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- W3193751016 endingPage "2110" @default.
- W3193751016 startingPage "2110" @default.
- W3193751016 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 AIS, AIS regulation through axo-axonic synapses, and axo−glial interactions. In the second half, to understand the relationship between NDDs and AIS, we discuss the activity-dependent plasticity of AIS, the human mutation of AIS regulatory genes, and the pathophysiological role of an abnormal AIS in NDD model animals and patients. We propose that the AIS may provide a potentially valuable structural biomarker in response to abnormal network activity in vivo as well as a new treatment concept at the neural circuit level." @default.
- W3193751016 created "2021-08-30" @default.
- W3193751016 creator A5001320985 @default.
- W3193751016 creator A5006817280 @default.
- W3193751016 creator A5074496941 @default.
- W3193751016 date "2021-08-17" @default.
- W3193751016 modified "2023-10-14" @default.
- W3193751016 title "Pathophysiological Roles of Abnormal Axon Initial Segments in Neurodevelopmental Disorders" @default.
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