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A Drug Combination Rescues Frataxin-Dependent Neural and Cardiac Pathophysiology in FA Models.

Rosella Abeti1, Mittal Jasoliya2, Sahar Al-Mahdawi3

  • 1Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL, Institute of Neurology, London, United Kingdom.

Frontiers in Molecular Biosciences
|June 6, 2022
PubMed
Summary

A novel combination of dimethyl fumarate and resveratrol shows promise for Friedreich

Keywords:
Dimethyl fumarate (DMF)Frataxin (FXN)Friedreich’s Ataxia (FA)Mitochondrial membrane potential (ΔΨm)Reactive Oxygen species (ROS)Resveratrol (Resv)

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Area of Science:

  • Neurogenetics
  • Mitochondrial Biology
  • Pharmacology

Background:

  • Friedreich's ataxia (FA) is a rare inherited neurodegenerative disorder affecting nervous system and heart.
  • Current FA therapies lack FDA/EMA approval, with strategies focusing on single agents to increase frataxin or support cellular functions.
  • Existing treatments have limitations, necessitating exploration of novel therapeutic approaches.

Purpose of the Study:

  • To investigate a combinatorial drug approach using FDA-approved compounds for Friedreich's ataxia.
  • To evaluate the efficacy of combining dimethyl fumarate and resveratrol in FA patient cells and animal models.
  • To assess the potential of this combination therapy in improving cellular function and mitigating disease pathology.

Main Methods:

  • Screened eight single-drug molecules, including dimethyl fumarate and resveratrol, in FA patient fibroblast cells.
  • Assessed the impact of single agents and their combination on FXN gene transcription and mitochondrial biogenesis.
  • Evaluated the combination's effect on neuronal and cardiomyocyte mitochondrial function, reactive oxygen species, and FA mouse model behavior.

Main Results:

  • Dimethyl fumarate and resveratrol individually increased FXN transcription and mitochondrial biogenesis in FA cells.
  • The combination of dimethyl fumarate and resveratrol demonstrated synergistic effects on FXN mRNA and mitochondrial biogenesis.
  • Combined treatment improved mitochondrial function, reduced oxidative stress, and enhanced motor performance in an FA mouse model.

Conclusions:

  • Dimethyl fumarate and resveratrol combination therapy shows significant potential in counteracting Friedreich's ataxia pathophysiology.
  • This combination offers additive benefits over single-agent therapy, with no observed toxicity.
  • Further research is warranted to fully elucidate the therapeutic potential of combined strategies for FA.