Potassium channel abnormalities are consistent with early axon degeneration of motor axons in the G127X SOD1 mouse model of amyotrophic lateral sclerosis.

Potassium channel abnormalities are consistent with early axon degeneration of motor axons in the G127X SOD1 mouse model of amyotrophic lateral sclerosis. Exp Neurol. 2017 Mar 16;: Authors: Maglemose R, Hedegaard A, Lehnhoff J, Dimintiyanova KP, Moldovan M, Grøndahl L, Meehan CF Abstract Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease, which selectively affects upper and lower motoneurones. The underlying pathophysiology of the disease is complex but electrophysiological studies of peripheral nerves in ALS patients as well as human autopsy studies indicate that a potassium channel dysfunction/loss is present early in the symptomatic phase. It remains unclear to what extent potassium channel abnormalities reflect a specific pathogenic mechanism in ALS. The aim of this study was therefore to investigate the temporal changes in the expression and/or function of potassium channels in motoneurones in the adult G127X SOD1 mouse model of ALS, a model which has a very long presymptomatic phase. Evidence from animal models indicates that the early progressive motoneurone dysfunction and degeneration can be largely compensated by motor unit remodeling, delaying the clinical symptom onset. Experiments were therefore performed both before and after symptom onset. Immunohistochemistry of motor axons in the ventral roots of G127X SOD1 mice, was used to investigate juxta-paranodal Kv1.2 potassium channels along with nodal...
Source: Experimental Neurology - Category: Neurology Authors: Tags: Exp Neurol Source Type: research