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标题:Mitochondrial Diseases of the Brain
时间:2020-03-26 23:18:42
DOI:10.1016/j.freeradbiomed.2013.03.018
作者:Chaturvedi, Rajnish K.;Flint Beal, M.
关键词:Parkinson's disease; Alzheimer's disease; Huntington's disease; Amyotrophic lateral sclerosis; Charcot-Marie-Tooth disease and Friedreich's ataxia; Neurodegenerative diseases; Mitochondrial dysfunction; Creatine; Co-Q10; PGC-1 alpha; Sirtuins; Free radicals
出版源: 《Free Radic Biol Med》 ,63 :1-29
摘要:Neurodegenerative disorders are debilitating diseases of the brain, characterized by behavioral, motor and cognitive impairments. Ample evidence underpins mitochondrial dysfunction as a central causal factor in the pathogenesis of neurodegenerative disorders including Parkinson's disease, Huntington's disease, Alzheimer's disease, Amyotrophic lateral sclerosis, Friedreich's ataxia and Charcot-Marie-Tooth disease. In this review, we discuss the role of mitochondrial dysfunction such as bioenergetics defects, mitochondrial DNA mutations, gene mutations, altered mitochondrial dynamics (mitochondrial fusion/fission, morphology, size, transport/trafficking, and movement), impaired transcription and the association of mutated proteins with mitochondria in these diseases. We highlight the therapeutic role of mitochondrial bioenergetic agents in toxin and in cellular and genetic animal models of neurodegenerative disorders. We also discuss clinical trials of bioenergetics agents in neurodegenerative disorders. Lastly, we shed light on PGC-1 alpha, TORC-1, AMP kinase, Nrf2-ARE, and Sirtuins as novel therapeutic targets for neurodegenerative disorders. (C) 2013 Elsevier Inc. All rights reserved.
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目录:
  • Mitochondrial Diseases of the Brain
    • Mitochondrial dysfunction in Parkinson’s disease (PD)
    • Bioenergetic defects in PD
    • Mitochondrial DNA defects in PD
    • Mitochondrial DNA mutations and polymorphisms in PD:
    • Gene mutations implicate mitochondrial dysfunction in PD:
    • Impaired mitochondrial movement, mitochondrial fission/fusion and mitophagy in PD
    • Mitochondrial dysfunction in Huntington’s disease (HD)
    • Impaired bioenergetics and decreased mitochondrial complexes activities in HD
    • mtDNA mutations and polymorphisms in HD:
    • Mitochondrial localization of mutant huntingtin in HD
    • Altered mitochondrial calcium handling
    • Altered mitochondrial dynamics and trafficking in HD
    • Transcriptional dysregulation in HD
    • Mitochondrial dysfunction in Alzheimer’s disease (AD):
      • Mitochondrial bioenergetics impairment in AD
      • Mitochondrial localization of Aβ, impaired mitochondrial dynamics and trafficking in AD
      • mtDNA encoded defects in AD
    • Mitochondrial dysfunction in Amyotrophic lateral sclerosis (ALS)
      • Interaction of mutant SOD1 with mitochondria in ALS
    • Mitochondrial dysfunction in Friedreich’s ataxia (FA)
    • Mitochondrial dysfunction in Charcot-Marie-Tooth disease (CMT)
    • Mitochondrial therapeutics for neurodegenerative diseases
      • Creatine
      • Clinical trials with creatine in PD
      • Clinical trials of creatine in HD
      • CoQ10
      • Clinical trials with CoQ10 in HD
      • Clinical trials with CoQ10 in PD
      • Clinical trials with CoQ10 in ALS, AD and Friedreich's ataxia
      • Idebenone
      • MitoQ and Mitochondrial targeted peptides
    • Nrf2/ARE pathway/Triterpenoids
      • Lipoic acid, Carnitine, Nicotinamide, and β-hydroxybutyrate
      • PGC-1α and PPARs
      • Transduceres of Creb-related binding protein (TORC)
    • AMP Kinase
      • Sirtuins (Sir2) and resveratrol
    • Conclusion and future perspectives
    • Acknowledgements
    • References

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