AUTHOR=Barrett Ellen F., Barrett John N., David Gavriel
TITLE=Dysfunctional mitochondrial Ca2+ handling in mutant SOD1 mouse models of fALS: integration of findings from motor neuron somata and motor terminals
JOURNAL=Frontiers in Cellular Neuroscience
VOLUME=8
YEAR=2014
URL=https://www.frontiersin.org/articles/10.3389/fncel.2014.00184
DOI=10.3389/fncel.2014.00184
ISSN=1662-5102
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.