TY - JOUR
T1 - Links between electrophysiological and molecular pathology of amyotrophic lateral sclerosis
AU - Quinlan, Katharina A.
N1 - Funding Information:
Funding was provided by NIH NINDS F32 NS063535 and NS034382.
PY - 2011/12
Y1 - 2011/12
N2 - Multiple deficits have been described in amyotrophic lateral sclerosis (ALS), from the first changes in normal functioning of the motoneurons and glia to the eventual loss of spinal and cortical motoneurons. In this review, current results, including changes in size, and electrical properties of motoneurons, glutamate excitotoxicity, calcium buffering, deficits in mitochondrial and cellular transport, impediments to proteostasis which lead to stress of the endoplasmic reticulum (ER), and glial contributions to motoneuronal vulnerability are recapitulated. Results are mainly drawn from the mutant SOD1 mouse model of ALS, and emphasis is placed on early changes that precede the onset of symptoms and the interplay between molecular and electrical processes.
AB - Multiple deficits have been described in amyotrophic lateral sclerosis (ALS), from the first changes in normal functioning of the motoneurons and glia to the eventual loss of spinal and cortical motoneurons. In this review, current results, including changes in size, and electrical properties of motoneurons, glutamate excitotoxicity, calcium buffering, deficits in mitochondrial and cellular transport, impediments to proteostasis which lead to stress of the endoplasmic reticulum (ER), and glial contributions to motoneuronal vulnerability are recapitulated. Results are mainly drawn from the mutant SOD1 mouse model of ALS, and emphasis is placed on early changes that precede the onset of symptoms and the interplay between molecular and electrical processes.
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U2 - 10.1093/icb/icr116
DO - 10.1093/icb/icr116
M3 - Article
C2 - 21989221
AN - SCOPUS:82255181339
VL - 51
SP - 913
EP - 925
JO - Integrative and Comparative Biology
JF - Integrative and Comparative Biology
SN - 1540-7063
IS - 6
ER -