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.
Highlights
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
Mitochondria are important for temporary buffering of large Ca2+ loads in both the soma and peripheral motor terminals of motor neurons
Indicators demonstrate that mitochondria normally buffer about 50% of large Ca2+ loads in hypoglossal motor neurons (Fuchs et al, 2013)
Summary
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). SYMPTOMATIC SOD1-G93A MOTOR NEURON SOMATA AND TERMINALS SHOW REDUCED MITOCHONDRIAL Ca2+ UPTAKE AND TRANSIENT mPTP OPENING DURING STIMULATION
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