1. Natural products, PGC-1 alpha, and Duchenne muscular dystrophy
- Author
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Seyed Mohammad Nabavi, Samira Shirooie, Rosa Anna Vacca, Ipek Süntar, Tarun Belwal, Ana Sanches Silva, Antoni Sureda, Maryam Majidinia, Suowen Xu, Giuseppe D'Antona, Ahmad Reza Dehpour, Seyed Fazel Nabavi, Devesh Tewari, Eduardo Sobarzo-Sánchez, Bahman Yousefi, Maria Daglia, Suntar, I., Sureda, A., Belwal, T., Sanches Silva, A., Vacca, R. A., Tewari, D., Sobarzo-Sanchez, E., Nabavi, S. F., Shirooie, S., Dehpour, A. R., Xu, S., Yousefi, B., Majidinia, M., Daglia, M., D'Antona, G., and Nabavi, S. M.
- Subjects
MSCs, mesenchymal stem cells ,Duchenne muscular dystrophy ,Peroxisome proliferatoractivated receptor gamma coactivator 1 alpha ,MD, muscular dystrophy ,Peroxisome proliferator-activated receptor ,HDAC, histone deacetylase ,Review ,GPX, glutathione peroxidase ,TNF-α, tumor necrosis factor-α ,CREB, cyclic AMP response element-binding protein ,AAV, adeno-associated virus ,AMPK, 5′ adenosine monophosphate-activated protein kinase ,0302 clinical medicine ,BMD, Becker muscular dystrophy ,ASO, antisense oligonucleotides ,General Pharmacology, Toxicology and Pharmaceutics ,Muscular dystrophy ,NOS, NO synthase ,PGC-1, peroxisome proliferator-activated receptor γ coactivator 1 ,chemistry.chemical_classification ,EGCG, epigallocatechin-3-gallate ,0303 health sciences ,LDH, lactate dehydrogenase ,SIRT1, silent mating type information regulation 2 homolog 1 ,Chemistry ,NF-κB, nuclear factor kappa-light-chain-enhancer of activated B cells ,iPSCs, induced pluripotent stem cells ,DMD, Duchenne muscular dystrophy ,MCP-1, monocyte chemoattractant protein-1 ,DRP1, dynamin-related protein 1 ,NMJ, neuromuscular junctions ,VEGF, vascular endothelial growth factor ,DS, Down syndrome ,ECM, extracellular matrix ,Cell biology ,Mitochondrial oxidative phosphorylation ,PPARγ activation ,MyoD, myogenic differentiation ,030220 oncology & carcinogenesis ,FOXO1, forkhead box class-O1 ,UCP, uncoupling protein ,Signal transduction ,ATF2, activating transcription factor 2 ,ATP, adenosine triphosphate ,FDA, U. S. Food and Drug Administration ,PDGF, platelet derived growth factor ,HIF-1α, hypoxia-inducible factors ,GSK3b, glycogen synthase kinase 3b ,Peroxisome proliferator-activated receptor ? coactivator 1? ,Natural product ,PPAR gamma activation ,MEF2, myocyte enhancer factor 2 ,03 medical and health sciences ,ROS, reactive oxygen species ,ERRα, estrogen-related receptor alpha ,SOD, superoxide dismutase ,Coactivator ,medicine ,DGC, dystrophin–glycoprotein complex ,Transcription factor ,DAGC, dystrophin-associated glycoprotein complex ,030304 developmental biology ,NO, nitric oxide ,SPP1, secreted phosphoprotein 1 ,lcsh:RM1-950 ,Autophagy ,GABP, GA-binding protein ,NADPH, nicotinamide adenine dinucleotide phosphate ,medicine.disease ,CnA, calcineurin a ,FGF, fibroblast growth factor ,IL, interleukin ,PPARγ, peroxisome proliferator-activated receptor γ ,HCT, hydrochlorothiazide ,lcsh:Therapeutics. Pharmacology ,COPD, chronic obstructive pulmonary disease ,Mitochondrial biogenesis ,AMP, adenosine monophosphate ,cGMP, cyclic guanosine monophosphate ,p38 MAPK, p38 mitogen-activated protein kinase ,Peroxisome proliferator-activated receptor γ coactivator 1α ,Reactive oxygen species - Abstract
Peroxisome proliferator-activated receptor gamma (PPAR gamma) is a transcriptional coactivator that binds to a diverse range of transcription factors. PPAR gamma 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-1 alpha 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-1 alpha is influenced by different patho-physiological/pharmacological stimuli. Natural products have been reported to display modulatory effects on PPAR gamma 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-1 alpha., This work was supported by the crowd funding #Sport4Therapy to Giuseppe D'Antona (Italy). A. Sureda was supported by Instituto de Salud Carlos III, Grant Number: CIBEROBN CB12/03/30038.
- Published
- 2020
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