Journal
EXPERIMENTAL NEUROLOGY
Volume 267, Issue -, Pages 209-218Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.expneurol.2015.03.009
Keywords
Action potential; Nerve conduction study; Sensory nerve action potential; Compound muscle action potential; Ion channel; Voltage-gated sodium channel; Voltage-gated potassium channel; Node of Ranvier; Paranode; Juxtaparanode; Septate-like junction; Tight junction; Adherens junction; Myelination; Demyelination; Axonal degeneration; Polyneuropathy; Charcot-Marie-Tooth disease; Inherited neuropathy; CMT1A; CMT2; CMTX1; Hereditary neuropathy with liability to pressure palsies; HNPP; PMP22; Connexin-1; Caspr
Categories
Funding
- NINDS [R21NS081364, R01NS066927]
- U.S. Department of Veterans Affairs (RD funds)
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Myelinated nerve fibers are highly compartmentalized. Helically wrapped lipoprotein membranes of myelin are integrated with subsets of proteins specifically in each compartment to shape the physiological behavior of these nerve fibers. With the advance of molecular biology and genetics, many functions of these proteins have been revealed over the past decade. In this review, we will first discuss how action potential propagation has been understood by classical electrophysiological studies. In particular, the discussion will be concentrated on how the geometric dimensions of myelinated nerve fibers (such as internodal length and myelin thickness) may affect nerve conduction velocity. This discussion will then extend into how specific myelin proteins may shape these geometric parameters, thereby regulating action potential propagation. For instance, periaxin may specifically affect the internodal length, but not other parameters. In contrast, neuregulin-1 may affect myelin thickness, but not axon diameter or intenodal length. Finally, we will discuss how these basic neurobiological observations can be applied to inherited peripheral nerve diseases. Published by Elsevier Inc.
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