Molecular regulators of nerve conduction - Lessons from inherited neuropathies and rodent genetic models

Experimental Neurology
Jun Li

Abstract

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 internodal length. Finally, we will discuss how these basic neurobiological observations can be applied to inherited peripheral nerve diseases.

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Citations

May 6, 2016·Frontiers in Systems Neuroscience·John S Torday, William B Miller
Jul 15, 2016·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·F ManganelliUNKNOWN CMT-TRIAAL and CMT-TRAUK Group
Sep 13, 2016·Annals of Neurology·Bo HuJun Li
Mar 11, 2017·Scientific Reports·Martin WeberKimberly Scearce-Levie
Aug 16, 2017·Annals of Clinical and Translational Neurology·Jun Li
Aug 3, 2019·Human Mutation·Jin HeWanjin Chen
Jan 10, 2018·Journal of Neurochemistry·Yannick PoitelonMaria Laura Feltri
Jul 31, 2020·Annals of Clinical and Translational Neurology·Sadaf SabaJun Li
Mar 7, 2020·International Journal of Molecular Sciences·Pedram HonarpishehBhanu P Ganesh
Aug 29, 2020·Annals of Clinical and Translational Neurology·Nora E FritzJun Li
Dec 7, 2017·Frontiers in Cellular Neuroscience·Sophie BelinYannick Poitelon
Nov 9, 2019·F1000Research·Yongsheng ChenJun Li
Jan 1, 2021·Frontiers in Cell and Developmental Biology·Inês Lago-BaldaiaSarah D Ackerman
Mar 17, 2021·Annual Review of Neuroscience·Klaus-Armin Nave, Hauke B Werner

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