Mitochondria in motor nerve terminals

Function in health and in mutant superoxide dismutase 1 mouse models of familial ALS

Ellen Barrett, John Barrett, Gavriel David

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

Mitochondria contribute to neuronal function not only via their ability to generate ATP, but also via their ability to buffer large Ca 2+ loads. This review summarizes evidence that mitochondrial Ca 2+ sequestration is especially important for sustaining the function of vertebrate motor nerve terminals during repetitive stimulation. Motor terminal mitochondria can sequester large amounts of Ca 2+ because they have mechanisms for limiting both the mitochondrial depolarization and the increase in matrix free [Ca 2+] associated with Ca 2+ influx. In mice expressing mutations of human superoxide dismutase ?1 (SOD1) that cause some cases of familial amyotrophic lateral sclerosis (fALS), motor terminals degenerate well before the death of motor neuron cell bodies. This review presents evidence for early and progressive mitochondrial dysfunction in motor terminals of mutant SOD1 mice (G93A, G85R). This dysfunction would impair mitochondrial ability to sequester stimulation-associated Ca 2+ loads, and thus likely contributes to the early degeneration of motor terminals.

Original languageEnglish
Pages (from-to)581-586
Number of pages6
JournalJournal of Bioenergetics and Biomembranes
Volume43
Issue number6
DOIs
StatePublished - Dec 1 2011

Fingerprint

Mitochondria
Health
Motor Neurons
Superoxide Dismutase
Vertebrates
Buffers
Adenosine Triphosphate
Mutation
Superoxide Dismutase-1
Cell Body
Amyotrophic lateral sclerosis 1

Keywords

  • Amyotrophic lateral sclerosis
  • Calcium
  • Mitochondria
  • Motor nerve terminal
  • Superoxide dismutase 1

ASJC Scopus subject areas

  • Physiology
  • Cell Biology

Cite this

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abstract = "Mitochondria contribute to neuronal function not only via their ability to generate ATP, but also via their ability to buffer large Ca 2+ loads. This review summarizes evidence that mitochondrial Ca 2+ sequestration is especially important for sustaining the function of vertebrate motor nerve terminals during repetitive stimulation. Motor terminal mitochondria can sequester large amounts of Ca 2+ because they have mechanisms for limiting both the mitochondrial depolarization and the increase in matrix free [Ca 2+] associated with Ca 2+ influx. In mice expressing mutations of human superoxide dismutase ?1 (SOD1) that cause some cases of familial amyotrophic lateral sclerosis (fALS), motor terminals degenerate well before the death of motor neuron cell bodies. This review presents evidence for early and progressive mitochondrial dysfunction in motor terminals of mutant SOD1 mice (G93A, G85R). This dysfunction would impair mitochondrial ability to sequester stimulation-associated Ca 2+ loads, and thus likely contributes to the early degeneration of motor terminals.",
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