon, Visith Thongboonkerd Cell adhesion & migration . 2018,第3期

机译：钾缺乏引起肾小管细胞增殖，氧化应激反应，组织修复和紧密连接完整性方面的缺陷，但增强了能量产生，蛋白酶体功能和细胞对K +的吸收，氧化应激反应，组织修复和紧密连接完整性，但增强了能量产生，蛋白酶体功能和细胞+缺乏会导致肾小管细胞增殖，氧化应激反应，组织修复和紧密连接完整性方面的缺陷，但能量产生，蛋白酶体功能和细胞对K +摄取的增加会导致肾小管细胞增殖，氧化应激反应，组织修复和紧密连接完整性方面的缺陷，但能量增加生产，蛋白酶体功能和细胞K ^{+ 吸收}
4. Hydrocarbons Adsorbed to Combustion-Derived PM Localize to Lipid Droplets and Activate Oxidative Stress Response Genes In Respiratory Cells (128910) [C] . Arthur L. Penn, Gleeson Murphy, Rodney L. Rouse American Institute of Chemical Engineers Annual Meeting . 2008

机译：吸附到燃烧衍生的PM的烃定位于脂质液滴，并在呼吸细胞中激活氧化应激响应基因（128910）
5. The molecular and biochemical characterization of human mitochondrial respiratory chain deficiencies [D] . Merante, Frank. 1996

机译：人类线粒体呼吸链缺陷的分子和生化特征
6. Mitochondrial respiratory chain complex IV deficiency presenting as neonatal respiratory distress syndrome [O] . Shrinal Kotecha, Venkatesh Kairamkonda 2019

机译：线粒体呼吸链复合IV缺乏呈现为新生儿呼吸窘迫综合征
7. Tissue-Specific Loss of DARS2 Activates Stress Responses Independently of Respiratory Chain Deficiency in the Heart [O] . Dogan Sukru Anil, Pujol Claire, Maiti Priyanka, 2014

机译：DARS2的组织特异性丢失独立于心脏中的呼吸链缺乏激活应激反应。

Tissue-Specif sc Loss of DARS2 Activates Stress Responses Independently of Respiratory Chain Deficiency in the Heart

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