Electron Transport Disturbances and Neurodegeneration: From Albert Szent-Györgyi's Concept (Szeged) till Novel Approaches to Boost Mitochondrial Bioenergetics

Levente Szalárdy, Dénes Zádori, P. Klivényi, J. Toldi, L. Vécsei

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

Impaired function of certain mitochondrial respiratory complexes has long been linked to the pathogenesis of chronic neurodegenerative disorders such as Parkinson's and Huntington's diseases. Furthermore, genetic alterations of mitochondrial genome or nuclear genes encoding proteins playing essential roles in maintaining proper mitochondrial function can lead to the development of severe systemic diseases associated with neurodegeneration and vacuolar myelinopathy. At present, all of these diseases lack effective disease modifying therapy. Following a brief commemoration of Professor Albert Szent-Györgyi, a Nobel Prize laureate who pioneered in the field of cellular respiration, antioxidant processes, and the roles of free radicals in health and disease, the present paper overviews the current knowledge on the involvement of mitochondrial dysfunction in central nervous system diseases associated with neurodegeneration including Parkinson's and Huntington's disease as well as mitochondrial encephalopathies. The review puts special focus on the involvement and the potential therapeutic relevance of peroxisome proliferator-Activated receptor-gamma coactivator 1-Alpha (PGC-1), a nuclear-encoded master regulator of mitochondrial biogenesis and antioxidant responses in these disorders, the transcriptional activation of which may hold novel therapeutic value as a more system-based approach aiming to restore mitochondrial functions in neurodegenerative processes.

Original languageEnglish
Article number498401
JournalOxidative Medicine and Cellular Longevity
Volume2015
DOIs
Publication statusPublished - 2015

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Electron Transport
Energy Metabolism
Huntington Disease
Parkinson Disease
Antioxidants
Cell Respiration
Nobel Prize
Mitochondrial Genome
Central Nervous System Diseases
Organelle Biogenesis
Neurodegenerative Diseases
Transcriptional Activation
Free Radicals
Therapeutics
Gene encoding
Neurology
Health
Genes
Chemical activation
Proteins

ASJC Scopus subject areas

  • Cell Biology
  • Ageing
  • Biochemistry

Cite this

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abstract = "Impaired function of certain mitochondrial respiratory complexes has long been linked to the pathogenesis of chronic neurodegenerative disorders such as Parkinson's and Huntington's diseases. Furthermore, genetic alterations of mitochondrial genome or nuclear genes encoding proteins playing essential roles in maintaining proper mitochondrial function can lead to the development of severe systemic diseases associated with neurodegeneration and vacuolar myelinopathy. At present, all of these diseases lack effective disease modifying therapy. Following a brief commemoration of Professor Albert Szent-Gy{\"o}rgyi, a Nobel Prize laureate who pioneered in the field of cellular respiration, antioxidant processes, and the roles of free radicals in health and disease, the present paper overviews the current knowledge on the involvement of mitochondrial dysfunction in central nervous system diseases associated with neurodegeneration including Parkinson's and Huntington's disease as well as mitochondrial encephalopathies. The review puts special focus on the involvement and the potential therapeutic relevance of peroxisome proliferator-Activated receptor-gamma coactivator 1-Alpha (PGC-1), a nuclear-encoded master regulator of mitochondrial biogenesis and antioxidant responses in these disorders, the transcriptional activation of which may hold novel therapeutic value as a more system-based approach aiming to restore mitochondrial functions in neurodegenerative processes.",
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AU - Toldi, J.

AU - Vécsei, L.

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