AI Article Synopsis

  • - Mitochondrial diseases are genetic disorders caused by mutations in nuclear and mitochondrial DNA, affecting thousands globally, but most remain incurable despite ongoing research efforts.
  • - Recent studies suggest targeting cellular compensatory pathways like mitophagy and the mitochondrial unfolded protein response (UPR) as potential treatment strategies for these diseases.
  • - A promising compound, pterostilbene, was found to enhance mitochondrial function, especially when combined with a cocktail of other beneficial molecules (CoC3), improving conditions in mutant cells and neurons.

Article Abstract

Mitochondrial diseases are genetic disorders caused by mutations in genes in the nuclear DNA (nDNA) and mitochondrial DNA (mtDNA) that encode mitochondrial structural or functional proteins. Although considered "rare" due to their low incidence, such diseases affect thousands of patients' lives worldwide. Despite intensive research efforts, most mitochondrial diseases are still incurable. Recent studies have proposed the modulation of cellular compensatory pathways such as mitophagy, AMP-activated protein kinase (AMPK) activation or the mitochondrial unfolded protein response (UPR) as novel therapeutic approaches for the treatment of these pathologies. UPR is an intracellular compensatory pathway that signals mitochondrial stress to the nucleus for the activation of mitochondrial proteostasis mechanisms including chaperones, proteases and antioxidants. In this work a potentially beneficial molecule, pterostilbene (a resveratrol analogue), was identified as mitochondrial booster in drug screenings. The positive effects of pterostilbene were significantly increased in combination with a mitochondrial cocktail (CoC3) consisting of: pterostilbene, nicotinamide, riboflavin, thiamine, biotin, lipoic acid and l-carnitine. CoC3 increases sirtuins' activity and UPR activation, thus improving pathological alterations in mutant fibroblasts and induced neurons.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8971666PMC
http://dx.doi.org/10.3389/fphar.2022.862085DOI Listing

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