关键词： AAV, adeno-associated virus AMP, adenosine monophosphate AMPK, 5′ adenosine monophosphate-activated protein kinase ASO, antisense oligonucleotides ATF2, activating transcription factor 2 ATP, adenosine triphosphate BMD, Becker muscular dystrophy COPD, chronic obstructive pulmonary disease CREB, cyclic AMP response element-binding protein CnA, calcineurin a DAGC, dystrophin-associated glycoprotein complex DGC, dystrophin–glycoprotein complex DMD, Duchenne muscular dystrophy DRP1, dynamin-related protein 1 DS, Down syndrome ECM, extracellular matrix EGCG, epigallocatechin-3-gallate ERRα, estrogen-related receptor alpha FDA, U. S. Food and Drug Administration FGF, fibroblast growth factor FOXO1, forkhead box class-O1 GABP, GA-binding protein GPX, glutathione peroxidase GSK3b, glycogen synthase kinase 3b HCT, hydrochlorothiazide HDAC, histone deacetylase HIF-1α, hypoxia-inducible factors IL, interleukin LDH, lactate dehydrogenase MCP-1, monocyte chemoattractant protein-1 MD, muscular dystrophy MEF2, myocyte enhancer factor 2 MSCs, mesenchymal stem cells Mitochondrial oxidative phosphorylation Muscular dystrophy MyoD, myogenic differentiation NADPH, nicotinamide adenine dinucleotide phosphate NF-κB, nuclear factor kappa-light-chain-enhancer of activated B cells NMJ, neuromuscular junctions NO, nitric oxide NOS, NO synthase Natural product PDGF, platelet derived growth factor PGC-1, peroxisome proliferator-activated receptor γ coactivator 1 PPARγ activation PPARγ, peroxisome proliferator-activated receptor γ Peroxisome proliferator-activated receptor γ coactivator 1α ROS, reactive oxygen species Reactive oxygen species SIRT1, silent mating type information regulation 2 homolog 1 SOD, superoxide dismutase SPP1, secreted phosphoprotein 1 TNF-α, tumor necrosis factor-α UCP, uncoupling protein VEGF, vascular endothelial growth factor cGMP, cyclic guanosine monophosphate iPSCs, induced pluripotent stem cells p38 MAPK, p38 mitogen-activated protein kinase

来  源：   DOI：10.1016/j.apsb.2020.01.001   PDF(Sci-hub)   PDF(Pubmed)

Abstract：
Peroxisome proliferator-activated receptor γ (PPARγ) is a transcriptional coactivator that binds to a diverse range of transcription factors. PPARγ coactivator 1 (PGC-1) coactivators possess an extensive range of biological effects in different tissues, and play a key part in the regulation of the oxidative metabolism, consequently modulating the production of reactive oxygen species, autophagy, and mitochondrial biogenesis. Owing to these findings, a large body of studies, aiming to establish the role of PGC-1 in the neuromuscular system, has shown that PGC-1 could be a promising target for therapies targeting neuromuscular diseases. Among these, some evidence has shown that various signaling pathways linked to PGC-1α are deregulated in muscular dystrophy, leading to a reduced capacity for mitochondrial oxidative phosphorylation and increased reactive oxygen species (ROS) production. In the light of these results, any intervention aimed at activating PGC-1 could contribute towards ameliorating the progression of muscular dystrophies. PGC-1α is influenced by different patho-physiological/pharmacological stimuli. Natural products have been reported to display modulatory effects on PPARγ activation with fewer side effects in comparison to synthetic drugs. Taken together, this review summarizes the current knowledge on Duchenne muscular dystrophy, focusing on the potential effects of natural compounds, acting as regulators of PGC-1α.
摘要：
过氧化物酶体增殖物激活受体γ(PPARγ)是与多种转录因子结合的转录共激活因子。PPARγ共激活因子1(PGC-1)在不同组织中具有广泛的生物学效应,并在调节氧化代谢中起关键作用，从而调节活性氧的产生，自噬,和线粒体生物发生。由于这些发现，大量的研究，旨在确定PGC-1在神经肌肉系统中的作用，已经表明PGC-1可能是针对神经肌肉疾病的疗法的有希望的靶标。其中,一些证据表明，与PGC-1α相关的各种信号通路在肌营养不良中失调，导致线粒体氧化磷酸化能力降低，活性氧（ROS）产生增加。根据这些结果,任何旨在激活PGC-1的干预措施都可能有助于改善肌营养不良的进展.PGC-1α受不同病理生理/药理学刺激的影响。据报道，天然产物对PPARγ活化具有调节作用，与合成药物相比副作用较少。一起来看,这篇综述总结了杜氏肌营养不良的最新知识，关注天然化合物的潜在影响，作为PGC-1α的调节剂。
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