Modified age-dependent expression of NaV1.6 in an ALS model correlates with motor cortex excitability alterations.
Action Potentials
/ physiology
Age Factors
Amyotrophic Lateral Sclerosis
/ genetics
Animals
Calcium
/ metabolism
Disease Models, Animal
Disease Progression
Gene Expression Regulation
Male
Mice
Mice, Transgenic
Motor Cortex
/ metabolism
Motor Neurons
/ metabolism
NAV1.6 Voltage-Gated Sodium Channel
/ genetics
ALS
Calcium de-regulation
Electrophysiology
G93A
NaV1.6
Neuronal excitability
Persistent Na(+) current
Journal
Neurobiology of disease
ISSN: 1095-953X
Titre abrégé: Neurobiol Dis
Pays: United States
ID NLM: 9500169
Informations de publication
Date de publication:
10 2019
10 2019
Historique:
received:
29
05
2019
revised:
28
06
2019
accepted:
10
07
2019
pubmed:
16
7
2019
medline:
25
3
2020
entrez:
15
7
2019
Statut:
ppublish
Résumé
Cortical hyperexcitability is an early and intrinsic feature of Amyotrophic Lateral Sclerosis (ALS), but the mechanisms underlying this critical neuronal dysfunction are poorly understood. Recently, we have demonstrated that layer V pyramidal neurons (PNs) in the primary motor cortex (M1) of one-month old (P30) G93A ALS mice display an early hyperexcitability status compared to Control mice. In order to investigate the time-dependent evolution of the cortical excitability in the G93A ALS model, here we have performed an electrophysiological and immunohistochemical study at three different mouse ages. M1 PNs from 14-days old (P14) G93A mice have shown no excitability alterations, while M1 PNs from 3-months old (P90) G93A mice have shown a hypoexcitability status, compared to Control mice. These age-dependent cortical excitability dysfunctions correlate with a similar time-dependent trend of the persistent sodium current (INa
Identifiants
pubmed: 31302244
pii: S0969-9961(19)30200-1
doi: 10.1016/j.nbd.2019.104532
pii:
doi:
Substances chimiques
NAV1.6 Voltage-Gated Sodium Channel
0
Scn8a protein, mouse
0
Calcium
SY7Q814VUP
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
104532Informations de copyright
Copyright © 2019 Elsevier Inc. All rights reserved.