Dysfunctional mitochondrial Ca2+ handling in mutant SOD1 mouse models of fALS: Integration of findings from motor neuron somata and motor terminals

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Abstract

Abundant evidence indicates that mitochondrial dysfunction and Ca2+ dysregulation contribute to the muscle denervation and motor neuron death that occur in mouse models of familial amyotrophic lateral sclerosis (fALS). This perspective considers measurements of mitochondrial function and Ca2+ handling made in both motor neuron somata and motor nerve terminals of SOD1-G93A mice at different disease stages. These complementary studies are integrated into a model of how mitochondrial dysfunction disrupts handling of stimulation-induced Ca2+ loads in presymptomatic and end-stages of this disease. Also considered are possible mechanisms underlying the findings that some treatments that preserve motor neuron somata fail to postpone degeneration of motor axons and terminals.

Original languageEnglish (US)
Article number184
JournalFrontiers in Cellular Neuroscience
Volume8
Issue numberJULY
DOIs
StatePublished - Jul 8 2014

Keywords

  • Mitochondria
  • Motor nerve terminal
  • Motor neuron
  • Mutant SOD1 models of fALS

ASJC Scopus subject areas

  • Cellular and Molecular Neuroscience

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