Mitochondrial pathobiology in ALS

Research output: Contribution to journalReview articlepeer-review


Amyotrophic lateral sclerosis (ALS) is the third most common human adult-onset neurodegenerative disease. Some forms of ALS are inherited, and disease-causing genes have been identified. Nevertheless, the mechanisms of neurodegeneration in ALS are unresolved. Genetic, biochemical, and morphological analyses of human ALS as well as cell and animal models of ALS reveal that mitochondria could have roles in this neurodegeneration. The varied functions and properties of mitochondria might render subsets of selectively vulnerable neurons intrinsically susceptible to cellular aging and stress and overlying genetic variations. Changes occur in mitochondrial respiratory chain enzymes and mitochondrial programmed cell death proteins in ALS. Transgenic mouse models of ALS reveal possible principles governing the biology of neurodegeneration that implicate mitochondria and the mitochondrial permeability transition pore. This paper reviews how mitochondrial pathobiology might contribute to the mechanisms of neurodegeneration in ALS.

Original languageEnglish (US)
Pages (from-to)569-579
Number of pages11
JournalJournal of Bioenergetics and Biomembranes
Issue number6
StatePublished - Dec 2011


  • Apoptosis
  • Cyclophilin D.
  • Mitochondrial permeability transition pore
  • Motor neuron
  • Voltage-dependent anion channel

ASJC Scopus subject areas

  • Physiology
  • Cell Biology


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