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Related Experiment Video

Updated: Jul 18, 2025

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Mitochondrial Dysfunction in Repeat Expansion Diseases.

Alberto Giménez-Bejarano1,2,3, Eva Alegre-Cortés1,2,3, Sokhna M S Yakhine-Diop1,2,3

  • 1Departamento de Bioquímica y Biología Molecular y Genética, Facultad de Enfermería y Terapia Ocupacional, Universidad de Extremadura, 10003 Cáceres, Spain.

Antioxidants (Basel, Switzerland)
|August 26, 2023
PubMed
Summary

Mitochondrial dysfunction is a key factor in repeat expansion diseases, affecting cellular processes and organelle health. Understanding these mitochondrial alterations offers potential for developing new neuroprotective treatments.

Keywords:
C9orf72Ca2+, mitophagyHuntington diseaseROSapoptosismyotonic dystrophy type 1

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Area of Science:

  • Genetics and Molecular Biology
  • Neuroscience
  • Cell Biology

Background:

  • Repeat expansion diseases are a class of over 50 neuromuscular and neurodegenerative disorders.
  • These disorders arise from expansions of repetitive DNA sequences, leading to diverse pathogenic mechanisms.
  • Mitochondrial dysfunction is increasingly recognized as a significant contributor to the pathogenesis of these diseases.

Purpose of the Study:

  • To review signaling pathways and proteins involved in mitochondrial function within repeat expansion diseases.
  • To analyze and compare mitochondrial alterations in Huntington's disease, C9orf72-associated frontotemporal dementia/amyotrophic lateral sclerosis, and myotonic dystrophy type 1.
  • To identify knowledge gaps and propose future research directions for neuroprotection.

Main Methods:

  • Comprehensive review of published literature on mitochondrial involvement in repeat expansion diseases.
  • Focus on analyzing data related to Huntington's disease, C9orf72-FTD/ALS, and myotonic dystrophy type 1.
  • Discussion of commonalities and differences in mitochondrial alterations across these disorders.

Main Results:

  • Repeat expansion diseases exhibit significant alterations in mitochondrial dynamics and biogenesis.
  • Specific proteins involved in mitochondrial processes have been identified as critical in these disorders.
  • Common and distinct patterns of mitochondrial dysfunction are observed across the studied diseases.

Conclusions:

  • Mitochondrial dysfunction is a central pathological feature in repeat expansion diseases.
  • Understanding these mechanisms is crucial for developing targeted neuroprotective strategies.
  • Further research into mitochondrial dynamics and specific protein involvement is warranted.