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 language | English |
|---|---|
| Article number | 184 |
| Journal | Frontiers in Cellular Neuroscience |
| Volume | 8 |
| Issue number | JULY |
| DOIs | |
| State | Published - Jul 8 2014 |
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Keywords
- Mitochondria
- Motor nerve terminal
- Motor neuron
- Mutant SOD1 models of fALS
ASJC Scopus subject areas
- Cellular and Molecular Neuroscience
Cite this
Dysfunctional mitochondrial Ca2+ handling in mutant SOD1 mouse models of fALS : Integration of findings from motor neuron somata and motor terminals. / Barrett, Ellen; Barrett, John; David, Gavriel.
In: Frontiers in Cellular Neuroscience, Vol. 8, No. JULY, 184, 08.07.2014.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Dysfunctional mitochondrial Ca2+ handling in mutant SOD1 mouse models of fALS
T2 - Integration of findings from motor neuron somata and motor terminals
AU - Barrett, Ellen
AU - Barrett, John
AU - David, Gavriel
PY - 2014/7/8
Y1 - 2014/7/8
N2 - 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.
AB - 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.
KW - Mitochondria
KW - Motor nerve terminal
KW - Motor neuron
KW - Mutant SOD1 models of fALS
UR - http://www.scopus.com/inward/record.url?scp=84904069426&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84904069426&partnerID=8YFLogxK
U2 - 10.3389/fncel.2014.00184
DO - 10.3389/fncel.2014.00184
M3 - Article
AN - SCOPUS:84904069426
VL - 8
JO - Frontiers in Cellular Neuroscience
JF - Frontiers in Cellular Neuroscience
SN - 1662-5102
IS - JULY
M1 - 184
ER -