Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype.

Wenz T; Diaz F; Spiegelman BM

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Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype.

机译：PPAR / PGC-1alpha途径的激活可防止生物能不足，并有效改善线粒体肌病表型。

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Neuromuscular disorders with defects in the mitochondrial ATP-generating system affect a large number of children and adults worldwide, but remain without treatment. We used a mouse model of mitochondrial myopathy, caused by a cytochrome c oxidase deficiency, to evaluate the effect of induced mitochondrial biogenesis on the course of the disease. Mitochondrial biogenesis was induced either by transgenic expression of peroxisome proliferator-activated receptor gamma (PPARgamma) coactivator alpha (PGC-1alpha) in skeletal muscle or by administration of bezafibrate, a PPAR panagonist. Both strategies successfully stimulated residual respiratory capacity in muscle tissue. Mitochondrial proliferation resulted in an enhanced OXPHOS capacity per muscle mass. As a consequence, ATP levels were conserved resulting in a delayed onset of the myopathy and a markedly prolonged life span. Thus, induction of mitochondrial biogenesis through pharmacological or metabolic modulation of the PPAR/PGC-1alpha pathway promises to be an effective therapeutic approach for mitochondrial disorders.

机译：线粒体ATP生成系统中存在缺陷的神经肌肉疾病会影响全世界的大量儿童和成人，但仍未得到治疗。我们使用了由细胞色素C氧化酶缺乏症引起的线粒体肌病的小鼠模型，来评估诱导的线粒体生物发生对疾病进程的影响。线粒体过氧化物酶体增殖物激活受体γ（PPARgamma）共激活因子α（PGC-1alpha）在骨骼肌中的转基因表达或通过给予苯扎贝特（一种PPAR激动剂）来诱导线粒体生物发生。两种策略均成功地刺激了肌肉组织中的残余呼吸能力。线粒体增殖导致每肌肉质量的OXPHOS能力增强。结果，ATP水平被保守，导致肌病的发作延迟和生命周期显着延长。因此，通过PPAR / PGC-1alpha途径的药理或代谢调节诱导线粒体生物发生有望成为线粒体疾病的有效治疗方法。

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《Cell metabolism》 |2008年第3期|共8页
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Wenz T; Diaz F; Spiegelman BM;
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正文语种 eng
中图分类细胞生物学;
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1. Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype. [J] . Wenz T, Diaz F, Spiegelman BM Cell metabolism . 2008,第3期

机译：PPAR / PGC-1alpha途径的激活可防止生物能不足，并有效改善线粒体肌病表型。
2. Bezafibrate activation of PPAR drives disturbances in mitochondrial redox bioenergetics and decreases the viability of cells from patients with VLCAD deficiency [J] . Lund Martin, Andersen Kathrine G., Heaton Robert, Biochimica et biophysica acta. Molecular basis of disease: BBA . 2021,第6期

机译：PPAR的Bezafibrate激活PPAR的紊乱在线粒体氧化还原生物能器中的障碍，并降低了VLCAD缺乏患者的细胞的活力
3. Major royal jelly proteins prevents NAFLD by improving mitochondrial function and lipid accumulation through activating the AMPK/ SIRT3 pathway in vitro [J] . Xiaochen Zhang, Xinyang Lu, Yingjun Zhou, Journal of Food Science . 2021,第1a3期

机译：主要的蜂王浆蛋白通过在体外激活AMPK / SIRT3途径来改善线粒体功能和脂质积累来防止NAFLD
4. Targeting Heat Shock Protein 70 to Improve Oxidative Stress and Mitochondrial Bioenergetics in Diabetic Sensory Neurons [D] . You, Zhenyuan. 2017

机译：靶向热休克蛋白70改善糖尿病感觉神经元的氧化应激和线粒体生物能。
5. Retraction Notice to: Activation of the PPAR/PGC-1α Pathway Prevents a Bioenergetic Deficit and Effectively Improves a Mitochondrial Myopathy Phenotype [O] . Tina Wenz, Francisca Diaz, Bruce M. Spiegelman, -1

机译：撤回通知：PPAR /PGC-1α途径的激活可防止生物能不足并有效改善线粒体肌病表型
6. Retraction Notice to: Activation of the PPAR/PGC-1α Pathway Prevents a Bioenergetic Deficit and Effectively Improves a Mitochondrial Myopathy Phenotype [O] . Tina Wenz, Francisca Diaz, Bruce M. Spiegelman, 2016

机译：缩回通知：PPAR / PGC-1α途径的激活可防止生物能量缺陷，有效改善线粒体肌病表型

Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype.

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