» Articles » PMID: 32528825

Natural Products, PGC-1 , and Duchenne Muscular Dystrophy

Abstract

Peroxisome proliferator-activated receptor (PPAR) is a transcriptional coactivator that binds to a diverse range of transcription factors. PPAR 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 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 is influenced by different patho-physiological/pharmacological stimuli. Natural products have been reported to display modulatory effects on PPAR 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.

Citing Articles

The role of sirtuins in the regulation of reactive oxygen species in myocardial ischemia/reperfusion injury.

Wang Z, Zhao X, Lu M, Wang N, Xu S, Min D Mol Cell Biochem. 2025; .

PMID: 39920412 DOI: 10.1007/s11010-024-05204-9.


Muscular TOR knockdown and endurance exercise ameliorate high salt and age-related skeletal muscle degradation by activating the MTOR-mediated pathway.

Wang S, Wen D, Gao Y, Wang J, Ma X PLoS One. 2025; 20(1):e0311159.

PMID: 39841657 PMC: 11753686. DOI: 10.1371/journal.pone.0311159.


Sarcopenia and cachexia: molecular mechanisms and therapeutic interventions.

Wang T, Zhou D, Hong Z MedComm (2020). 2025; 6(1):e70030.

PMID: 39764565 PMC: 11702502. DOI: 10.1002/mco2.70030.


RBBP6-Mediated ERRα Degradation Contributes to Mitochondrial Injury in Renal Tubular Cells in Diabetic Kidney Disease.

Hu H, Hu J, Chen Z, Yang K, Zhu Z, Hao Y Adv Sci (Weinh). 2024; 11(46):e2405153.

PMID: 39441040 PMC: 11633482. DOI: 10.1002/advs.202405153.


Exploring the Mechanism of Ferroptosis Induction by Sappanone A in Cancer: Insights into the Mitochondrial Dysfunction Mediated by NRF2/xCT/GPX4 Axis.

Wang J, Zhuang H, Yang X, Guo Z, Zhou K, Liu N Int J Biol Sci. 2024; 20(13):5145-5161.

PMID: 39430236 PMC: 11488586. DOI: 10.7150/ijbs.96748.


References
1.
Arany Z . PGC-1 coactivators and skeletal muscle adaptations in health and disease. Curr Opin Genet Dev. 2008; 18(5):426-34. PMC: 2629557. DOI: 10.1016/j.gde.2008.07.018. View

2.
Pan Y, Chen C, Shen Y, Zhu C, Wang G, Wang X . Curcumin alleviates dystrophic muscle pathology in mdx mice. Mol Cells. 2008; 25(4):531-7. View

3.
Dorchies O, Wagner S, Vuadens O, Waldhauser K, Buetler T, Kucera P . Green tea extract and its major polyphenol (-)-epigallocatechin gallate improve muscle function in a mouse model for Duchenne muscular dystrophy. Am J Physiol Cell Physiol. 2006; 290(2):C616-25. DOI: 10.1152/ajpcell.00425.2005. View

4.
Wang M, Wu B, Shah S, Lu P, Lu Q . Saponins as Natural Adjuvant for Antisense Morpholino Oligonucleotides Delivery In Vitro and in mdx Mice. Mol Ther Nucleic Acids. 2018; 11:192-202. PMC: 5992344. DOI: 10.1016/j.omtn.2018.02.004. View

5.
Minetti G, Colussi C, Adami R, Serra C, Mozzetta C, Parente V . Functional and morphological recovery of dystrophic muscles in mice treated with deacetylase inhibitors. Nat Med. 2006; 12(10):1147-50. DOI: 10.1038/nm1479. View