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Physiology of Myelinated Nerve Conduction and Pathophysiology of Demyelination

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Myelin

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 1190))

Abstract

Nerve conduction in myelinated axons is a fascinating subject due to the intricate structure and complex properties of the axon and its relation to the equally complex Schwann cells surrounding it. This chapter first deals with normal functional aspects of voltage-gated ion channels in the axon and Schwann cell membranes as well as their related proteins. Next, the pathophysiological alterations that are induced by experimental studies to mimic and study neuropathic disorders in humans are discussed. Finally, a link is made with human neuropathies associated with antibodies against gangliosides, and the putative mechanisms of axonal degeneration in demyelinating neuropathies are discussed. Although this chapter is relevant to understand symptoms in human neuropathies, the reader is referred to Franssen and Straver (Muscle Nerve 49:4–20, 2014) for a review of translational and clinical studies in human patients.

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Abbreviations

AMAN:

Acute motor axonal neuropathy

AMP:

Adenine monophosphate

ATP:

Adenine triphosphate

cAMP:

Cyclic adenine triphosphate

CAP:

Compound action potential

Caspr:

Contactin-associated glycoprotein

CMAP:

Compound muscle action potential

Cx29:

Connexin-29

EAN:

Experimental allergic neuritis

ECF:

Extracellular fluid

ERM:

Ezrin-radixin-moesin

GD1a:

Ganglioside GD1a

GM1:

Ganglioside GM1

GT1b:

Ganglioside GT1b

HCN:

Hyperpolarization-activated cyclic-nucleotide-gated

HSPG:

Heparin-sulfate proteoglycan

Kv:

Voltage-gated potassium channel nomenclature

L-type:

Long-duration and large current generated by calcium channels

MAC:

Membrane attack complex

MAG:

Myelin-associated glycoprotein

MMN:

Multifocal motor neuropathy

Nav:

Voltage-gated sodium channel nomenclature

NF:

Neurofascin

NO:

Nitric oxide

Nr-CAM:

Neuronal cell adhesion molecule

P0:

Protein zero

P2:

Protein two

P2X:

Purinergic receptor nomenclature

PMP22:

Peripheral myelin protein twenty-two

T-type:

Transient and tiny current generated by calcium channels

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Authors and Affiliations

  1. Neurology and Clinical Neurophysiology, Department of Neuromuscular Disorders, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands

    Hessel Franssen

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  1. Hessel Franssen
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Correspondence to Hessel Franssen .

Editor information

Editors and Affiliations

  1. Diabetic Neuropathy Project, Department of Sensory and Motor Systems, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan

    Kazunori Sango

  2. Laboratory of Molecular Neuroscience and Neurology, Tokyo University of Pharmacy and Life Science, Hachioji, Tokyo, Japan

    Junji Yamauchi

  3. Department of Rehabilitation for the Movement Functions, Research Institute, National Rehabilitation Center, Tokorozawa, Saitama, Japan

    Toru Ogata

  4. Department of Neuroscience, Cell Biology, and Physiology, Boonshoft School of Medicine, Wright State University, Dayton, OH, USA

    Keiichiro Susuki

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Franssen, H. (2019). Physiology of Myelinated Nerve Conduction and Pathophysiology of Demyelination. In: Sango, K., Yamauchi, J., Ogata, T., Susuki, K. (eds) Myelin. Advances in Experimental Medicine and Biology, vol 1190. Springer, Singapore. https://doi.org/10.1007/978-981-32-9636-7_7

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