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

Ellen Barrett, John Barrett, Gavriel David

Research output: Contribution to journalArticle

13 Citations (Scopus)

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
Article number184
JournalFrontiers in Cellular Neuroscience
Volume8
Issue numberJULY
DOIs
StatePublished - Jul 8 2014

Fingerprint

Carisoprodol
Motor Neurons
Muscle Denervation
Presynaptic Terminals
Amyotrophic lateral sclerosis 1

Keywords

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

ASJC Scopus subject areas

  • Cellular and Molecular Neuroscience

Cite this

@article{fef15a1849a64f32b9891a1ea1888005,
title = "Dysfunctional mitochondrial Ca2+ 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 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.",
keywords = "Mitochondria, Motor nerve terminal, Motor neuron, Mutant SOD1 models of fALS",
author = "Ellen Barrett and John Barrett and Gavriel David",
year = "2014",
month = "7",
day = "8",
doi = "10.3389/fncel.2014.00184",
language = "English",
volume = "8",
journal = "Frontiers in Cellular Neuroscience",
issn = "1662-5102",
publisher = "Frontiers Research Foundation",
number = "JULY",

}

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 -