title: Molecular regulators of nerve conduction - Lessons from inherited neuropathies and rodent genetic models.
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.
authors: Department of Neurology, Center for Human Genetic Research, Vanderbilt Brain Institute, Vanderbilt University School of Medicine, Nashville, TN, USA; Tennessee Valley Healthcare System, Nashville VA, Nashville, TN, USA. Electronic address: jun.li.2@vanderbilt.edu.
authors: Jun
authors: J
authors: Li
mesh_terms: D000200:Action Potentials / Q000235:genetics; D000818:Animals; D004195:Disease Models, Animal; D009185:Myelin Proteins / Q000378:metabolism; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D009431:Neural Conduction / Q000235:genetics*; D010523:Peripheral Nervous System Diseases / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D012377:Rodentia
chemical_list: D009185:Myelin Proteins
keywords: Action potential; Adherens junction; Axonal degeneration; CMT1A; CMT2; CMTX1; Caspr; Charcot–Marie–Tooth disease; Compound muscle action potential; Connexin-1; Demyelination; HNPP; Hereditary neuropathy with liability to pressure palsies; Inherited neuropathy; Ion channel; Juxtaparanode; Myelination; Nerve conduction study; Node of Ranvier; PMP22; Paranode; Polyneuropathy; Sensory nerve action potential; Septate-like junction; Tight junction; Voltage-gated potassium channel; Voltage-gated sodium channel