Axonal Excitability in Amyotrophic Lateral Sclerosis : Axonal Excitability in ALS.

ABSTRACT: Axonal excitability testing provides in vivo assessment of axonal ion channel function and membrane potential. Excitability techniques have provided insights into the pathophysiological mechanisms underlying the development of neurodegeneration and clinical features of amyotrophic lateral sclerosis (ALS) and related neuromuscular disorders. Specifically, abnormalities of Na⁺ and K⁺ conductances contribute to development of membrane hyperexcitability in ALS, thereby leading to symptom generation of muscle cramps and fasciculations, in addition to promoting a neurodegenerative cascade via Ca²⁺-mediated processes. Modulation of axonal ion channel function in ALS has resulted in significant symptomatic improvement that has been accompanied by stabilization of a

SUBMITTER: Park SB 

PROVIDER: S-EPMC5233634 | biostudies-literature | 2017 Jan

REPOSITORIES: biostudies-literature