Pathophysiological Roles of Abnormal Axon Initial Segments in Neurodevelopmental Disorders.

ABSTRACT: The 20-60 μm axon initial segment (AIS) is proximally located at the interface between the axon and cell body. AIS has characteristic molecular and structural properties regulated by the crucial protein, ankyrin-G. The AIS contains a high density of Na+ channels relative to the cell body, which allows low thresholds for the initiation of action potential (AP). Molecular and physiological studies have shown that the AIS is also a key domain for the control of neuronal excitability by homeostatic mechanisms. The AIS has high plasticity in normal developmental processes and pathological activities, such as injury, neurodegeneration, and neurodevelopmental disorders (NDDs). In the first half of this review, we provide an overview of the molecular, structural, and ion-channel characteristics of

SUBMITTER: Fujitani M 

PROVIDER: S-EPMC8392614 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature