title: Dysfunctional mitochondrial Ca(2+) handling in mutant SOD1 mouse models of fALS: integration of findings from motor neuron somata and motor terminals.
abstract: Abundant evidence indicates that mitochondrial dysfunction and Ca(2+) 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 Ca(2+) 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 Ca(2+) 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.
authors: Department of Physiology and Biophysics, and Neuroscience Program, University of Miami Miller School of Medicine Miami, FL, USA.
authors: Ellen F
authors: EF
authors: Barrett
authors: Department of Physiology and Biophysics, and Neuroscience Program, University of Miami Miller School of Medicine Miami, FL, USA.
authors: John N
authors: JN
authors: Barrett
authors: Department of Physiology and Biophysics, and Neuroscience Program, University of Miami Miller School of Medicine Miami, FL, USA.
authors: Gavriel
authors: G
authors: David
keywords: Ca2+ regulation; mitochondria; motor nerve terminal; motor neuron; mutant SOD1 models of fALS