Mitochondrial dynamic abnormalities in amyotrophic lateral sclerosis

Zhen Jiang, Wenzhang Wang, George Perry, Xiongwei Zhu, Xinglong Wang

Research output: Contribution to journalArticlepeer-review

26 Scopus citations

Abstract

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease characterized by progressive loss of motor neurons in the brainstem and spinal cord. Currently, there is no cure or effective treatment for ALS and the cause of disease is unknown in the majority of ALS cases. Neuronal mitochondria dysfunction is one of the earliest features of ALS. Mitochondria are highly dynamic organelles that undergo continuous fission, fusion, trafficking and turnover, all of which contribute to the maintenance of mitochondrial function. Abnormal mitochondrial dynamics have been repeatedly reported in ALS and increasing evidence suggests altered mitochondrial dynamics as possible pathomechanisms underlying mitochondrial dysfunction in ALS. Here, we provide an overview of mitochondrial dysfunction and dynamic abnormalities observed in ALS, and discuss the possibility of targeting mitochondrial dynamics as a novel therapeutic approach for ALS.

Original languageEnglish (US)
Article number14
JournalTranslational Neurodegeneration
Volume4
Issue number1
DOIs
StatePublished - Jul 29 2015
Externally publishedYes

Keywords

  • ALS
  • Mitochondrial biogenesis and mitophagy
  • Mitochondrial dynamics
  • Mitochondrial dysfunction
  • Mitochondrial fission and fusion
  • Mitochondrial trafficking

ASJC Scopus subject areas

  • Clinical Neurology
  • Cognitive Neuroscience
  • Cellular and Molecular Neuroscience

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